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pulmonary-renal syndrome
Etiology:
1) Wegener's granulomatosis
2) anti-glomerular basement membrane disease (anti-GBM disease)
3) immune-complex disease (cryoglobulinemia, endocarditis)
Clinical manifestations:
1) cough, hemoptysis, pulmonary hemorrhage, alveolar hemorrhage
2) glomerulonephritis
3) sinusitis, nasal congestion, epistaxis, nasal ulcers (Wegener's granulomatosis)
2) arthralgias mylagias, fever, serositis skin rashes (SLE)
3) mononeuritis multiplex, cranial nerve palsy
Laboratory:
1) complete blood count (CBC):
- anemia, leukopenia, thrombocytopenia (SLE)
1) urinalysis: hematuria, RBC casts (glomerular disease)
2) anti-neutrophil cytoplasmic antibody (ANCA) (Wegener's granulomatosis)
3) anti-GBM antibody (anti-GBM disease)
4) anti-dsDNA, anti-Sm antibody (SLE)
5) serum cyroglobulins (immune complex disease cryoglobulinemia)
6) blood cultures immune complex disease (endocarditis)
Special laboratory:
1) echocardiogram (endocarditis)
2) pulmonary function testing
Radiology:
1) chest X-ray:
- pulmonary infiltrates, pulmonary nodules (Wegener's granulomatosis)
2) renal ultrasound is normal in glomerulonephritis
Management:
1) anti-GBM disease
- plasmapheresis, corticosteroids, cyclophosphamide as indicated
2) Wegener's granulomatosis, SLE:
- corticosteroids, cyclophosphamide as indicated
3) see specific etiology
Specific
anti-glomerular basement membrane [GBM] disease
cryoglobulinemia (cryoglobulinemic vasculitis)
endocarditis
granulomatosis with polyangiitis; Wegener's granulomatosis
General
syndrome
kidney disease; renal disease
lung disease
References
- Medical Knowledge Self Assessment Program (MKSAP) 14,
American College of Physicians, Philadelphia 2006