Shivraj J Desai, M.D.;([unkeyable])...

Shivraj J Desai, M.D.;([unkeyable]) Gordon N Gephardt, MD F.C.C.P.([unkeyable]) and James K rover M.D., F.C.C.P. Section

To expand the clinical image of ulcerative colitis-associated lung disease, we describe a patient with panbronchiolitis associated with ulcerative colitis. In addition to his having a distinctive pulmonary manifestation of this bowel disease, other noteworthy aspects of this patient's course include the attack of pulmonary symptoms several years prior to bowel manifestations and the partial resolution of his pulmonary symptoms following colectomy.

(Chest 1989;45:1342-44)

Extraintestinal manifestations of ulcerative colitis are frequent occurring in up to 45 percent of patients, and include uveitis, arthritis, skin lesions, and liver disease.[1] In contrast, pulmonary involvement in ulcerative colitis is highly uncommon, having been reported in no other than three of 1,400 patients (021 percent) with inflammatory bowel disease.[2] However, a variety of bronchopulmonary disorders has been associated with ulcerative colitis, including bronchiectasis, pleural effusions, apical fibrosis, and pulmonary vasculitis.[3-22] When existing bronchopulmonary manifestations of ulcerative colitis usually tread close upon the onset of intestinal symptoms and may actually current many years after proctocolectomy for ulcerative colitis.

To expand the clinical representation of ulcerative colitis-associated lung disease, we describe a patient with panbronchiolitis associated with ulcerative colitis whose lung disease preced the first brunt of bowel symptoms by five years. The sum of two units distinctive aspects of this case include the association between panbronchiolitis and ulcerative colitis, as well as the storm of pulmonary symptoms prior to bowel manifestations.

CASE REPORT

A 25-year-old man, a nonsmoker, was first admitted to the Cleveland Clinic Hospital in July 1987 for a total proctocolectomy, following three years of persisting abdominal cramps and sanguinary diarrhea refractory to therapy with high doses of prednisone and azulfidine. Previous colonoscopic biopsy had shown ulcerative colitis. An initially unexplained pulmonary disease had begun in 1979 five years prior to his initial intestinal complaints. Pulmonary symptoms consisted of congestion, dyspnea, and cough productive of copious feculent sputum. Early evaluation of his pulmonary symptoms included a nondiagnostic bronchoscopy and initial empiric therapy with prednisone controll his symptoms completely although reduction in the prednisone dose below 10 mg/day issueed in a relapse of cough sluggishness and dyspnea.

He was referr to the Mayo Clinic in August 1982 for initial pulmonary evaluation, when physical examination springs revealed diffuse wheezing and rales.

rises of pulmonary function tests (Table 1) showed moderate airway obstruction, a significant replication (20.4 percent rise in [FEVsub1]) to inhaled salbutamol administration, and air trapping. The chest roentgenogram revealed patchy infiltrates in one as well as the other lungs (Fig 1). Bronchoscopy was performed and showed evidence of bronchitis bilaterally. Bilateral bronchograms showed no evidence of bronchiectasis, and a Gram stain of the sputum showed many white enclosed spaces but no organisms. The sputum cytologic research was negative for malignant confined apartments and acid-fast smears were negative. An intermediate-strength PPD produc no induration. Because of uncertainty about the cause of the pulmonary infiltrates, an render free of access lung biopsy was performed in November 1982 Stains for Pneumocystis carinii, mycobacterial, and fungal organisms were negative, and civilizations of the biopsy specimens grew no organisms. Histologic examination showed diffuse panbronchiolitis (Fig 2) without granulomata. The inflammatory infiltrate in the alveolar walls (Fig 3) consisted of small lymphocyte and plasma small cavitys This histologic picture of panbronchiolitis conformed with the criteria outlined at Homma et al.[23] [TABULAR DATA OMITTED]

The patient was discharged receiving daily bronchodilators and prednisone therapy and did well until late 1984 (60 month after his initial presentation), when he noted the attack of his intestinal symptoms.

Prior to proctocolectomy, attempts to render his daily prednisone below 10 mg/day eventuateed in return of both intestinal and pulmonary symptoms. However, for four month after protocolectomy, he remained stable without corticosteroid therapy (both inhaled and systemic) and was at liberty of pulmonary symptoms. Beginning in the fifth month after protocolectomy, slight cough and wheezing go [i]or[/i] come backed although his chest roentgenogram remained normal. Corticosteroid therapy was reinstituted with religious control of his pulmonary symptoms. [TABULAR DATA OMITTED]

DISCUSSION

Although the image of pulmonary abnormalities associated with inflammatory bowel disease is broad, associated lung disease is unusual. For example, in reviewing the extraintestinal manifestations of inflammatory bowel disease in 700 patients with either Crohn's disease or ulcerative colitis, Greenstein and colleagues[1] recognized no patients with bowel-associated lung disease. Roger et al[2] cited a similarly subdued prevalence (0.21 percent).