Epidemiology and Etiology

Epidemiology

• PrevalencePrevalenceThe total number of cases of a given disease in a specified population at a designated time. It is differentiated from incidence, which refers to the number of new cases in the population at a given time.Measures of Disease Frequency: 2.3 to 146 cases per million individuals
• IncidenceIncidenceThe number of new cases of a given disease during a given period in a specified population. It also is used for the rate at which new events occur in a defined population. It is differentiated from prevalence, which refers to all cases in the population at a given time.Measures of Disease Frequency: 0.4 to 11.9 cases per million person-years
  • PeakincidenceIncidenceThe number of new cases of a given disease during a given period in a specified population. It also is used for the rate at which new events occur in a defined population. It is differentiated from prevalence, which refers to all cases in the population at a given time.Measures of Disease Frequency: 65–74 years of age
• Geographic distribution: More common in northern latitudes, regions with predominantly European ancestry, and less common in EastAsiaASIASpinal Cord Injuries
• Male-to-female ratio: approximately equal

Etiology

• IdiopathicIdiopathicDermatomyositis
• Tends to occur more afterinfectionsInfectionsInvasion of the host organism by microorganisms or their toxins or by parasites that can cause pathological conditions or diseases.Chronic Granulomatous Disease, especially of the respiratory tract
• Genetic predisposition (positivefamily historyFamily HistoryAdult Health Maintenance)
• Autoimmune reaction

Pathophysiology

• InflammationInflammationInflammation is a complex set of responses to infection and injury involving leukocytes as the principal cellular mediators in the body’s defense against pathogenic organisms. Inflammation is also seen as a response to tissue injury in the process of wound healing. The 5 cardinal signs of inflammation are pain, heat, redness, swelling, and loss of function.Inflammation of small to medium-sized blood vessels, primarily affecting the upper and lower respiratory tract andkidneysKidneysThe kidneys are a pair of bean-shaped organs located retroperitoneally against the posterior wall of the abdomen on either side of the spine. As part of the urinary tract, the kidneys are responsible for blood filtration and excretion of water-soluble waste in the urine.Kidneys: Anatomy
• AutoimmunetriggerTriggerThe type of signal that initiates the inspiratory phase by the ventilatorInvasive Mechanical Ventilation may be related to bacterialcolonizationColonizationBacteriology withStaphylococcus aureusStaphylococcus aureusPotentially pathogenic bacteria found in nasal membranes, skin, hair follicles, and perineum of warm-blooded animals. They may cause a wide range of infections and intoxications.Brain Abscess
• Antineutrophil cytoplasmicantibodiesAntibodiesImmunoglobulins (Igs), also known as antibodies, are glycoprotein molecules produced by plasma cells that act in immune responses by recognizing and binding particular antigens. The various Ig classes are IgG (the most abundant), IgM, IgE, IgD, and IgA, which differ in their biologic features, structure, target specificity, and distribution.Immunoglobulins: Types and Functions (ANCAs) are responsible for theinflammationInflammationInflammation is a complex set of responses to infection and injury involving leukocytes as the principal cellular mediators in the body’s defense against pathogenic organisms. Inflammation is also seen as a response to tissue injury in the process of wound healing. The 5 cardinal signs of inflammation are pain, heat, redness, swelling, and loss of function.Inflammation in granulomatosis with polyangiitis (GPA).
  • ANCAs in GPA are those that react with proteinase 3, an enzyme in neutrophilgranulocytesGranulocytesLeukocytes with abundant granules in the cytoplasm. They are divided into three groups according to the staining properties of the granules: neutrophilic, eosinophilic, and basophilic. Mature granulocytes are the neutrophils; eosinophils; and basophils.White Myeloid Cells: Histology. Referred to asc-ANCAc-ANCAAntineutrophil Cytoplasmic Antibody (ANCA)-Associated Vasculitis (cytoplasmicANCAANCAGroup of systemic vasculitis with a strong association with anca. The disorders are characterized by necrotizing inflammation of small and medium size vessels, with little or no immune-complex deposits in vessel walls.Rapidly Progressive Glomerulonephritis; characteristic staining pattern shown in image below)
  • ANCAs form due to an aberrant epigenetic expression of proteinase 3 on all neutrophil membranes.
• ANCAs can activateneutrophilsNeutrophilsGranular leukocytes having a nucleus with three to five lobes connected by slender threads of chromatin, and cytoplasm containing fine inconspicuous granules and stainable by neutral dyes.Innate Immunity: Phagocytes and Antigen Presentation, increase their adherence toendotheliumEndotheliumA layer of epithelium that lines the heart, blood vessels (vascular endothelium), lymph vessels (lymphatic endothelium), and the serous cavities of the body.Arteries: Histology, and induce their degranulation, which leads to damage to the vessel wall.

