INTRODUCTION: Crohn's Disease (CD) is a granulomatous systemic disorder of unknown etiology. We report a case of constrictive bronchiolitis in addition to interstitial granulomatous lung disease in a patient with CD.
CASE PRESENTATION: This is a 53-year-old Caucasian woman who initially presented with abdominal pain, diarrhea, nausea and vomiting. Diagnosed with CD in 1987 by colonoscopy and biopsy, she was treated with prednisone for 6 weeks and sulfasalazine for one year with remission. Three years later, she received Mesalamine for 4 months and had been clinically stable since then except for gastroesophageal reflux disease for which she underwent laparoscopic Nissen fundoplication. In 2000, she complained of dyspnea on exertion, which progressed and was symptomatic at rest at the time of presentation in February of 2005. She has a five pack-year history of smoking but quit 30 years ago. She has two dogs at home and owned a parrot (parochete) for a few years but not for the last three. There is no history of grain processing, lumber milling, wood stripping, construction, paper manufacturing or exposure to chemicals. Her current medications include neurontin, Topamax (for migraine), propranolol, valacyclovir for herpes infection. Her family history is positive for CD in one sibling and a cousin. At presentation she denied any joint pains, fever, weight loss, chest pain, cough, hemoptysis, muscle weakness, tenderness, tingling or numbness, skin changes, night sweats or swollen lymph nodes. Her vital signs were stable. Her BMI was 39. She had bilateral inspiratory rales and no cyanosis, clubbing or edema.She had a Forced Expiratory Volume in 1 second (FEV1) of 1.54 liters (52% of predicted), a Forced Vital Capacity (FVC) of 1.72 liters (47% of predicted), an FEV1/FVC of 89.6 (110% of predicted) and diffusion capacity of 24.3% predicted. There was no pulmonary hypertension by echocardiogram. Except for mildly elevated Homocysteine, Antithrombin III and Antinuclear Antibody (ANA), the hypercoagulable and serological work up was negative. Purified Protein Derivative (PPD) skin testing was negative. A computed tomogram of the chest revealed mild basal atelectasis with linear fibrosis and non-specific mediastinal adenopathy. An open lung biopsy was performed. The pathology showed mild interstitial expansion with loosely formed granulomas within the alveolar interstitial spaces and in a bronchocentric and perivascular distribution. In addition, bronchial scarring and features of constrictive bronchiolitis were noted. Special stains for fungus and acid-fast bacilli were negative.
DISCUSSIONS: Pulmonary involvement in UC is rare and is usually characterized by bronchiectasis and obstruction(1). In contrast, CD and its association with granulomatous pneumonitis and tracheo-bronchitis is well described in the literature. The pulmonary manifestations and their relationship to the clinical activity of CD are not well defined. We present a case of chronic interstitial granulomatous pneumonitis with features of constrictive bronchiolitis in the setting of remote personal and strong family history of CD. While sarcoidosis and drug reaction can explain the presence of granulomas, the presence of constriction and the remoteness of drug use to treat CD make them unlikely causes. Furthermore, constrictive bronchiolitis is not a feature of hypersensitivity pneumonitis. The presence of granulomas argues against UC being the underlying disease process.
CONCLUSION: Granulomatous inflammation of the lung is a known association with CD. We present a case where constrictive bronchiolitis was also noted in a patient with otherwise clinically silent disease. Whether this feature is associated with a poorer prognosis or resistance to treatment is unknown.
DISCLOSURE: Vaidehi Kaza, None.