Ulcerative Colitis and Steroid-Responsive, Diffuse Interstitial Lung Disease

Abstract

We describe a patient with ulcerative colitis and extracolonic manifestations in whom diffuse interstitial pulmonary disease developed that was responsive to glucocorticoid therapy one year after total proctocolectomy. The patient presented in December 1983 with a subacute course marked by cough and progressive exertional dyspnea, abnormal chest examination results, and a chest roentgenogram that revealed diffuse interstitial and alveolar infiltrates. A transbronchial biopsy specimen revealed a polymorphic interstitial infiltrate, mild interstitial fibrosis without apparent intraluminal fibrosis, and no vasculitis, granulomas, or significant eosinophilic infiltration. Within one week of the initiation of daily high-dose steroid therapy, the patient's symptoms dramatically improved; chest roentgenogram and forced vital capacity (60%) improved at a slower rate. All three measures deteriorated when alternate-day prednisone therapy was started but once again improved until the patient was totally asymptomatic, chest roentgenograms were normal, and forced vital capacity was 80% of the predicted value 2 1/2 years later.

Ulcerative colitis combined with acute interstitial lung disease and airway disease: A case report and literature review

“…Only four out of the 24 cases mentioned colectomy, including two cases (2/4, 50%) in which the ILD occurred subsequent to the colon resection (3,18), suggesting that the colectomy may be a stimulus for the occurrence of UC complicated by ILD. The two cases in which the ILD occurred prior to the colectomy were in different situations.…”

“…Among the 24 cases, 10 (11–13) did not report the order of the ILD and UC incidences. In 12 cases (12/14, 85.71%) the ILD occurred 1–15 years after the diagnosis of UC (3,4,7–9,14–18); this included only five cases in which the ILD occurred during active UC (5/12, 41.67%) (3,4,14,15). In two cases it was reported that the ILD occurred prior to the UC (17,19); in one of these cases (19) the ILD occurred immediately subsequent to the UC relapse, indicating that UC activity may be associated with the incidence of ILD.…”

Localized giant inflammatory polyposis of the cecum associated with distal ulcerative colitis

“…BOOP, a kind of interstitial pneumonitis, is rarely associated with UC. [16][17][18][19] There have been four reported cases of UC coexistent with BOOP. [16][17][18][19] In these patients, BOOP occurred at various stages of UC and there was a symptomatic and radiological alleviation of pneumonia and UC with steroid therapy.…”

“…[16][17][18][19] There have been four reported cases of UC coexistent with BOOP. [16][17][18][19] In these patients, BOOP occurred at various stages of UC and there was a symptomatic and radiological alleviation of pneumonia and UC with steroid therapy. One hypothesis is that both the lung and the colon are vulnerable to autoantibodies; both BOOP and UC have been associated with circulating immune complexes, and altered effector-cell function has been described in both disorders.…”

“…One hypothesis is that both the lung and the colon are vulnerable to autoantibodies; both BOOP and UC have been associated with circulating immune complexes, and altered effector-cell function has been described in both disorders. 18 These factors may be related to the reason why the steroid therapy was effective for LGIP.…”