Clinical Presentation

• Early stage symptoms includeconstitutional symptomsConstitutional SymptomsAntineutrophil Cytoplasmic Antibody (ANCA)-Associated Vasculitis, chronic upper respiratory tractinfectionsInfectionsInvasion of the host organism by microorganisms or their toxins or by parasites that can cause pathological conditions or diseases.Chronic Granulomatous Disease, nosebleed,ear painEar PainAcute Otitis Media,hemoptysisHemoptysisHemoptysis is defined as the expectoration of blood originating in the lower respiratory tract. Hemoptysis is a consequence of another disease process and can be classified as either life threatening or non-life threatening. Hemoptysis can result in significant morbidity and mortality due to both drowning (reduced gas exchange as the lungs fill with blood) and hemorrhagic shock.Hemoptysis, andhematuriaHematuriaPresence of blood in the urine.Renal Cell Carcinoma.
• Late stage symptoms include bronchialstenosisStenosisHypoplastic Left Heart Syndrome (HLHS),renal failureRenal failureConditions in which the kidneys perform below the normal level in the ability to remove wastes, concentrate urine, and maintain electrolyte balance; blood pressure; and calcium metabolism. Renal insufficiency can be classified by the degree of kidney damage (as measured by the level of proteinuria) and reduction in glomerular filtration rate.Crush Syndrome,saddle nose deformitySaddle Nose DeformityAntineutrophil Cytoplasmic Antibody (ANCA)-Associated Vasculitis, andsensorySensoryNeurons which conduct nerve impulses to the central nervous system.Nervous System: HistologyneuropathiesNeuropathiesChédiak-Higashi Syndrome.
• General symptoms:
  • Loss of appetite
  • Weight lossWeight lossDecrease in existing body weight.Bariatric Surgery
  • FeverFeverFever is defined as a measured body temperature of at least 38°C (100.4°F). Fever is caused by circulating endogenous and/or exogenous pyrogens that increase levels of prostaglandin E2 in the hypothalamus. Fever is commonly associated with chills, rigors, sweating, and flushing of the skin.Fever
  • FatigueFatigueThe state of weariness following a period of exertion, mental or physical, characterized by a decreased capacity for work and reduced efficiency to respond to stimuli.Fibromyalgia
• Mainly involves the kidney and thelungsLungsLungs are the main organs of the respiratory system. Lungs are paired viscera located in the thoracic cavity and are composed of spongy tissue. The primary function of the lungs is to oxygenate blood and eliminate CO2.Lungs: Anatomy:hematuriaHematuriaPresence of blood in the urine.Renal Cell Carcinoma andhemoptysisHemoptysisHemoptysis is defined as the expectoration of blood originating in the lower respiratory tract. Hemoptysis is a consequence of another disease process and can be classified as either life threatening or non-life threatening. Hemoptysis can result in significant morbidity and mortality due to both drowning (reduced gas exchange as the lungs fill with blood) and hemorrhagic shock.Hemoptysis
  • Rarely affects the heart, gastrointestinal tract,brainBrainThe part of central nervous system that is contained within the skull (cranium). Arising from the neural tube, the embryonic brain is comprised of three major parts including prosencephalon (the forebrain); mesencephalon (the midbrain); and rhombencephalon (the hindbrain). The developed brain consists of cerebrum; cerebellum; and other structures in the brain stem.Nervous System: Anatomy, Structure, and Classification, and other organs
• Renal symptoms:
  • Rapidly progressive glomerulonephritisRapidly Progressive GlomerulonephritisRapidly progressive glomerulonephritis (RPGN) is a syndrome of severe glomerular disease with progressive loss of kidney function within weeks to months. Histologically, crescents (the proliferation of epithelial cells and the infiltration of monocytes/macrophages in the Bowman space) are found in the glomeruli and arise from immunologic injury.Rapidly Progressive Glomerulonephritis
  • Chronic kidney diseaseChronic Kidney DiseaseChronic kidney disease (CKD) is kidney impairment that lasts for ≥ 3 months, implying that it is irreversible. Hypertension and diabetes are the most common causes; however, there are a multitude of other etiologies. In the early to moderate stages, CKD is usually asymptomatic and is primarily diagnosed by laboratory abnormalities.Chronic Kidney Disease (can progress torenal failureRenal failureConditions in which the kidneys perform below the normal level in the ability to remove wastes, concentrate urine, and maintain electrolyte balance; blood pressure; and calcium metabolism. Renal insufficiency can be classified by the degree of kidney damage (as measured by the level of proteinuria) and reduction in glomerular filtration rate.Crush Syndrome)
  • HematuriaHematuriaPresence of blood in the urine.Renal Cell Carcinoma (most commonly seen renal symptom)
  • HypertensionHypertensionHypertension, or high blood pressure, is a common disease that manifests as elevated systemic arterial pressures. Hypertension is most often asymptomatic and is found incidentally as part of a routine physical examination or during triage for an unrelated medical encounter.Hypertension
• Ear,noseNoseThe nose is the human body’s primary organ of smell and functions as part of the upper respiratory system. The nose may be best known for inhaling oxygen and exhaling carbon dioxide, but it also contributes to other important functions, such as tasting. The anatomy of the nose can be divided into the external nose and the nasal cavity.Nose Anatomy (External & Internal), andthroatThroatThe pharynx is a component of the digestive system that lies posterior to the nasal cavity, oral cavity, and larynx. The pharynx can be divided into the oropharynx, nasopharynx, and laryngopharynx. Pharyngeal muscles play an integral role in vital processes such as breathing, swallowing, and speaking.Pharynx: Anatomy symptoms:
  • NoseNoseThe nose is the human body’s primary organ of smell and functions as part of the upper respiratory system. The nose may be best known for inhaling oxygen and exhaling carbon dioxide, but it also contributes to other important functions, such as tasting. The anatomy of the nose can be divided into the external nose and the nasal cavity.Nose Anatomy (External & Internal): nosebleeds, runnynoseNoseThe nose is the human body’s primary organ of smell and functions as part of the upper respiratory system. The nose may be best known for inhaling oxygen and exhaling carbon dioxide, but it also contributes to other important functions, such as tasting. The anatomy of the nose can be divided into the external nose and the nasal cavity.Nose Anatomy (External & Internal), and saddle-nosedeformityDeformityExamination of the Upper Limbs due to destruction of nasalcartilageCartilageCartilage is a type of connective tissue derived from embryonic mesenchyme that is responsible for structural support, resilience, and the smoothness of physical actions. Perichondrium (connective tissue membrane surrounding cartilage) compensates for the absence of vasculature in cartilage by providing nutrition and support.Cartilage: Histology
  • Ears: otitis media,conductive hearing lossConductive hearing lossHearing loss due to interference with the mechanical reception or amplification of sound to the cochlea. The interference is in the outer or middle ear involving the ear canal; tympanic membrane; or ear ossicles.Hearing Loss due to auditory tube dysfunction,sensorineural hearing lossSensorineural hearing lossHearing loss resulting from damage to the cochlea and the sensorineural elements which lie internally beyond the oval and round windows. These elements include the auditory nerve and its connections in the brainstem.Hearing Loss (unclear mechanism)
  • Oral cavity: strawberrygingivitisGingivitisInflammation of gum tissue (gingiva) without loss of connective tissue.Chédiak-Higashi Syndrome,boneBoneBone is a compact type of hardened connective tissue composed of bone cells, membranes, an extracellular mineralized matrix, and central bone marrow. The 2 primary types of bone are compact and spongy.Bones: Structure and Types destruction with the loosening ofteethTeethNormally, an adult has 32 teeth: 16 maxillary and 16 mandibular. These teeth are divided into 4 quadrants with 8 teeth each. Each quadrant consists of 2 incisors (dentes incisivi), 1 canine (dens caninus), 2 premolars (dentes premolares), and 3 molars (dentes molares). Teeth are composed of enamel, dentin, and dental cement.Teeth: Anatomy, non-specificulcerationUlcerationCorneal Abrasions, Erosion, and Ulcers
• Tracheal symptoms:
  • WheezingWheezingWheezing is an abnormal breath sound characterized by a whistling noise that can be relatively high-pitched and shrill (more common) or coarse. Wheezing is produced by the movement of air through narrowed or compressed small (intrathoracic) airways.Wheezing
  • SubglotticstenosisStenosisHypoplastic Left Heart Syndrome (HLHS)
• Lung symptoms:
  • Cough
  • HemoptysisHemoptysisHemoptysis is defined as the expectoration of blood originating in the lower respiratory tract. Hemoptysis is a consequence of another disease process and can be classified as either life threatening or non-life threatening. Hemoptysis can result in significant morbidity and mortality due to both drowning (reduced gas exchange as the lungs fill with blood) and hemorrhagic shock.Hemoptysis (most commonly seen lung symptom)
  • Pulmonary nodules (sometimes referred to as “coin lesions”)
  • Infiltrates (often interpreted aspneumoniaPneumoniaPneumonia or pulmonary inflammation is an acute or chronic inflammation of lung tissue. Causes include infection with bacteria, viruses, or fungi. In more rare cases, pneumonia can also be caused through toxic triggers through inhalation of toxic substances, immunological processes, or in the course of radiotherapy.Pneumonia; represent intraparenchymal hemorrhage)
  • Cavitary lesions
  • BronchialstenosisStenosisHypoplastic Left Heart Syndrome (HLHS)
• Other symptoms:
  • ArthritisArthritisAcute or chronic inflammation of joints.Osteoarthritis
  • Subcutaneous nodules (granulomasGranulomasA relatively small nodular inflammatory lesion containing grouped mononuclear phagocytes, caused by infectious and noninfectious agents.Sarcoidosis) on the elbow, purpura
  • SensorySensoryNeurons which conduct nerve impulses to the central nervous system.Nervous System: HistologyneuropathyNeuropathyLeprosy and, rarely, mono-neuritis multiplex

