Pulmonary Renal Syndrome
Definition: Diffuse alveolar hemorrhage + Glomerulonephritis
Presentation (Pulmonary and renal manifestations can occur weeks apart): Cough, Dyspnea, Fever, Hematuria, Hemoptysis, Peripheral edema, Disease-specific symptomatology
Differential Diagnosis for Pulmonary Renal Syndromes:
• Pauci-Immune:
- ANCA-positive vasculitis (Eosinophilic granulomatosis with polyangiitis, Granulomatosis with polyangiitis, Microscopic polyangiitis)
- ANCA-negative vasculitis (IgA-associated vasculitis, Behcet disease, Cryoglobulinemia)
• Immune Complex: Systemic lupus erythematosus, Rheumatoid arthritis, Systemic sclerosis
• Anti-Glomerular Basement Membrane: Goodpasture's Disease
• Other: Drugs (hydralazine, propylthiouracil, allopurinol), Infection, Alport Syndrome
Investigations
• Labs: CBC, cr, ESR, CRP
• Urinalysis (proteinuria, dysmorphic RBCs)
• Disease-specific testing: anti-ANCA ab, anti-GBM ab, ANA, C3, C4, anti-CCP
• CXR (diffuse infiltrates) → high resolution chest CT
• Bronchoscopy with serial BAL → confirm DAH, rule out infection, biopsy
By Tess Litchman, MD @TessLitchman via @PulmPEEPs
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