Obliterative bronchiolitis (OB) is a potentially fatal complication of rheumatoid arthritis (RA), which is challenging in terms of both diagnosis and management. We report the case of a 53-year-old woman with severe RA-associated OB who was successfully treated with double lung transplantation. This patient had suffered from erosive, anti-cyclic citrullinated peptide antibody and rheumatoid factor-positive RA for 30 years. She had been prescribed penicillamine between 1992 and 1994, and stopped smoking 20 years ago. She had been on etanercept monotherapy since 8 years, in addition to 5–15 mg of prednisolone. Her RA was considered stable. In 2015, she presented at the Clinic of Respiratory Medicine with a history of wheezing, cough, and dyspnoea on exertion, which had progressed over a 2 year period. Pulmonary function tests (PFTs) revealed a non-reversible obstructive disease [forced expiratory volume in 1 s (FEV1)= 1.1 L and forced vital capacity (FVC)= 2.3 L](Figure 1). Chest radiography findings were normal. High-resolution computed tomography (HRCT) showed a mosaic pattern with areas of decreased attenuation containing pulmonary vessels that were reduced in calibre owing to hypoxic vasoconstriction and bronchial wall thickening, in keeping with small airway disease and obstruction (1). There were no signs of interstitial lung disease (ILD), and no radiological, laboratory or clinical signs of infection. The patient was diagnosed with RA-OB. Etanercept was terminated and she was prescribed prednisolone 30 mg daily together with azithromycin 250 mg three times daily. Follow-up PFTs indicated progressive disease with no effect of the pharmaceutical interventions. After tapering the prednisolone, her RA flared and she was prescribed azathioprine together with rituximab 1000 mg, which was given once. In the following months, her lung function deteriorated further (Figure 1).

Eventually, she suffered from a pulmonary aspergillus infection from which she only partly recovered, exhibiting permanent dyspnoea at rest. Follow-up HRCT showed progression of RA-OB. She was then referred to the Lung Transplantation Unit. After evaluation, she was put on the waiting list and, shortly thereafter, underwent an operation with sequential bilateral lung transplantation via clam-shell incision without the use of assisting haemodynamic support (extracorporeal circulation/extracorporeal membrane oxygenation). The