Diagnosis

• Laboratory studies:
  • Complete blood count (CBC) often showsleukocytosisLeukocytosisA transient increase in the number of leukocytes in a body fluid.West Nile Virus, thrombocytosis (>400,000/microL), and normochromic,normocytic anemiaNormocytic AnemiaAnemia: Overview and Types
  • Erythrocyte sedimentation rateErythrocyte Sedimentation RateSoft Tissue Abscess (ESRESRSoft Tissue Abscess) is elevated
  • C-reactive protein (CRP) is elevated
  • Complete metabolic panel will show a low serumalbuminAlbuminSerum albumin from humans. It is an essential carrier of both endogenous substances, such as fatty acids and bilirubin, and of xenobiotics in the blood.Liver Function Tests level, elevated creatinine, and highly elevated bloodureaUreaA compound formed in the liver from ammonia produced by the deamination of amino acids. It is the principal end product of protein catabolism and constitutes about one half of the total urinary solids.Urea CyclenitrogenNitrogenAn element with the atomic symbol n, atomic number 7, and atomic weight [14. 00643; 14. 00728]. Nitrogen exists as a diatomic gas and makes up about 78% of the earth’s atmosphere by volume. It is a constituent of proteins and nucleic acids and found in all living cells.Urea Cycle
  • ANCAANCAGroup of systemic vasculitis with a strong association with anca. The disorders are characterized by necrotizing inflammation of small and medium size vessels, with little or no immune-complex deposits in vessel walls.Rapidly Progressive Glomerulonephritis testing:
    • 82-94% ofpatientsPatientsIndividuals participating in the health care system for the purpose of receiving therapeutic, diagnostic, or preventive procedures.Clinician–Patient Relationship with GPA orMPAMPAA primary systemic vasculitis of small- and some medium-sized vessels. It is characterized by a tropism for kidneys and lungs, positive association with anti-neutrophil cytoplasmic antibodies (ANCA), and a paucity of immunoglobulin deposits in vessel walls.Vasculitides have a positiveANCAANCAGroup of systemic vasculitis with a strong association with anca. The disorders are characterized by necrotizing inflammation of small and medium size vessels, with little or no immune-complex deposits in vessel walls.Rapidly Progressive Glomerulonephritis
    • GPA is predominantly associated withPR3-ANCAPR3-ANCAAntineutrophil Cytoplasmic Antibody (ANCA)-Associated Vasculitis (65-75% of cases)
    • MPAMPAA primary systemic vasculitis of small- and some medium-sized vessels. It is characterized by a tropism for kidneys and lungs, positive association with anti-neutrophil cytoplasmic antibodies (ANCA), and a paucity of immunoglobulin deposits in vessel walls.Vasculitides is predominantly associated withMPO-ANCAMPO-ANCAAntineutrophil Cytoplasmic Antibody (ANCA)-Associated Vasculitis (55-65% of cases)
    • 20-30% ofpatientsPatientsIndividuals participating in the health care system for the purpose of receiving therapeutic, diagnostic, or preventive procedures.Clinician–Patient Relationship with clinical GPA orMPAMPAA primary systemic vasculitis of small- and some medium-sized vessels. It is characterized by a tropism for kidneys and lungs, positive association with anti-neutrophil cytoplasmic antibodies (ANCA), and a paucity of immunoglobulin deposits in vessel walls.Vasculitides have the alternativeANCAANCAGroup of systemic vasculitis with a strong association with anca. The disorders are characterized by necrotizing inflammation of small and medium size vessels, with little or no immune-complex deposits in vessel walls.Rapidly Progressive Glomerulonephritis
    • At least 10% ofpatientsPatientsIndividuals participating in the health care system for the purpose of receiving therapeutic, diagnostic, or preventive procedures.Clinician–Patient Relationship are ANCA-negative
  • Additional testing to exclude alternative diagnoses:
    • Antinuclear antibody (ANA)
    • Anti-glomerularbasement membraneBasement membraneA darkly stained mat-like extracellular matrix (ecm) that separates cell layers, such as epithelium from endothelium or a layer of connective tissue. The ecm layer that supports an overlying epithelium or endothelium is called basal lamina. Basement membrane (bm) can be formed by the fusion of either two adjacent basal laminae or a basal lamina with an adjacent reticular lamina of connective tissue. Bm, composed mainly of type IV collagen; glycoprotein laminin; and proteoglycan, provides barriers as well as channels between interacting cell layers.Thin Basement Membrane Nephropathy (TBMN) (anti-GBM)antibodiesAntibodiesImmunoglobulins (Igs), also known as antibodies, are glycoprotein molecules produced by plasma cells that act in immune responses by recognizing and binding particular antigens. The various Ig classes are IgG (the most abundant), IgM, IgE, IgD, and IgA, which differ in their biologic features, structure, target specificity, and distribution.Immunoglobulins: Types and Functions
    • Serum C3 and C4 complement levels
    • Cryoglobulins
    • Tests forhepatitis BHepatitis BHepatitis B virus (HBV) is a partially double-stranded DNA virus, which belongs to the Orthohepadnavirus genus and the Hepadnaviridae family. Most individuals with acute HBV infection are asymptomatic or have mild, self-limiting symptoms. Chronic infection can be asymptomatic or create hepatic inflammation, leading to liver cirrhosis and hepatocellular carcinoma (HCC).Hepatitis B Virus and CvirusesVirusesMinute infectious agents whose genomes are composed of DNA or RNA, but not both. They are characterized by a lack of independent metabolism and the inability to replicate outside living host cells.Virology andHIVHIVAnti-HIV Drugs
    • Liver function testsLiver function testsLiver function tests, also known as hepatic function panels, are one of the most commonly performed screening blood tests. Such tests are also used to detect, evaluate, and monitor acute and chronic liver diseases.Liver Function Tests
    • TuberculosisTuberculosisTuberculosis (TB) is an infectious disease caused by Mycobacterium tuberculosis complex bacteria. The bacteria usually attack the lungs but can also damage other parts of the body. Approximately 30% of people around the world are infected with this pathogen, with the majority harboring a latent infection. Tuberculosis spreads through the air when a person with active pulmonary infection coughs or sneezes.Tuberculosis screen
    • Blood cultures
• UrinalysisUrinalysisExamination of urine by chemical, physical, or microscopic means. Routine urinalysis usually includes performing chemical screening tests, determining specific gravity, observing any unusual color or odor, screening for bacteriuria, and examining the sediment microscopically.Urinary Tract Infections (UTIs) in Children:
  • ProteinuriaProteinuriaThe presence of proteins in the urine, an indicator of kidney diseases.Nephrotic Syndrome in Children
  • MicroscopichematuriaHematuriaPresence of blood in the urine.Renal Cell Carcinoma
  • RBC casts and dysmorphicRBCsRBCsErythrocytes, or red blood cells (RBCs), are the most abundant cells in the blood. While erythrocytes in the fetus are initially produced in the yolk sac then the liver, the bone marrow eventually becomes the main site of production.Erythrocytes: Histology (nephritic sediment)
• Imaging
  • ChestX-rayX-rayPenetrating electromagnetic radiation emitted when the inner orbital electrons of an atom are excited and release radiant energy. X-ray wavelengths range from 1 pm to 10 nm. Hard x-rays are the higher energy, shorter wavelength x-rays. Soft x-rays or grenz rays are less energetic and longer in wavelength. The short wavelength end of the x-ray spectrum overlaps the gamma rays wavelength range. The distinction between gamma rays and x-rays is based on their radiation source.Pulmonary Function Tests: cavitary nodules, diffuse opacities,atelectasisAtelectasisAtelectasis is the partial or complete collapse of a part of the lung. Atelectasis is almost always a secondary phenomenon from conditions causing bronchial obstruction, external compression, surfactant deficiency, or scarring.Atelectasis, andconsolidationConsolidationPulmonary Function Tests
  • Computed tomography of the chest:consolidationConsolidationPulmonary Function Tests, ground-glass opacities,stenosisStenosisHypoplastic Left Heart Syndrome (HLHS) oflarynxLarynxThe larynx, also commonly called the voice box, is a cylindrical space located in the neck at the level of the C3-C6 vertebrae. The major structures forming the framework of the larynx are the thyroid cartilage, cricoid cartilage, and epiglottis. The larynx serves to produce sound (phonation), conducts air to the trachea, and prevents large molecules from reaching the lungs.Larynx: Anatomy or tracheobronchial tree, thickening of bronchus,bronchiectasisBronchiectasisBronchiectasis is a chronic disease of the airways that results from permanent bronchial distortion. This results from a continuous cycle of inflammation, bronchial damage and dilation, impaired clearance of secretions, and recurrent infections.Bronchiectasis, pleural thickening or effusion, andlymphadenopathyLymphadenopathyLymphadenopathy is lymph node enlargement (> 1 cm) and is benign and self-limited in most patients. Etiologies include malignancy, infection, and autoimmune disorders, as well as iatrogenic causes such as the use of certain medications. Generalized lymphadenopathy often indicates underlying systemic disease.Lymphadenopathy
• BiopsyBiopsyRemoval and pathologic examination of specimens from the living body.Ewing Sarcoma(confirmation of diagnosis)
  • BiopsyBiopsyRemoval and pathologic examination of specimens from the living body.Ewing Sarcoma of affected tissue should be performed ultimately in any patient suspected to have GPA.
  • BiopsyBiopsyRemoval and pathologic examination of specimens from the living body.Ewing Sarcomaconfirmsa diagnosis of granulomatosis with polyangiitis.
  • Poorly formedgranulomasGranulomasA relatively small nodular inflammatory lesion containing grouped mononuclear phagocytes, caused by infectious and noninfectious agents.Sarcoidosis,necrosisNecrosisThe death of cells in an organ or tissue due to disease, injury or failure of the blood supply.Ischemic Cell Damage, and manygiant cellsGiant cellsMultinucleated masses produced by the fusion of many cells; often associated with viral infections. In aids, they are induced when the envelope glycoprotein of the HIV virus binds to the CD4 antigen of uninfected neighboring T4 cells. The resulting syncytium leads to cell death and thus may account for the cytopathic effect of the virus.Giant Cell Arteritis are seen.

