Sulfasalazine pulmonary toxicity in ulcerative colitis mimicking clinical features of Wegener's granulomatosis

The centrally accentuated antineutrophil cytoplasmic antibody test (c-ANCA) is widely regarded as a sensitive and specific marker for Wegener's granulomatosis (WG). There are increasing reports, however, of false-positive c-ANCAs, usually in the setting of other vasculidities. We report a case of a 27-year-old man with ulcerative colitis who developed pulmonary symptoms, peripheral nodular lung infiltrates, and an elevated c-ANCA suggesting WG. Chest CT and open lung biopsy specimens were consistent with WG. The symptoms and pulmonary infiltrates resolved after discontinuation of sulfasalazine therapy. The c-ANCA remained elevated due to the occurrence of false-positive values in ulcerative colitis. We conclude sulfasalazine toxicity can mimic clinical aspects of WG and that c-ANCA testing should be interpreted with caution in patients with ulcerative colitis.