Bronchiolitis Obliterans without Joint Disease. A rare non-articular manifestation of Rheumatoid Arthritis
I.Nadeem, M.P. Kennedy
Department of Respiratory Medicine, Cork University Hospital, Cork, Ireland
Pulmonary involvement with Rheumatoid Arthritis (RA) may present prior to joint disease. Obliterative bronchiolitis (OB) is a rare, obstructive lung condition with progressive narrowing of bronchioles and it can eventually lead to fibrosis of lungs.
We report a case of OB in an 80 years old male who presented with progressive dyspnea of six months duration. He had been cycling six months before but was unable to do so and was getting shortness of breath on walking for one mile on a flat floor (MRC Dyspnea Score 2). The patient didn’t have any complains of joint pains, He had background history of hypertension, mild aortic Sclerosis, Hyperlipidaemia and Obstructive Sleep Apnoea (OSA). Medication wise he was on Aspirin 75mg OD, Nebivolol 5mg OD, Valsartan 40mg OD, Atorvastatin 40mg Nocte and CPAP for OSA. He denied cough, sputum production or haemoptysis. His Family history was insignificant.
Clinical Examination yields BP of 130/70, Pulse 72/min, Saturations 93% Room Air and Respiratory Rate of 18/min. His Lung Examination yields Normal Vesicular breathing with no wheeze, crackles and squeaks. His Cardiovascular examination yields Normal Pulse with 3/6 aortic sclerosis murmur and there was no joint deformity or active synovitis.His Echocardiogram showed Normal Ejection Fraction with No Valvular abnormalities and no evidence of Pulmonary Hypertension. Further testing revealed: Progressive obstructive small airways disease with reduction in FEV1 (2.16 (75% predicted) and DLCO (67.5%). Mosaic attenuation on CT Thorax with distal airway thickening. Culture negative BAL with equal lymphocyte (20%) and neutrophil (20%) counts. A VQ scan without evidence of chronic thrombo-embolic disease. Anti CCP antibody (150 au/ml n<5) and IgM RF (500 IU/ml n<10-20).
After discussion at Multidisciplinary Meeting comprising of Respiratory Physicians, Radiologists, Rheumatologists and Cardiothoracic Surgeons, the consensus was not to do Lung Biopsy as it’s a risky procedure and we have sufficient evidence in terms of Radiology, Lung Function Testing, Bronchoscopy and Biochemical testing to make a diagnosis of OB. This approach of making a diagnosis of OB without doing Lung Biopsy has also been documented before1. The patient was started on high dose steroids (1mg/Kg for one month and then slow tapering over 1 year) and macrolide therapy. Patient’s symptoms, lung functions (FEV1 (2.31 (83% predicted) & DLCO (72.4%) and CT Thorax improved after only three months of treatment.
OB in male population has been reported in the setting of a high RF with no joint disease only once before 2 but to our knowledge it has never been reported in Caucasian Population.
Dr. Iftikhar Nadeem
Portiuncula University Hospital,
E-mail: [email protected]
Reference 1. Biopsy-Verified Bronchiolitis Obliterans and Other Noninfectious Lung Pathologies after Allogeneic Hematopoietic Stem Cell Transplantation. Uhlving, H.H, Andersen Claus, Christensen Ib, Gormsen. Magdalena, Pedersen Karen, Buchvald Frederik, Heilmann Carsten, Nielsen Kim, Mortensen Jann, Moser Claus, Sengelov Henrik, Muller. Klaus, 2015; 21(3): 531-538 2. Bronchiolitis obliterans: the lone manifestation of rheumatoid arthritis? Schwarz MI, Lynch DA, Tuder R. Eur Respir J. 1994;7(4): 817.