Abstract
BackgroundCTD-related pleural effusions are rare and challenging to diagnose. Our lung inflammation service (with expertise in rheumatology, interstitial lung disease and respiratory failure) works closely with the pleural team. This study aims to review the multidisciplinary approach to CTD-related pleural effusions at a tertiary centre.MethodsAll patients with CTD-related pleural effusions at St Thomas’ Hospital, London were included. Retrospective data were collected from Dec 2013 to 2016.ResultsThe lung inflammation service performed an expert clinical assessment and targeted investigations. 11 patients (ages 23–77) were identified with CTD related pleural disease. 9 (82%) patients were given a new CTD diagnosis, with pleural disease as the first manifestation. The range of conditions were: rheumatoid arthritis [3] ,IgG4-related disease [2] ,adult Still’s disease [2] ,vasculitis [1] ,SLE [1] ,drug-induced lupus [1] ,and Behcet’s [1].The pleural team review took place 1 day (median) after referral. 73% of diagnoses (8 patients) were achieved with local anaesthetic pleural interventions (a combination of: aspiration, drain, or percutaneous biopsy). This included 1 patient who required no pleural intervention. 1 required medical thoracoscopy, and 2 underwent thoracic surgery.Diagnoses were made by integrating all available evidence such as clinical assessment, imaging, and autoimmune serology. No diagnosis was achieved by pleural cytology or histology analysis alone.8 (73%) were commenced on prednisolone acutely (vasculitis, SLE, drug-related lupus, 1 patient with rheumatoid arthritis, Behcet’s, 2 patients with Adult Still’s disease, 1 patient with IgG4-related disease). Of these 8, one patient with rheumatoid arthritis received IV methylprednisolone beforehand, one patient with IgG4-related disease was weaned off prednisolone to methothrexate, two patients with Adult Still’s disease were on colchicine as well, and one patient with Behcet’s was on cyclophosphamide as well. 7 (64%) were managed as outpatients; 4 required admission. The median time from pleural review to diagnosis was 53 days.ConclusionsDiagnosis can be challenging in patients presenting with pleural disease as the first manifestation of a CTD. We recommend a multidisciplinary approach in management.
Highlights
connective tissue diseases (CTD)-related pleural effusions are rare and challenging to diagnose
Eleven patients were identified with CTD related pleural disease (Table 1). They were seen by the lung inflammation service, who performed an expert clinical assessment and targeted investigations, usually after review by the pleural team. 9 (82%) patients were given a new CTD diagnosis, with pleural disease as the first manifestation
One patient who underwent thoracic surgery was referred directly to the surgical team, subsequently requiring a pericardiectomy for a diagnosis of IgG4-related disease. Another patient underwent video-assisted thoracoscopic surgery (VATS) to rule out a pleural malignancy, before commencing treatment for a pleural effusion related to drug-induced lupus
Summary
CTD-related pleural effusions are rare and challenging to diagnose. This study aims to review the multidisciplinary approach to CTD-related pleural effusions at a tertiary centre. A new pleural effusion may be caused by a wide range of conditions. A number of these are caused by connective tissue diseases (CTD). Pleural effusions from CTD are caused by increased capillary permeability, as extravascular fluid moves from the lung’s interstitium, across the mesothelium into the pleural space [3]. This may be due to a number of reasons [4], such as a pleural infiltrative process. Enzyme and free radical release from white blood cells accentuate the inflammatory process
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Free) 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call
