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diagnosis rheumatology differential management pulmonary syndromes treatment champs classification clinicopathology disease erythematosus gammopathy glomerulonephritis hematology huv igg4 laboratory lupus mgrs
Pathophysiology of Hypocomplementemic Urticarial Vasculitis C1q/anti-C1q antibiody -> Immune Complex Formation - Complement Pathway Activation (C3a and C5a)
Pathophysiology of Hypocomplementemic ... Urticarial Vasculitis ... Complex Formation - Complement ... Formation -> Vasculitis ... TerrierBen #Hypocomplementemic
Causes of Hypocomplementemia - CHAMPS Mnemonic C - Cryoglobulinemia (85%), C3 glomerulopathy, cirrhosis H - Heavy Chain deposition
Causes of Hypocomplementemia ... Erythematosus (total 50, renal ... @AnnKumfer #Hypocomplementemia ... #Low #Complement
Pulmonary Renal Syndromes and Mimics - Differential Diagnosis Framework Diffuse alveolar hemorrhage + rapidly progressive glomerulonephritis 90%: -
Pulmonary Renal ... hemorrhage + rapidly progressive ... anti-GBM) 10%: - Low ... Complement Levels ... - Normal Complement
Vasculitis and Vasculitides - Differential Diagnosis Framework When to Consider Vasculitis: • Purpura, ischemic skin lesions •
proteinuria, rapidly progressing ... often involved • Hypocomplementemic ... Urticaria and hypocomplementemia ... against MPO • Complement ... (Low levels mixed
Rapidly Progressive Glomerulonephritis (RPGN) RPGN has three primary pathophysiologic causes differentiated by immunofluorescence Immune Complex Mediated (granular staining):
Rapidly Progressive ... staining): • Low ... Complement: Post-strep ... Glomerulonephritis #Vasculitis ... differential #diagnosis #algorithm
Vasculitis - Differential Diagnosis Framework When to Consider Vasculitis: • Purpura, ischemic skin lesions • Mononeuritis multiplex • Hematuria, proteinuria,
proteinuria, Rapidly progressing ... pulmonary and renal ... is 50 years • Progressive ... Elevated AST ALT • Hypocomplementemia ... ) • Hypocomplementemic
IgG4-Related Disease Clinical history: Man, 60 years, multiorgan involvement, unique or multiple inflammatory pseudotumor 4 phenotypes: •
pancreas, biliary, renal ... rheumatoid factor), ± hypocomplementemia ... No necrosis, No vasculitis
Pulmonary Renal Syndromes - OnePager Summary Autoimmune ANCA vasculitis (AAV): GPA (granulomatous with polyangiitis), EGPA (eosinophilic granulomatosis
Autoimmune ANCA vasculitis ... palpable purpura 2/2 vasculitis ... non-specific, ESR usually low ... hematuria, RBC casts, low ... Path: rapidly progressive
Sjogren's Syndrome Overview Epidemiology: • F > M: 9:1 • 5-6th Decades (can be any age) Autoimmune exocrinopathy multisystemic disease
Cytopenia: 2% • Hypocomplementemia ... ) • Cutaneous vasculitis ... cirrhosis: 3%-20% Renal ... Distal (Type I) renal ... : 2% Systemic Vasculitis
SLE (Systemic Lupus Erythematosus) Suspect: Clinical evidence of (fatigue, rash, photosensitivity, inflammatory arthritis, weight loss, and fever) and
anti-Smith Ab, low ... complements and ... APLA/LAC: • Renal ... , lymphopenia, low ... RNP antibodies, Complement
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