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Systemic Sclerosis Associated Autoantibodies Anti-topoisomerase I (anti-Sc170): Diffuse skin disease, Pulmonary fibrosis Anti-centromere: Limited skin disease (~93-95%), Pulmonary
Autoantibodies Anti-topoisomerase ... I (anti-Sc170): ... skin disease, Pulmonary ... fibrosis Anti-centromere ... disease (~93-95%), Pulmonary
Pulmonary Renal Syndrome Definition: Diffuse alveolar hemorrhage + Glomerulonephritis Presentation (Pulmonary and renal manifestations can occur weeks apart):
Pulmonary Renal ... Presentation (Pulmonary ... Diagnosis for Pulmonary ... ab, anti-GBM ab ... , ANA, C3, C4, anti-CCP
Rapidly Progressive Glomerulonephritis (RPGN) RPGN has three primary pathophysiologic causes differentiated by immunofluorescence Immune Complex Mediated (granular staining):
Henoch-SchonIein Purpura Anti-GBM ... Limited to Kidney: Anti-GBM ... With associated pulmonary ... involvement: Anti-GBM
Pulmonary Renal Syndromes - OnePager Summary Autoimmune ANCA vasculitis (AAV): GPA (granulomatous with polyangiitis), EGPA (eosinophilic granulomatosis
Pulmonary Renal ... polyangiitis) Anti-basement ... Although pulmonary ... ANCA antibody, Anti-GBM ... usually low in anti-GBM
Biologic therapy for eosinophilic asthma • Mepolizumab (Nucala) • Anti-lL-5, 100 mg SQ q4
(Nucala) • Anti-lL ... Cinqair) • Anti-lL ... Fasenra) • Anti-lL ... Dupixent) • Anti-lL ... #pharmacology #pulmonary
Systemic Lupus Erythematosus - Summary Antinuclear (ANA) - 95% - Initial screening test Anti-dsDNA - 50% • Associated
screening test Anti-dsDNA ... disease activity Anti-Ro ... Sjogren syndrome Anti-UI-ribonucleoprotein ... overlap syndrome) Anti-Smith ... Hydroxychloroquine *Short
Pulmonary Renal Syndromes and Mimics - Differential Diagnosis Framework Diffuse alveolar hemorrhage + rapidly progressive glomerulonephritis 90%: -
Pulmonary Renal ... Vasculitis - Anti-GBM ... Positive (ANCA + anti-GBM ... Kumfer @AnnKumfer #Pulmonary
Rapidly Progressive Glomerulonephritis (RPGN) - Loss of kidney function over a short period of tirne
function over a short ... post-infectious GN Anti-Glomerular ... IF • Majority anti-neutrophil ... + • c-ANCA: anti-proteinase ... staining • p-ANCA: anti-myeloperioxidase
Antinuclear antibodies and Systemic lupus Anti-dsDNA 60-80% Association with disease activity (when with Farr assay) and lupus nephritis. Can
Systemic lupus Anti-dsDNA ... when ANA+ without anti-dsDNA ... Anti-histone 60 ... neonatal lupus Anti ... -15% If SLE is clinically
Medications for High-Altitude Sickness Acetazolarnide • Carbonic Anydrase Inhibitor Bicarb diuresis & metabolic Acidosis ->
pressure • Anti-emetic ... • Decreases pulmonary
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