Multidisciplinary Approach Recommended for Diagnosis of Connective Tissue Disease-Related Pleural Effusions

CT scan of the chest of a patient with large loculated pleural effusion in his left thoracic cavity. Diagnostic findings in patient with dyspnea. Medical education.
Researchers of the study reviewed a multidisciplinary approach to connective tissue disease-related pleural effusions at a tertiary center in London.

Connective tissue disease (CTD)-related pleural effusions are best diagnosed and managed using a multidisciplinary approach, according to study results published in BMC Pulmonary Medicine.

Investigators conducted a retrospective study of all patients diagnosed with CTD-related pleural effusions at St Thomas’ Hospital in London between November 2012 and 2016. Clinical information of all eligible patients was extracted, including the type of treatment intervention received, computed tomography diagnosis, symptoms at referral, and pleural laboratory figures.

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A total of 11 patients (age, 23-77 years) with CTD-related pleural disease were seen by the lung inflammation service, among whom 8 (73%) were men; 9 patients (82%) were given a new CTD diagnosis at the clinic, with pleural disease as the first manifestation. The range of observed CTD conditions included rheumatoid arthritis (n=3), immunoglobulin G4 (IgG4)-related disease (n=2), adult Still disease (n=2), vasculitis (n=1), systemic lupus erythematosus (n=1), carbamazepine-induced lupus (n=1), and Behcet disease (n=1).

Median time from referral to review by the pleural team was 1 day; median time from pleural review to diagnosis was 53 days. The majority (n=8; 73%) of diagnoses were achieved through local anesthetic interventions: a combination of aspirations, drains, and percutaneous biopsies. One patient required no pleural intervention, while 1 received a medical thoracoscopy and 2 underwent thoracic surgery. No diagnoses were made from pleural cytology or histology alone, and pleural fluid pH was not measured. Diagnoses were achieved by integrating various data from clinical assessment, imaging, and autoimmune serology.

Prednisolone was started immediately in 8 patients (73%), and among them the following treatment variations were observed: 1 patient with rheumatoid arthritis received intravenous methylprednisolone beforehand; 1 patient with IgG4-related disease was weaned off prednisolone and received methotrexate; 2 patients with adult Still disease received colchicine; and 1 patient with Behcet disease received cyclophosphamide. Seven patients (64%) were managed in the outpatient unit, while 4 (36%) required admission.

These data emphasize the challenges of diagnosing CTD-related pleural disease, however, a multidisciplinary approach integrating all available diagnostic information was most efficient in diagnosis. Further study in a larger cohort is necessary to explore the best means of managing CTD-related pleural disease.


Ip H, Sivakumar P, McDermott EA, et al. Multidisciplinary approach to connective tissue disease (CTD) related pleural effusions: a four-year retrospective evaluation. BMC Pulm Med. 2019;19(1):161.