Management

Treatment

• Immunosuppression in different combinations is the primary method of treatment. 
• Formild disease (i.e., no glomerulonephritis or possibly life-threatening issues) →corticosteroidsCorticosteroidsChorioretinitis PLUSmethotrexateMethotrexateAn antineoplastic antimetabolite with immunosuppressant properties. It is an inhibitor of tetrahydrofolate dehydrogenase and prevents the formation of tetrahydrofolate, necessary for synthesis of thymidylate, an essential component of DNA.Antimetabolite Chemotherapy
• Formoderate to severe disease (i.e., with glomerulonephritis, pulmonary hemorrhage,hemoptysisHemoptysisHemoptysis is defined as the expectoration of blood originating in the lower respiratory tract. Hemoptysis is a consequence of another disease process and can be classified as either life threatening or non-life threatening. Hemoptysis can result in significant morbidity and mortality due to both drowning (reduced gas exchange as the lungs fill with blood) and hemorrhagic shock.Hemoptysis,etcETCThe electron transport chain (ETC) sends electrons through a series of proteins, which generate an electrochemical proton gradient that produces energy in the form of adenosine triphosphate (ATP).Electron Transport Chain (ETC).) →corticosteroidsCorticosteroidsChorioretinitis PLUS one of the followingimmunosuppressantsImmunosuppressantsImmunosuppressants are a class of drugs widely used in the management of autoimmune conditions and organ transplant rejection. The general effect is dampening of the immune response.Immunosuppressants:
  • CyclophosphamideCyclophosphamidePrecursor of an alkylating nitrogen mustard antineoplastic and immunosuppressive agent that must be activated in the liver to form the active aldophosphamide. It has been used in the treatment of lymphoma and leukemia. Its side effect, alopecia, has been used for defleecing sheep. Cyclophosphamide may also cause sterility, birth defects, mutations, and cancer.Immunosuppressants: Due to itstoxicityToxicityDosage Calculation,cyclophosphamideCyclophosphamidePrecursor of an alkylating nitrogen mustard antineoplastic and immunosuppressive agent that must be activated in the liver to form the active aldophosphamide. It has been used in the treatment of lymphoma and leukemia. Its side effect, alopecia, has been used for defleecing sheep. Cyclophosphamide may also cause sterility, birth defects, mutations, and cancer.Immunosuppressants is used only untilremissionRemissionA spontaneous diminution or abatement of a disease over time, without formal treatment.Cluster Headaches of the disease (approximately 3–6 months).
    • AfterremissionRemissionA spontaneous diminution or abatement of a disease over time, without formal treatment.Cluster Headaches, it is replaced with another immunosuppressive agent to maintainremissionRemissionA spontaneous diminution or abatement of a disease over time, without formal treatment.Cluster Headaches.
  • RituximabRituximabA murine-derived monoclonal antibody and antineoplastic agent that binds specifically to the cd20 antigen and is used in the treatment of leukemia; lymphoma and rheumatoid arthritis.Immunosuppressants
  • AzathioprineAzathioprineAn immunosuppressive agent used in combination with cyclophosphamide and hydroxychloroquine in the treatment of rheumatoid arthritis. According to the fourth annual report on carcinogens, this substance has been listed as a known carcinogen.Immunosuppressants
  • MethotrexateMethotrexateAn antineoplastic antimetabolite with immunosuppressant properties. It is an inhibitor of tetrahydrofolate dehydrogenase and prevents the formation of tetrahydrofolate, necessary for synthesis of thymidylate, an essential component of DNA.Antimetabolite Chemotherapy
  • Mycophenolate-mofetil (off-label use)
• PlasmapheresisPlasmapheresisProcedure whereby plasma is separated and extracted from anticoagulated whole blood and the red cells retransfused to the donor. Plasmapheresis is also employed for therapeutic use.Stevens-Johnson Syndrome indicated for:
  • Severe active renal disease
  • Presence of anti-GBMautoantibodiesAutoantibodiesAntibodies that react with self-antigens (autoantigens) of the organism that produced them.Blotting Techniques
  • Pulmonary hemorrhage

