SESSION TITLE: Interstitial Lung Disease Cases II
SESSION TYPE: Affiliate Case Report Slide
PRESENTED ON: Wednesday, October 29, 2014 at 11:00 AM - 12:15 PM
INTRODUCTION: Pulmonary nodules and cavitary lesions are known pulmonary manifestation of rheumatoid arthritis (RA). While these are well-described findings, we present a patient who had a unique and rare pulmonary manifestation of RA with rapidly growing thin-walled cavities accompanied by pneumothorax and a bronchopleural (BP) fistula.1
CASE PRESENTATION: A 51 year-old female with a history of ductal cell carcinoma of the breast and seropositive RA (treated with methotrexate and prednisone) presented with 1 month of dyspnea and cough. She had known RA pulmonary nodules and cavitary lesions found previous to her diagnosis of RA, and had undegone bronchoscopy and later computerized tomography (CT)-guided biopsy. The pathology was consistent with necrotizing granulomas, and stains for microorganisms and CD1a (a Langerhans cells marker) were negative. On her current presentation a chest x-ray showed a left-sided pneumothorax, multiple non-calcified nodules and thin-walled cavities. The patient underwent immediate chest tube insertion, but given a persistent air leak required open thoracotomy with wedge resection. The pathology showed necrotizing granulomatous inflammation with a thin-walled, cystic cavity. Chronic pleuritis with fibrosis, non-specific interstitial pneumonia-like pattern and lymphoid hyperplasia were also present. Given ongoing symptoms of RA she was initiated on a TNF- inhibitor in hopes to halt further progression of her lung disease.
DISCUSSION: Approximately 40% of patients with RA have extra-articular manifestations of the disease, with pulmonary involvement being a diverse and often devastating consequence. Pulmonary involvement can include pleural pathology, interstitial lung disease, and nodular disease. The nodules and cavitations may enlarge, resolve or recur and are often unrelated to disease course. Our patient was unique given the large, thin-walled cystic lesions on chest imaging accompanied by pneumothorax and BP fistula that are not characteristic of RA-associated nodules.
CONCLUSIONS: Our patient highlights an unusual presentation of the rheumatoid pulmonary disease with rapidly progressing thin-walled cavities. While not a hallmark of RA, pulmonary nodules can also be seen before a diagnosis is made and should always be considered in the differential in an otherwise asymptomatic patient.
Reference #1: 1 Kobayashi T, Satoh K, Ohkawa M, et al. Multiple rheumatoid nodules with rapid thin-walled cavity formation producing pneumothorax. Journal of thoracic imaging 2005; 20:47-49
DISCLOSURE: The following authors have nothing to disclose: Sarah Narotzky, Lynn Fussner, Eva Carmona Porquera
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