WEGENERS GRANULOMATOSIS WITH ANTIPROTEINASE-3 ANTIBODIES OCCURRING AFTER HODGKINS-DISEASE

We describe the first association between Hodgkin's lymphoma and Wegen er's granulomatosis, heralded by renal involvement. A 43-year-old man developed rapidly progressive glomerulonephritis requiring chronic hem odialysis 8 months after remission of Hodgkin's lymphoma. At that mome nt, no extrarenal involvement was found, despite extensive investigati on. Antineutrophil cytoplasm antibodies were positive, without specifi city for proteinase-3 or myeloperoxydase. Six months after beginning h emodialysis, multiple pulmonary nodules appeared, along with rapid cli nical worsening. A surgical biopsy was performed which disclosed a gia nt cell granuloma. Antimyeloperoxydase antibodies remained negative, w hereas proteinase-3 antibodies became positive. Wegener's granulomatos is was diagnosed and treatment with cyclophosphamide and steroids was started. Clinical and radiological improvement occurred promptly. Elev en months after treatment, both Wegener's disease and Hodgkin's lympho ma remained in remission.