PULMONARY VASCULITIS

Abstract

Case: A 26year old Caucasian female, never smoker, presented to an outside medical facility with right-sided pleuritic chest pain. She denied any other systemic complaints. She was not taking any medications. Chest radiograph followed by CT identified multiple bilateral ill-defined pulmonary nodules and infiltrates. Her past medical history was remarkable for a biopsy proven diagnosis of UC almost a year prior to this presentation. It was treated with sulfasalazine for initial six months and was in remission. Apart from a high sedimentation rate of 42 mm/hr & C-reactive protein of 28.2 mg/dl, her laboratory evaluation was normal including urine analysis, CBC, hypersensitivity panel, p-ANCA, c-ANCA, infectious etiology workup including PPD, fungal & HIV serologies & serologies for connective tissue diseases. CT guided biopsy of a nodule was suspicious for non-small cell carcinoma. Her cancer screening including mammogram, Pap smear, colonoscopy, EGD & CT scan of abdomen and pelvis were unremarkable. Thorascopic lung biopsy was done and she was referred to us. Her physical examination was normal. Repeat tests revealed positive p-ANCA & antibody to proteinase 3 at 154.7 EU/ml (NL <5) but negative antibody to myeloperoxidase & c-ANCA. Her initial colon biopsy was reviewed again at our institute and the diagnosis of UC was confirmed. Her pulmonary pathology showed necrotizing granulomatous vasculitis. She was started on tapering dose of corticosteroid. At three month follow- up, she remained asymptomatic and the pulmonary nodules had resolved on subsequent chest radiograph. Discussion: Extraintestinal manifestations of UC are common, however, pulmonary complications of UC are very rare. The etiology of pulmonary vasculitis is unknown and it appears to be unrelated to the underlying UC activity. Most of the patients described in seven case reports so far, presented with cough, pleuritic chest pain and dyspnea on exertion with evidence of bilateral infiltrates or nodular densities on chest radiograph. The significance of ANCA in the pathogenesis of the IBD remains unclear. Lung biopsy is essential to make the diagnosis. Conclusion: Pulmonary vasculitis is a rare complication of UC, but emphasizes the systemic nature of the disease. This case reiterates the fact that pulmonary manifestations of this entity can occur in the absence of overt bowel inflammation. Corticosteroid therapy appears to be the mainstay in the management of this condition.