Complications

• Hearing lossHearing lossHearing loss, also known as hearing impairment, is any degree of impairment in the ability to apprehend sound as determined by audiometry to be below normal hearing thresholds. Clinical presentation may occur at birth or as a gradual loss of hearing with age, including a short-term or sudden loss at any point.Hearing Loss
• Renal FailureRenal failureConditions in which the kidneys perform below the normal level in the ability to remove wastes, concentrate urine, and maintain electrolyte balance; blood pressure; and calcium metabolism. Renal insufficiency can be classified by the degree of kidney damage (as measured by the level of proteinuria) and reduction in glomerular filtration rate.Crush Syndrome
• Risk of deepvenous thrombosisVenous thrombosisThe formation or presence of a blood clot (thrombus) within a vein.Budd-Chiari Syndrome

PrognosisPrognosisA prediction of the probable outcome of a disease based on a individual’s condition and the usual course of the disease as seen in similar situations.Non-Hodgkin Lymphomas

• If left untreated,mortalityMortalityAll deaths reported in a given population.Measures of Health Status rate within 2 years is 90%.
• With optimal treatment:
  • MortalityMortalityAll deaths reported in a given population.Measures of Health Status in the first year is about 10% (due toinfectionsInfectionsInvasion of the host organism by microorganisms or their toxins or by parasites that can cause pathological conditions or diseases.Chronic Granulomatous Disease following immunosuppression).
  • 5-year survival in those treated is 80%.

