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diagnosis rheumatology differential management huv renal urticarial classification disease gammopathy glomerulonephritis mgrs mimics monoclonal myelitis neurology pathophysiology phenotypes pulmonary rapidly
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 ... #Urticarial #Vasculitis
Pathophysiology of Hypocomplementemic Urticarial Vasculitis C1q/anti-C1q antibiody -> Immune Complex Formation - Complement Pathway Activation (C3a and C5a)
Pathophysiology of Hypocomplementemic ... Urticarial Vasculitis ... Formation -> Vasculitis ... TerrierBen #Hypocomplementemic ... #Urticarial #Vasculitis
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
Vasculitis - Differential Diagnosis Framework When to Consider Vasculitis: • Purpura, ischemic skin lesions • Mononeuritis multiplex • Hematuria, proteinuria,
Vasculitis - Differential ... proteinuria, Rapidly progressing ... is 50 years • Progressive ... Elevated AST ALT • Hypocomplementemia ... ) • Hypocomplementemic
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
Pulmonary Renal Syndromes and Mimics - Differential Diagnosis Framework Diffuse alveolar hemorrhage + rapidly progressive glomerulonephritis 90%: -
hemorrhage + rapidly progressive ... ANCA Associated Vasculitis ... Syndrome, Rheumatoid Vasculitis ... - Normal Complement ... Renal Crisis, IgA Vasculitis
IgG4-Related Disease Clinical history: Man, 60 years, multiorgan involvement, unique or multiple inflammatory pseudotumor 4 phenotypes: •
IgG4-Related Disease ... node), high serum IgG4 ... rheumatoid factor), ± hypocomplementemia ... No necrosis, No vasculitis ... with increased IgG4
IGG-4 RELATED DISEASE WHAT? • A chronic, immune-mediated fibroinflammatory disease with tumefactive infiltration of IgG4+ plasma cells
IGG-4 RELATED DISEASE ... infiltration of IgG4 ... inflammation -> progressive ... aortitis, mimics vasculitis ... - ↑ IgE, ↓ complement
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
Transverse Myelitis Overview Focal inflammatory disorder of the spinal cord resulting in rapid onset of weakness, sensory
index • Progression ... - Oligoclonal IgG ... levels • NMO-IgG ... negative, MOG-IgG ... - Sjogren - Vasculitis
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