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Mixed Connective Tissue Disease Manifesting as Unilateral Pleural Effusion

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A3054 - Mixed Connective Tissue Disease Manifesting as Unilateral Pleural Effusion
Author Block: A. F. Chaudhry1, M. E. Faquih1, U. L. Obih1, E. Flenaugh2, R. Sachdeva3, M. G. Foreman2; 1Morehouse School of Medicine, Atlanta, GA, United States, 2Pulmonary and Critical Care, Morehouse School of Medicine, Atlanta, GA, United States, 3Cardiology, Morehouse School of Medicine, Atlanta, GA, United States.
Introduction: We present a case of unilateral pleural effusion with mediastinal shift as an initial presentation of mixed connective tissue disease (MCTD) in a patient with respiratory distress.
Case Description: A 25-year-old woman with no significant past medical history presented with respiratory distress. Her breathing gradually worsened over 2 months. She denied fever, chills, chest pain, cough, expectoration, or weight loss. On exam, she had absent breath sounds on the left with dullness to percussion. Chest x-ray showed complete opacification of the left lung with marked rightward cardiomediastinal shift. Non-contrast chest CT confirmed a large left pleural effusion causing near complete collapse of the left lung, with displacement of the heart into the right hemithorax, without pericardial effusion or enlargement of pulmonary arteries. She underwent a therapeutic thoracentesis, with pleural fluid analysis consistent with an exudative process. Pleural fluid cultures yielded no growth. Pleural cytology showed predominance of macrophages mixed with mesothelial cells. Pleural biopsy was consistent with chronic inflammation and was negative for malignancy. Further blood tests revealed positive anti-nuclear antibody, with subtype analysis indicating mixed connective tissue disease.
Discussion: MCTD commonly presents in females 30-39 years of age. Although respiratory manifestations including interstitial lung disease and pulmonary hypertension occur in up to 80% of patients with MCTD, they are not typically present in the early clinical course. Pleural effusion without associated pulmonary parenchymal involvement is an unusual presentation of MCTD, with only 2 cases reported in the literature. Additionally, unilateral pleural effusion as the sole pulmonary manifestation of MCTD, as in our case, has only been reported once before.
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