Sulfasalazine Pulmonary Toxicity in Ulcerative Colitis Mimicking Clinical Features of Wegener's Granulomatosis

doi:10.1378/chest.110.2.556

Chest

Volume 110, Issue 2, August 1996, Pages 556-559

https://doi.org/10.1378/chest.110.2.556 Get rights and content

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.

Section snippets

CASE REPORT

A 27-year-old man had an 18-month history of ulcerative proctosigmoiditis. He had taken sulfasalazine, 1,000 mg po bid, since diagnosis with no disease activity until flares at 3 and 2 months before onset of pulmonary symptoms. Both ulcerative colitis exacerbations were responsive to hydrocortisone enemas and oral prednisone therapy. Two weeks before onset of pulmonary symptoms, treatment with his second prednisone burst was tapered off, and the sulfasalazine dose was increased to 1,000 mg po

DISCUSSION

The present report describes the mimicry of pulmonary aspects of WG by sulfasalazine lung toxicity. We also describe the first report (to our knowledge) of a false-positive c-ANCA in a patient with UC and pulmonary disease.

Traditionally, open lung biopsy has served as the gold standard to diagnose WG. However, in recent years, some authors have suggested that a positive ANCA test result could replace open lung biopsy in patients with no infectious etiology and characteristic manifestations of

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Numerous drugs can cause pulmonary reactions in children. Chemotherapeutic agents are most frequently implicated, although toxic effects of other medications have been recognized. Reactions to chemotherapeutic agents may be dose-related; most reactions to noncytotoxic agents develop idiosyncratically. Clinical/pathologic syndromes described include diffuse interstitial pneumonitis and fibrosis, hypersensitivity pneumonitis, noncardiogenic pulmonary edema, pleural effusion, bronchiolitis obliterans, and alveolar hemorrhage. Presenting symptoms vary but often include fever, cough, and dyspnea, and radiologic studies demonstrate diffuse alveolar and/or interstitial abnormalities. Abnormalities in lung function may precede symptoms and radiographic changes. Patterns of abnormalities on chest CT, bronchoalveolar fluid analysis and cultures, and lung histopathology may be useful in categorizing and defining extent of disease but may be nonspecific. Other disorders, including infection, hemorrhage, lung disease related to underlying disease, and radiation damage, must be considered. Criteria for the diagnosis of drug-induced lung disease have been outlined. Although some drug-induced lung injury is reversible, persistent and even fatal dysfunction may occur. Survivors of childhood cancer require follow-up as lung disease may develop remotely from drug exposure and be progressive. More precise information about risk factors, including genetic predisposition and mechanisms of injury, along with more sensitive diagnostic and monitoring approaches are needed for development of improved therapeutic strategies, including early intervention.
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Citation Excerpt :
Patients commonly present with dyspnea on exertion, fever, chest pain, and cough [2, 3, 4]. Patients' laboratory findings may include eosinophilia and a positive perinuclear staining anti-neutrophilic cytoplasmic antibody (positive p-ANCA typically found in patients with ulcerative colitis) [2, 5]. The sedimentation rate was elevated in our case, but it can be normal [3].
We present the case of 16-year-old woman with a 2-month history of ulcerative colitis who presented with cough, fever, dyspnea on exertion, and nasal congestion. Computed tomography of the chest demonstrated peripheral alveolar opacities with relative sparing of the central portions of the lungs. The clinical and radiologic findings raised the suspicion of eosinophilic pneumonia, possibly drug-related. The patient had recently been started on a trial of Mesalamine (5-aminosalicylic acid or 5-ASA) for treatment of her ulcerative colitis 2 months ago. The patient's condition improved after discontinuation of mesalamine and treatment with prednisone. The clinical course and radiologic features supported the presumptive diagnosis of Mesalamine-induced eosinophilic pneumonia.
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: The opinions or assertations contained herein are the private views of the authors and are not to be construed as official or as reflecting the views of the Department of the Army or the Department or Defense

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Selected ReportsSulfasalazine Pulmonary Toxicity in Ulcerative Colitis Mimicking Clinical Features of Wegener's Granulomatosis

Section snippets

CASE REPORT

DISCUSSION

Mayo Clin Proc

Anticytoplasmic autoantibodies: their immunodiagnostic value in Wegener granulomatosis

Ann Intern Med

Wegener granulomatosis: an analysis of 158 patients

Ann Intern Med

The clinical and pathological spectrum of antineutrophil cytoplasmic autoantibody-related pulmonary disease

Arch Pathol Lab Med.

Use of antineutrophil cytoplasmic autoantibodies in diagnosing vasculitis in a Chinese patient population

Am J Nephrol

Selected Reports
Sulfasalazine Pulmonary Toxicity in Ulcerative Colitis Mimicking Clinical Features of Wegener's Granulomatosis