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diagnosis differential rheumatology management hematology table algorithm classification glomerulonephritis nephrology renal treatment vasculitis allergy clinical comparison hypersensitivity infectiousdiseases lupus monoclonal
Clarkson’s Disease - Capillary leak syndrome Epidemiology: Roughly 150 published cases, Median age 50 years, No sex
Clarkson’s Disease ... predominance, Relapsing-remitting ... Treatments - To treat disease ... Breillat #Clarksons ... #Disease #Capillary
Membranoproliferative Glomerulonephritis (MPGN) AKA: Mesangiocapillary GN • Active Urine Sediment (dysmorphic RBC, RBC casts, +/- proteinuria)
staining (lg + complement ... MGPN • Complement ... of alternative complement ... (Dense deposit disease ... MPGN without complement
Pulmonary Renal Syndromes and Mimics - Differential Diagnosis Framework Diffuse alveolar hemorrhage + rapidly progressive glomerulonephritis 90%: -
Vasculitis - Anti-GBM Disease ... anti-GBM) 10%: - Low Complement ... Vasculitis - Normal Complement ... Vasculitis, Behcet's Disease ... , mitral valve disease
Effect of Selected Diseases on Complement Levels - C3, C4 • SLE C3 ↓ C4 ↓↓
Effect of Selected Diseases ... on Complement Levels ... ThePeanutKidney #Complement
Rapidly Progressive Glomerulonephritis (RPGN) RPGN has three primary pathophysiologic causes differentiated by immunofluorescence Immune Complex Mediated (granular staining):
staining): • Low Complement ... Idiopathic • Normal Complement ... involvement: Anti-GBM Disease
C5-C9 Deficiency - Pathogenesis and Clinical Findings Note: • Risk of invasive meningococcal disease is 1000-10000x higher
meningococcal disease ... is rare Normal complement ... response: The complement ... activation -> Complement ... C7, C8, C9 -> Complement
Lyme Disease: Early and Late Clinical manifestations Stage 1. Localized • EM appears 7-14 days at the
Lyme Disease: Early ... joints - Remitting ... WuidQ #Lyme #Disease
Causes of Hypocomplementemia - CHAMPS Mnemonic C - Cryoglobulinemia (85%), C3 glomerulopathy, cirrhosis H - Heavy Chain deposition
Chain deposition disease ... Hypocomplementemia #Low #Complement
Monoclonal Gammopathy of Clinical Significance (MGUS) - Differential Diagnosis Neurologic-Centered: (+) Systemic Features: POEMS, AL amyloid, Cryoglobulinemia
leak syndrome (Clarkson's ... Ig deposition disease
Complement 1 esterase inhibitor (C1-INH) Deficiency Testing Algorithm INDICATIONS FOR TESTING: • Recurrent angioedema (without urticaria) •
Complement 1 esterase ... abdominal pain and vomitting ... ARUPLabs #C1INH #Complement
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