11 results
diagnosis rheumatology differential management huv igg4 renal urticarial champs classification disease gammopathy glomerulonephritis hematology low mgrs mimics mnemonic monoclonal pathophysiology
Diagnosis and Management of Hypocomplementemic Urticarial Vasculitis (HUV) Diagnosis of HUV 1) Chronic urticaria 2) Leukocytoclastic vasculitis 3)
Management of Hypocomplementemic ... Urticarial Vasculitis ... Leukocytoclastic 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 ... 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 ... @AnnKumfer #Hypocomplementemia ... #Low #Complement
Vasculitis and Vasculitides - Differential Diagnosis Framework When to Consider Vasculitis: • Purpura, ischemic skin lesions •
Vasculitis and Vasculitides ... proteinuria, rapidly progressing ... often involved • Hypocomplementemic ... Urticaria and hypocomplementemia ... against MPO • Complement
Rapidly Progressive Glomerulonephritis (RPGN) RPGN has three primary pathophysiologic causes differentiated by immunofluorescence Immune Complex Mediated (granular staining):
Rapidly Progressive ... staining): • Low Complement ... RPGN #Rapidly #Progressive ... Glomerulonephritis #Vasculitis ... differential #diagnosis #algorithm
Vasculitis - Differential Diagnosis Framework When to Consider Vasculitis: • Purpura, ischemic skin lesions • Mononeuritis multiplex • Hematuria, proteinuria,
proteinuria, Rapidly progressing ... is 50 years • Progressive ... immune GN (rapidly progressive ... Elevated AST ALT • Hypocomplementemia ... ) • Hypocomplementemic
Pulmonary Renal Syndromes and Mimics - Differential Diagnosis Framework Diffuse alveolar hemorrhage + rapidly progressive glomerulonephritis 90%: -
hemorrhage + rapidly progressive ... ANCA Associated Vasculitis ... anti-GBM) 10%: - Low Complement ... - Normal Complement ... Renal Crisis, IgA Vasculitis
IgG4-Related Disease Clinical history: Man, 60 years, multiorgan involvement, unique or multiple inflammatory pseudotumor 4 phenotypes: •
rheumatoid factor), ± hypocomplementemia ... No necrosis, No vasculitis
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 ... : 2% Systemic Vasculitis
IGG-4 RELATED DISEASE WHAT? • A chronic, immune-mediated fibroinflammatory disease with tumefactive infiltration of IgG4+ plasma cells
inflammation -> progressive ... aortitis, mimics vasculitis ... - ↑ IgE, ↓ complement
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