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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