Antineutrophil cytoplasm antibody-positive pulmonary-renal syndrome in a patient with diffuse cutaneous systemic sclerosis. (13th June 2013)

• Record Type: Journal Article
• Authors: Tonneijck, Lennart; Tanna, Anisha; Pusey, Charles D
• Is Part Of: BMJ case reports. Volume 2013
• Publication Date: 2013-06-13
• Subjects: Medicine -- Case studies -- Periodicals; 610.5
• DOI: 10.1136/bcr-2012-008099
• Languages: English
• ISSNs: 1757-790X

Abstract

Abstract : A 72-year-old male patient with known diffuse cutaneous systemic sclerosis (SSc) presented with severe haemoptysis and blood and protein in the urine. In light of his known interstitial lung disease, he had been repeatedly treated for recurrent community-acquired pneumonia. Immunological testing demonstrated a strongly positive perinuclear antineutrophil cytoplasm antibody with a high titre antimyeloperoxidase antibody. The patient was diagnosed with pulmonary-renal syndrome as a consequence of antineutrophil cytoplasm antibody-associated vasculitis. He started immediate plasmapheresis in combination with methylprednisolone, followed by cyclophosphamide and rituximab, with good clinical outcome.