Immunosuppressant-Responsive Idiopathic Lymphocytic Pleuritis

Abstract

Lymphocyte-dominant pleural inflammation is seen in tuberculous, malignant and immune-mediated pleural disease. We describe two male patients who presented with chest pain and dyspnoea, in whom radiological and histological investigations showed idiopathic lymphocytic pleuritis that responded to immunosuppressive therapy with corticosteroids and/or azathioprine. Corticosteroid-responsive pleuritis has been described in association with many conditions, but little is known of idiopathic lymphocytic pleuritis. The authors suggest that patients with idiopathic pleural thickening undergo early thoracoscopic pleural biopsy, and that immunosuppressant therapy be considered for patients with idiopathic lymphocytic pleuritis.

Idiopathic Lymphocytic Pleuritis Responding to Corticosteroid Therapy

“…In two previous case reports, the histopathological features of ILP were consistent with the diffuse lymphocytic infiltration observed in the pleural tissue ( 4 , 5 ). There is a benign type of pleuritis called fibrous pleuritis that requires differentiation from malignant pleural mesothelioma ( 6 , 7 ).…”

“…First, the present case is inconsistent with the results of previous reports, as the pleural thickening was not extensive, but the pleural effusion was. Previous reports emphasized that pleural thickening improved the course of treatment ( 4 , 5 ), whereas our case showed that the pleural effusion decreased after steroid therapy. Second, our patient had PPFE-like findings.…”

“…Regarding pleural effusion that is undiagnosed by thoracoscopy, most cases are believed to have a benign prognosis and are self-limiting. However, previous case reports have described cases of undiagnosed lymphocytic pleural effusion that presented with high disease activity and required steroid therapy ( 4 , 5 ). In these reports, the authors describe several clinical features of ILP, including subacute-to-chronic pleuritic symptoms (chest pain or dyspnea, etc.)…”

“…Among undiagnosed cases of lymphocytic pleural effusion, a disease accompanied by highly inflammatory and exhaustive pleuritis that responds to immunosuppressive therapy has been reported, called idiopathic lymphocytic pleuritis (ILP) ( 4 ). Although it is considered clinically important because it requires appropriate therapeutic intervention, there have only been a few reports of the disease, and its pathology and treatment remain unclear.…”