Pulmonary renal vasculitis syndromes.
The term pulmonary renal vasculitis syndrome describes a clinical syndrome of diffuse alveolar haemorrhage (DAH) complicating acute glomerulonephritis that often heralds severe, life-threatening systemic vasculitis requiring urgent, aggressive therapy. "Anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis" and glomerular basement membrane ("Goodpasture's") disease are the commonest causes but other pathologies including systemic lupus erythematosus and the anti-phospholipid syndrome are also implicated. Such patients hence present most commonly to rheumatologists and nephrologists but usually require input from a number of specialties, respiratory and critical care medicine in particular. Such care is typically provided in specialist centres able to offer plasma exchange and experience in the use of immunosuppressants. We review clinical features, advances in therapy and understanding of pathophysiology.
Lee
Richard W
RW
Rayne Institute, St Thomas' Hospital, London, UK.
D'Cruz
David P
DP
D000818:Animals; D056648:Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / Q000150:complications / Q000276:immunology* / Q000503:physiopathology / Q000628:therapy; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000276:immunology / Q000378:metabolism*; D016736:Antiphospholipid Syndrome / Q000150:complications / Q000276:immunology* / Q000473:pathology / Q000503:physiopathology / Q000628:therapy; D050533:Glomerular Basement Membrane / Q000276:immunology / Q000473:pathology; D005921:Glomerulonephritis / Q000209:etiology / Q000276:immunology / Q000473:pathology / Q000503:physiopathology / Q000628:therapy; D006470:Hemorrhage / Q000209:etiology / Q000276:immunology / Q000473:pathology / Q000503:physiopathology / Q000628:therapy; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008171:Lung Diseases / Q000209:etiology / Q000276:immunology / Q000473:pathology / Q000503:physiopathology / Q000628:therapy; D008180:Lupus Erythematosus, Systemic / Q000150:complications / Q000276:immunology* / Q000473:pathology / Q000503:physiopathology / Q000628:therapy; D010951:Plasma Exchange
D019268:Antibodies, Antineutrophil Cytoplasmic; D007166:Immunosuppressive Agents