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diagnosis differential rheumatology vasculitis hematology huv management syndrome urticarial ahus atypical c3 c4 champs classification hemolytic levels low microangiopathy mimics
Diagnosis and Management of Hypocomplementemic Urticarial Vasculitis (HUV) Diagnosis of HUV 1) Chronic urticaria 2) Leukocytoclastic vasculitis 3)
Management of Hypocomplementemic ... Urticarial Vasculitis (HUV ... Diagnosis of HUV ... vasculitis 3) Hypocomplementemia ... TerrierBen #Hypocomplementemic
Pathophysiology of Hypocomplementemic Urticarial Vasculitis C1q/anti-C1q antibiody -> Immune Complex Formation - Complement Pathway Activation (C3a and C5a)
Pathophysiology of Hypocomplementemic ... Complex Formation - Complement ... TerrierBen #Hypocomplementemic ... Urticarial #Vasculitis #HUV
Causes of Hypocomplementemia - CHAMPS Mnemonic C - Cryoglobulinemia (85%), C3 glomerulopathy, cirrhosis H - Heavy Chain deposition
Causes of Hypocomplementemia ... A - Atypical HUS ... @AnnKumfer #Hypocomplementemia ... #Low #Complement
Atypical HUS - Hemolytic Uremic Syndrome Unlike typical HUS, which is shigatoxin mediated, aHUS is not typically
Atypical HUS - Hemolytic ... Unlike typical HUS ... autoimmune like SLE ... It is complement ... with Eculizumab (complement
Effect of Selected Diseases on Complement Levels - C3, C4 • SLE C3 ↓ C4 ↓↓
Selected Diseases on Complement ... Levels - C3, C4 • SLE ... • Infection eg SBE ... C3 ↓ C4 → • HUS ... ThePeanutKidney #Complement
Vasculitis and Vasculitides - Differential Diagnosis Framework When to Consider Vasculitis: • Purpura, ischemic skin lesions •
often involved • Hypocomplementemic ... Urticaria and hypocomplementemia ... against MPO • Complement ... vasculitis, and SLE
Pulmonary Renal Syndromes and Mimics - Differential Diagnosis Framework Diffuse alveolar hemorrhage + rapidly progressive glomerulonephritis 90%: -
anti-GBM) 10%: - Low Complement ... Levels: SLE, Cryoglobulinemic ... Vasculitis - Normal Complement ... hypertension - MAHA: HUS ... , atypical HUS,
Thrombotic Microangiopathy - TMAs 1. Not all MAHAs are due to TMAs but (almost) all TMAs cause
is due to TTP, compliment-mediated ... TTP (atypical HUS ... ), Shiga-toxin HUS ... catastrophic APS, SRC, SLE ... diagnosis #ttp #hus
Vasculitis - Differential Diagnosis Framework When to Consider Vasculitis: • Purpura, ischemic skin lesions • Mononeuritis multiplex • Hematuria, proteinuria,
diseases, such as SLE ... Elevated AST ALT • Hypocomplementemia ... Vasculitis) • Hypocomplementemic
Sjogren's Syndrome Overview Epidemiology: • F > M: 9:1 • 5-6th Decades (can be any age) Autoimmune exocrinopathy multisystemic disease
overlaps with RA, SLE ... Cytopenia: 2% • Hypocomplementemia
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