Differential Diagnosis

• Goodpasture syndromeGoodpasture SyndromeGoodpasture syndrome, also known as anti-glomerular basement membrane (GBM) disease, is an autoimmune disease characterized by circulating antibodies directed against glomerular and alveolar basement membranes. Affected individuals present with symptoms of rapidly progressive glomerulonephritis and alveolar hemorrhage.Goodpasture Syndrome (GPS): a subtype ofrapidly progressive glomerulonephritisRapidly Progressive GlomerulonephritisRapidly progressive glomerulonephritis (RPGN) is a syndrome of severe glomerular disease with progressive loss of kidney function within weeks to months. Histologically, crescents (the proliferation of epithelial cells and the infiltration of monocytes/macrophages in the Bowman space) are found in the glomeruli and arise from immunologic injury.Rapidly Progressive Glomerulonephritis (RPGNRPGNRapidly progressive glomerulonephritis (RPGN) is a syndrome of severe glomerular disease with progressive loss of kidney function within weeks to months. Histologically, crescents (the proliferation of epithelial cells and the infiltration of monocytes/macrophages in the Bowman space) are found in the glomeruli and arise from immunologic injury.Rapidly Progressive Glomerulonephritis) in which auto-antibodies are formed against the renal glomeruli and pulmonarycapillariesCapillariesCapillaries are the primary structures in the circulatory system that allow the exchange of gas, nutrients, and other materials between the blood and the extracellular fluid (ECF). Capillaries are the smallest of the blood vessels. Because a capillary diameter is so small, only 1 RBC may pass through at a time.Capillaries: Histology. Symptoms may includehemoptysisHemoptysisHemoptysis is defined as the expectoration of blood originating in the lower respiratory tract. Hemoptysis is a consequence of another disease process and can be classified as either life threatening or non-life threatening. Hemoptysis can result in significant morbidity and mortality due to both drowning (reduced gas exchange as the lungs fill with blood) and hemorrhagic shock.Hemoptysis and signs ofnephritic syndromeNephritic syndromeNephritic syndrome is a broad category of glomerular diseases characterized by glomerular hematuria, variable loss of renal function, and hypertension. These features are in contrast to those of nephrotic syndrome, which includes glomerular diseases characterized by severe proteinuria, although there is sometimes overlap of > 1 glomerular disease in the same individual.Nephritic Syndrome (hematuriaHematuriaPresence of blood in the urine.Renal Cell Carcinoma,oliguriaOliguriaDecreased urine output that is below the normal range. Oliguria can be defined as urine output of less than or equal to 0. 5 or 1 ml/kg/hr depending on the age.Renal Potassium Regulation,edemaEdemaEdema is a condition in which excess serous fluid accumulates in the body cavity or interstitial space of connective tissues. Edema is a symptom observed in several medical conditions. It can be categorized into 2 types, namely, peripheral (in the extremities) and internal (in an organ or body cavity).Edema,flank painFlank painPain emanating from below the ribs and above the ilium.Renal Cell Carcinoma). Labs show anti-glomerularbasement membraneBasement membraneA darkly stained mat-like extracellular matrix (ecm) that separates cell layers, such as epithelium from endothelium or a layer of connective tissue. The ecm layer that supports an overlying epithelium or endothelium is called basal lamina. Basement membrane (bm) can be formed by the fusion of either two adjacent basal laminae or a basal lamina with an adjacent reticular lamina of connective tissue. Bm, composed mainly of type IV collagen; glycoprotein laminin; and proteoglycan, provides barriers as well as channels between interacting cell layers.Thin Basement Membrane Nephropathy (TBMN) (GBM)antibodiesAntibodiesImmunoglobulins (Igs), also known as antibodies, are glycoprotein molecules produced by plasma cells that act in immune responses by recognizing and binding particular antigens. The various Ig classes are IgG (the most abundant), IgM, IgE, IgD, and IgA, which differ in their biologic features, structure, target specificity, and distribution.Immunoglobulins: Types and Functions. Immunosuppressive treatment withcorticosteroidsCorticosteroidsChorioretinitis andcyclophosphamideCyclophosphamidePrecursor of an alkylating nitrogen mustard antineoplastic and immunosuppressive agent that must be activated in the liver to form the active aldophosphamide. It has been used in the treatment of lymphoma and leukemia. Its side effect, alopecia, has been used for defleecing sheep. Cyclophosphamide may also cause sterility, birth defects, mutations, and cancer.Immunosuppressants is necessary to slow the progression.
• Polyarteritis nodosaPolyarteritis nodosaA form of necrotizing non-granulomatous inflammation occurring primarily in medium-sized arteries, often with microaneurysms. It is characterized by muscle, joint, and abdominal pain resulting from arterial infarction and scarring in affected organs. Polyarteritis nodosa with lung involvement is called churg-strauss syndrome.Vasculitides:a systemicvasculitisVasculitisInflammation of any one of the blood vessels, including the arteries; veins; and rest of the vasculature system in the body.Systemic Lupus Erythematosus of the small and medium vessels. The condition most commonly involves theskinSkinThe skin, also referred to as the integumentary system, is the largest organ of the body. The skin is primarily composed of the epidermis (outer layer) and dermis (deep layer). The epidermis is primarily composed of keratinocytes that undergo rapid turnover, while the dermis contains dense layers of connective tissue.Skin: Structure and Functions,peripheral nervesPeripheral NervesThe nerves outside of the brain and spinal cord, including the autonomic, cranial, and spinal nerves. Peripheral nerves contain non-neuronal cells and connective tissue as well as axons. The connective tissue layers include, from the outside to the inside, the epineurium, the perineurium, and the endoneurium.Nervous System: Histology, muscles, joints, gastrointestinal tract, andkidneysKidneysThe kidneys are a pair of bean-shaped organs located retroperitoneally against the posterior wall of the abdomen on either side of the spine. As part of the urinary tract, the kidneys are responsible for blood filtration and excretion of water-soluble waste in the urine.Kidneys: Anatomy. It is often associated withhepatitis BHepatitis BHepatitis B virus (HBV) is a partially double-stranded DNA virus, which belongs to the Orthohepadnavirus genus and the Hepadnaviridae family. Most individuals with acute HBV infection are asymptomatic or have mild, self-limiting symptoms. Chronic infection can be asymptomatic or create hepatic inflammation, leading to liver cirrhosis and hepatocellular carcinoma (HCC).Hepatitis B Virus or C (HPVHPVHuman papillomavirus (HPV) is a nonenveloped, circular, double-stranded DNA virus belonging to the Papillomaviridae family. Humans are the only reservoir, and transmission occurs through close skin-to-skin or sexual contact. Human papillomaviruses infect basal epithelial cells and can affect cell-regulatory proteins to result in cell proliferation.Papillomavirus (HPV) or HPC) infection.PatientsPatientsIndividuals participating in the health care system for the purpose of receiving therapeutic, diagnostic, or preventive procedures.Clinician–Patient Relationship often present with nonspecific symptoms (feverFeverFever is defined as a measured body temperature of at least 38°C (100.4°F). Fever is caused by circulating endogenous and/or exogenous pyrogens that increase levels of prostaglandin E2 in the hypothalamus. Fever is commonly associated with chills, rigors, sweating, and flushing of the skin.Fever, arthralgias),hypertensionHypertensionHypertension, or high blood pressure, is a common disease that manifests as elevated systemic arterial pressures. Hypertension is most often asymptomatic and is found incidentally as part of a routine physical examination or during triage for an unrelated medical encounter.Hypertension, increased risk ofmyocardial infarctionMyocardial infarctionMI is ischemia and death of an area of myocardial tissue due to insufficient blood flow and oxygenation, usually from thrombus formation on a ruptured atherosclerotic plaque in the epicardial arteries. Clinical presentation is most commonly with chest pain, but women and patients with diabetes may have atypical symptoms.Myocardial Infarction, andpolyneuropathyPolyneuropathyPolyneuropathy is any disease process affecting the function of or causing damage to multiple nerves of the peripheral nervous system. There are numerous etiologies of polyneuropathy, most of which are systemic and the most common of which is diabetic neuropathy.Polyneuropathy.Polyarteritis nodosaPolyarteritis nodosaA form of necrotizing non-granulomatous inflammation occurring primarily in medium-sized arteries, often with microaneurysms. It is characterized by muscle, joint, and abdominal pain resulting from arterial infarction and scarring in affected organs. Polyarteritis nodosa with lung involvement is called churg-strauss syndrome.Vasculitides usually spares thelungsLungsLungs are the main organs of the respiratory system. Lungs are paired viscera located in the thoracic cavity and are composed of spongy tissue. The primary function of the lungs is to oxygenate blood and eliminate CO2.Lungs: Anatomyand serologies will beANCAANCAGroup of systemic vasculitis with a strong association with anca. The disorders are characterized by necrotizing inflammation of small and medium size vessels, with little or no immune-complex deposits in vessel walls.Rapidly Progressive Glomerulonephritis negative.AngiographyAngiographyRadiography of blood vessels after injection of a contrast medium.Cardiac Surgery shows the classic beading appearance of alternating aneurysms andstenosisStenosisHypoplastic Left Heart Syndrome (HLHS) of small and medium vessels. Treatment involves immunosuppressive regimens and possibleantiviralAntiviralAntivirals for Hepatitis B therapy againstHBVHBVHepatitis B virus (HBV) is a partially double-stranded DNA virus, which belongs to the Orthohepadnavirus genus and the Hepadnaviridae family. Hepatitis B virus is transmitted by exposure to infectious blood or body fluids. Examples of types of exposure include sexual intercourse, IV drug use, and childbirth.Hepatitis B Virus andHCVHCVHepatitis C is an infection of the liver caused by the hepatitis C virus (HCV). Hepatitis C virus is an RNA virus and a member of the genus Hepacivirus and the family Flaviviridae. The infection can be transmitted through infectious blood or body fluids and may be transmitted during childbirth or through IV drug use or sexual intercourse.Hepatitis C Virus.
