Pleural Effusion in Churg-Strauss Syndrome: Case Report

15 Nov
2014

Pulmonary function tests revealed airflow limitation which improved after inhaled bronchodilator therapy. The chest x-ray film revealed bilateral pleural effusions with patchy basilar infiltrates (Fig 1). Computed tomography of the chest revealed an interstitial process in the right upper lung, right lower lung, and left lower lung, as well as bilateral effusions. A left thoracocentesis with Cope needle pleural biopsy revealed the following: clear yellow fluid; pH, 7.28; LDH, 1714 IU/L; protein, 6.5 g/dL, glucose, 6 mg/dL; cholesterol, 98 mg/dL; ANA, negative; rheumatoid factor, 1:10240; C3, 25.5 mg/dL; WBCs, 4290 cells/μl; eosinophils, 2,394 cells/cu mm; and RBCs, 5250 cells/cu mm.

Pleural fluid cytology was negative for malignant cells. Pleural biopsy showed chronic pleuritis with eosinophilic infiltration. Bron-choalveolar lavage yielded 1.28 x 106 WBC/ml with 89 percent eosinophils, 3 percent lymphocytes, and 7 percent macrophages. Open lung biospy of the right lower lung revealed patchy interstitial and intraalveolar inflammatory infiltrates rich in eosinophils, with prominent cuffs of inflammatory cells about small pulmonary vessels (Fig 2). Subpleural and interlobular connective tissue was heavily involved with an eosinophil-rich inflammatory infiltrate, and dilated lymphatic channels were present in these structures (Fig 3). There was hypertrophy of bronchiolar musculature, bronchial basement membrane thickening, and reduplication of bronchiolar mucosa with goblet cell hyperplasia. Occasional arteries contained segmental or circumferential transmural inflammatory infiltrates with associated necrosis of a component of the vessel wall; mural macrophage giant cells were often present in these lesions (Fig 4). These histologic findings confirmed the clinical diagnosis of Churg-Strauss syndrome. Prednisone, 60 mg/day was started, and within two weeks, the asthma symptoms, eosinophilia, and roentgeno-graphic abnormalities resolved. The patient has been followed for ten months on tapering doses of prednisone without recurrence of his symptoms. itat on

Figure 1. Chest x-ray film showing bilateral pleural effusions, right greater than the left, with patchy basilar infiltrates.

Figure 1. Chest x-ray film showing bilateral pleural effusions, right greater than the left, with patchy basilar infiltrates.

Figure 2. Open lung biopsy demonstrating eosinophilic pneumonia with interstitial and intraalveolar inflammatory infiltrates and prominent perivascular cuffing (hematoxylin-eosin, original magnification, x50). Insert: Bilobed eosinophils predominate in both air space and interstitial infiltrates (hematoxylin-eosin, original magnification x 100).

Figure 2. Open lung biopsy demonstrating eosinophilic pneumonia with interstitial and intraalveolar inflammatory infiltrates and prominent perivascular cuffing (hematoxylin-eosin, original magnification, x50). Insert: Bilobed eosinophils predominate in both air space and interstitial infiltrates (hematoxylin-eosin, original magnification x 100).

Figure 3. Highly cellular eosinophil-rich inflammation of subpleural and interlobular connective tissue is accompanied by lymphatic lumenal dilatation (arrows) (hematoxylin-eosin, original magnification x 10).

Figure 3. Highly cellular eosinophil-rich inflammation of subpleural and interlobular connective tissue is accompanied by lymphatic lumenal dilatation (arrows) (hematoxylin-eosin, original magnification x 10).

Figure 4. Necrotizing vasculitis is evident in this artery by a segmental transmural inflammatory infiltrate (arrowhead) containing a mural macrophage giant cell (hematoxylin-eosin, original magnification x25).

Figure 4. Necrotizing vasculitis is evident in this artery by a segmental transmural inflammatory infiltrate (arrowhead) containing a mural macrophage giant cell (hematoxylin-eosin, original magnification x25).

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