• Microscopic polyangiitisMicroscopic polyangiitisA primary systemic vasculitis of small- and some medium-sized vessels. It is characterized by a tropism for kidneys and lungs, positive association with anti-neutrophil cytoplasmic antibodies (ANCA), and a paucity of immunoglobulin deposits in vessel walls.Vasculitides:a necrotizingvasculitisVasculitisInflammation of any one of the blood vessels, including the arteries; veins; and rest of the vasculature system in the body.Systemic Lupus Erythematosus of small vessels (typically of thekidneysKidneysThe kidneys are a pair of bean-shaped organs located retroperitoneally against the posterior wall of the abdomen on either side of the spine. As part of the urinary tract, the kidneys are responsible for blood filtration and excretion of water-soluble waste in the urine.Kidneys: Anatomy,skinSkinThe skin, also referred to as the integumentary system, is the largest organ of the body. The skin is primarily composed of the epidermis (outer layer) and dermis (deep layer). The epidermis is primarily composed of keratinocytes that undergo rapid turnover, while the dermis contains dense layers of connective tissue.Skin: Structure and Functions, andlungsLungsLungs are the main organs of the respiratory system. Lungs are paired viscera located in the thoracic cavity and are composed of spongy tissue. The primary function of the lungs is to oxygenate blood and eliminate CO2.Lungs: Anatomy).PatientsPatientsIndividuals participating in the health care system for the purpose of receiving therapeutic, diagnostic, or preventive procedures.Clinician–Patient Relationship usually present with palpable purpura, pulmonaryvasculitisVasculitisInflammation of any one of the blood vessels, including the arteries; veins; and rest of the vasculature system in the body.Systemic Lupus Erythematosus withhemoptysisHemoptysisHemoptysis is defined as the expectoration of blood originating in the lower respiratory tract. Hemoptysis is a consequence of another disease process and can be classified as either life threatening or non-life threatening. Hemoptysis can result in significant morbidity and mortality due to both drowning (reduced gas exchange as the lungs fill with blood) and hemorrhagic shock.Hemoptysis, and pauci-immune glomerulonephritis withhypertensionHypertensionHypertension, or high blood pressure, is a common disease that manifests as elevated systemic arterial pressures. Hypertension is most often asymptomatic and is found incidentally as part of a routine physical examination or during triage for an unrelated medical encounter.Hypertension. This pathology will show positivep-ANCAp-ANCAAntineutrophil Cytoplasmic Antibody (ANCA)-Associated VasculitisantibodiesAntibodiesImmunoglobulins (Igs), also known as antibodies, are glycoprotein molecules produced by plasma cells that act in immune responses by recognizing and binding particular antigens. The various Ig classes are IgG (the most abundant), IgM, IgE, IgD, and IgA, which differ in their biologic features, structure, target specificity, and distribution.Immunoglobulins: Types and Functions (unlike in GPA). Treatment involves an immunosuppressive regimen.
• Churg-Strauss syndromeChurg-Strauss syndromeWidespread necrotizing angiitis with granulomas. Pulmonary involvement is frequent. Asthma or other respiratory infection may precede evidence of vasculitis. Eosinophilia and lung involvement differentiate this disease from polyarteritis nodosa.Vasculitides:also known as eosinophilic granulomatosis with polyangiitis. Churg-Strauss is amultisystem diseaseMultisystem diseaseMitochondrial Myopathies characterized by necrotizing granulomatousvasculitisVasculitisInflammation of any one of the blood vessels, including the arteries; veins; and rest of the vasculature system in the body.Systemic Lupus Erythematosus witheosinophiliaEosinophiliaAbnormal increase of eosinophils in the blood, tissues or organs.Autosomal Dominant Hyperimmunoglobulin E Syndrome which most commonly involves the lung and theskinSkinThe skin, also referred to as the integumentary system, is the largest organ of the body. The skin is primarily composed of the epidermis (outer layer) and dermis (deep layer). The epidermis is primarily composed of keratinocytes that undergo rapid turnover, while the dermis contains dense layers of connective tissue.Skin: Structure and Functions.PatientsPatientsIndividuals participating in the health care system for the purpose of receiving therapeutic, diagnostic, or preventive procedures.Clinician–Patient Relationship present with severe allergicasthmaAsthmaAsthma is a chronic inflammatory respiratory condition characterized by bronchial hyperresponsiveness and airflow obstruction. The disease is believed to result from the complex interaction of host and environmental factors that increase disease predisposition, with inflammation causing symptoms and structural changes. Patients typically present with wheezing, cough, and dyspnea.Asthma attacks,allergic rhinitisAllergic rhinitisAn inflammation of the nasal mucosa triggered by allergens.Rhinitis,sinusitisSinusitisSinusitis refers to inflammation of the mucosal lining of the paranasal sinuses. The condition usually occurs concurrently with inflammation of the nasal mucosa (rhinitis), a condition known as rhinosinusitis. Acute sinusitis is due to an upper respiratory infection caused by a viral, bacterial, or fungal agent.Sinusitis,polyneuropathyPolyneuropathyPolyneuropathy is any disease process affecting the function of or causing damage to multiple nerves of the peripheral nervous system. There are numerous etiologies of polyneuropathy, most of which are systemic and the most common of which is diabetic neuropathy.Polyneuropathy (footFootThe foot is the terminal portion of the lower limb, whose primary function is to bear weight and facilitate locomotion. The foot comprises 26 bones, including the tarsal bones, metatarsal bones, and phalanges. The bones of the foot form longitudinal and transverse arches and are supported by various muscles, ligaments, and tendons.Foot: Anatomy or wrist drop), andskinSkinThe skin, also referred to as the integumentary system, is the largest organ of the body. The skin is primarily composed of the epidermis (outer layer) and dermis (deep layer). The epidermis is primarily composed of keratinocytes that undergo rapid turnover, while the dermis contains dense layers of connective tissue.Skin: Structure and Functions nodules. They will have peripheral bloodeosinophiliaEosinophiliaAbnormal increase of eosinophils in the blood, tissues or organs.Autosomal Dominant Hyperimmunoglobulin E Syndrome and positivep-ANCAp-ANCAAntineutrophil Cytoplasmic Antibody (ANCA)-Associated VasculitisantibodiesAntibodiesImmunoglobulins (Igs), also known as antibodies, are glycoprotein molecules produced by plasma cells that act in immune responses by recognizing and binding particular antigens. The various Ig classes are IgG (the most abundant), IgM, IgE, IgD, and IgA, which differ in their biologic features, structure, target specificity, and distribution.Immunoglobulins: Types and Functions (unlike in GPA). Treatment involves immunosuppressive regimen.
• Giant cell arteritisGiant Cell ArteritisGiant cell arteritis (GCA), also known as temporal arteritis, is a type of large-vessel vasculitis that predominantly affects the aorta and its major branches, with a predilection for the branches of the carotid (including the temporal artery). Giant cell arteritis is defined by inflammatory leukocytes in the vessel walls leading to reactive damage, ischemia, and necrosis.Giant Cell Arteritis: also known astemporal arteritisTemporal arteritisGiant cell arteritis (GCA), also known as temporal arteritis, is a type of large-vessel vasculitis that predominantly affects the aorta and its major branches, with a predilection for the branches of the carotid (including the temporal artery). Giant cell arteritis is defined by inflammatory leukocytes in the vessel walls leading to reactive damage, ischemia, and necrosis.Giant Cell Arteritis. Giant cell is avasculitisVasculitisInflammation of any one of the blood vessels, including the arteries; veins; and rest of the vasculature system in the body.Systemic Lupus Erythematosus that causesinflammationInflammationInflammation is a complex set of responses to infection and injury involving leukocytes as the principal cellular mediators in the body’s defense against pathogenic organisms. Inflammation is also seen as a response to tissue injury in the process of wound healing. The 5 cardinal signs of inflammation are pain, heat, redness, swelling, and loss of function.Inflammation of medium and large-sizedarteriesArteriesArteries are tubular collections of cells that transport oxygenated blood and nutrients from the heart to the tissues of the body. The blood passes through the arteries in order of decreasing luminal diameter, starting in the largest artery (the aorta) and ending in the small arterioles. Arteries are classified into 3 types: large elastic arteries, medium muscular arteries, and small arteries and arterioles.Arteries: Histology, particularly thecarotid arteriesCarotid ArteriesEither of the two principal arteries on both sides of the neck that supply blood to the head and neck; each divides into two branches, the internal carotid artery and the external carotid artery.Carotid Arterial System: Anatomy and theaortaAortaThe main trunk of the systemic arteries.Mediastinum and Great Vessels: Anatomy.PatientsPatientsIndividuals participating in the health care system for the purpose of receiving therapeutic, diagnostic, or preventive procedures.Clinician–Patient Relationship usually present withconstitutional symptomsConstitutional SymptomsAntineutrophil Cytoplasmic Antibody (ANCA)-Associated Vasculitis, new-onsetheadacheHeadacheThe symptom of pain in the cranial region. It may be an isolated benign occurrence or manifestation of a wide variety of headache disorders.Brain Abscess, tender and hardened temporal artery,jaw claudicationJaw claudicationMandibular pain or fatigue during mastication that is relieved with restGiant Cell Arteritis, andamaurosis fugaxAmaurosis fugaxTransient complete or partial monocular blindness due to retinal ischemia. This may be caused by emboli from the carotid artery (usually in association with carotid stenosis) and other locations that enter the central retinal artery.Carotid Artery Stenosis. Laboratory studies show elevatedESRESRSoft Tissue Abscess and CRP.BiopsyBiopsyRemoval and pathologic examination of specimens from the living body.Ewing Sarcoma is needed for diagnosis, which shows mononuclear infiltration of vessel walls and formation ofgiant cellsGiant cellsMultinucleated masses produced by the fusion of many cells; often associated with viral infections. In aids, they are induced when the envelope glycoprotein of the HIV virus binds to the CD4 antigen of uninfected neighboring T4 cells. The resulting syncytium leads to cell death and thus may account for the cytopathic effect of the virus.Giant Cell Arteritis. Treatment involves prompt administration ofglucocorticoidsGlucocorticoidsGlucocorticoids are a class within the corticosteroid family. Glucocorticoids are chemically and functionally similar to endogenous cortisol. There are a wide array of indications, which primarily benefit from the antiinflammatory and immunosuppressive effects of this class of drugs.Glucocorticoids.
• Henoch-Schonlein purpura:an autoimmune small vesselvasculitisVasculitisInflammation of any one of the blood vessels, including the arteries; veins; and rest of the vasculature system in the body.Systemic Lupus Erythematosus. The condition typically presents as a triad ofabdominal painAbdominal PainAcute Abdomen,hematuriaHematuriaPresence of blood in the urine.Renal Cell Carcinoma, and purpuricrashRashRocky Mountain Spotted Fever. Pathophysiology involves deposition of immunoglobulin Aimmune complexesImmune complexesThe complex formed by the binding of antigen and antibody molecules. The deposition of large antigen-antibody complexes leading to tissue damage causes immune complex diseases.C3 Deficiency in multiple vessels following atriggerTriggerThe type of signal that initiates the inspiratory phase by the ventilatorInvasive Mechanical Ventilation (infection/environmental), with symptoms depending on the tissues supplied by these vessels. Henoch-Schonlein has a clinical diagnosis and is managed symptomatically. 
• RheumatoidarthritisArthritisAcute or chronic inflammation of joints.Osteoarthritis: an inflammatorypolyarthritisPolyarthritisRheumatoid Arthritis that presents withpainPainAn unpleasant sensation induced by noxious stimuli which are detected by nerve endings of nociceptive neurons.Pain: Types and Pathways in joints that are warm and “boggy” to touch. ThispainPainAn unpleasant sensation induced by noxious stimuli which are detected by nerve endings of nociceptive neurons.Pain: Types and Pathways is typically worse in the mornings and improves during the day. Joint involvement issymmetricalSymmetricalDermatologic Examination, with the inclusion of the proximal interphalangeal andmetacarpophalangeal jointsMetacarpophalangeal jointsThe articulation between a metacarpal bone and a phalanx.Examination of the Upper Limbs but sparing of the distalinterphalangeal jointsInterphalangeal jointsHand: Anatomy. Nonsteroidal anti-inflammatory drugs are the mainstay ofpainPainAn unpleasant sensation induced by noxious stimuli which are detected by nerve endings of nociceptive neurons.Pain: Types and Pathways treatment; disease-modifying treatment options includemethotrexateMethotrexateAn antineoplastic antimetabolite with immunosuppressant properties. It is an inhibitor of tetrahydrofolate dehydrogenase and prevents the formation of tetrahydrofolate, necessary for synthesis of thymidylate, an essential component of DNA.Antimetabolite Chemotherapy,sulfasalazineSulfasalazineA drug that is used in the management of inflammatory bowel diseases. Its activity is generally considered to lie in its metabolic breakdown product, 5-aminosalicylic acid released in the colon.Sulfonamides and Trimethoprim,hydroxychloroquineHydroxychloroquineA chemotherapeutic agent that acts against erythrocytic forms of malarial parasites. Hydroxychloroquine appears to concentrate in food vacuoles of affected protozoa. It inhibits plasmodial heme polymerase.Immunosuppressants, and TNFɑ-inhibitors.
• Systemic lupus erythematosusSystemic lupus erythematosusSystemic lupus erythematosus (SLE) is a chronic autoimmune, inflammatory condition that causes immune-complex deposition in organs, resulting in systemic manifestations. Women, particularly those of African American descent, are more commonly affected.Systemic Lupus Erythematosus:a chronic inflammatory condition characterized by the clinical involvement of theskinSkinThe skin, also referred to as the integumentary system, is the largest organ of the body. The skin is primarily composed of the epidermis (outer layer) and dermis (deep layer). The epidermis is primarily composed of keratinocytes that undergo rapid turnover, while the dermis contains dense layers of connective tissue.Skin: Structure and Functions, joints,kidneysKidneysThe kidneys are a pair of bean-shaped organs located retroperitoneally against the posterior wall of the abdomen on either side of the spine. As part of the urinary tract, the kidneys are responsible for blood filtration and excretion of water-soluble waste in the urine.Kidneys: Anatomy, blood cells, andcentral nervous systemCentral nervous systemThe main information-processing organs of the nervous system, consisting of the brain, spinal cord, and meninges.Nervous System: Anatomy, Structure, and Classification. The condition is believed to be anautoimmune disorderAutoimmune DisorderSeptic Arthritis and has been associated with the formation ofautoantibodiesAutoantibodiesAntibodies that react with self-antigens (autoantigens) of the organism that produced them.Blotting Techniques such as antinuclear antibody, anti-Smith, and anti-dsDNA. The main clinical features of lupus includemalar rashMalar RashSystemic Lupus Erythematosus, jointpainPainAn unpleasant sensation induced by noxious stimuli which are detected by nerve endings of nociceptive neurons.Pain: Types and Pathways,feverFeverFever is defined as a measured body temperature of at least 38°C (100.4°F). Fever is caused by circulating endogenous and/or exogenous pyrogens that increase levels of prostaglandin E2 in the hypothalamus. Fever is commonly associated with chills, rigors, sweating, and flushing of the skin.Fever,proteinuriaProteinuriaThe presence of proteins in the urine, an indicator of kidney diseases.Nephrotic Syndrome in Children,hypertensionHypertensionHypertension, or high blood pressure, is a common disease that manifests as elevated systemic arterial pressures. Hypertension is most often asymptomatic and is found incidentally as part of a routine physical examination or during triage for an unrelated medical encounter.Hypertension,anemiaAnemiaAnemia is a condition in which individuals have low Hb levels, which can arise from various causes. Anemia is accompanied by a reduced number of RBCs and may manifest with fatigue, shortness of breath, pallor, and weakness. Subtypes are classified by the size of RBCs, chronicity, and etiology.Anemia: Overview and Types, lymphopenia,seizuresSeizuresA seizure is abnormal electrical activity of the neurons in the cerebral cortex that can manifest in numerous ways depending on the region of the brain affected. Seizures consist of a sudden imbalance that occurs between the excitatory and inhibitory signals in cortical neurons, creating a net excitation. The 2 major classes of seizures are focal and generalized.Seizures, and/or psychosis.

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