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Giant cell arteritis usually presents as a typical clinical syndrome (temporal arteritis or polymyalgia rheumatica), but may also have unusual localization. We report a 69-year-old woman who presented with malaise and progressive dyspnea with chest pain on inspiration for 1 week. Eighteen months earlier, a biopsy-negative diagnosis of giant cell arteritis had been made based on the classic symptoms of temporal headache, neck and shoulder pain, jaw claudication, and hip stiffness. Treatment with prednisolone resulted in rapid resolution of symptoms. Twelve months later, she had an infection of both hip prostheses, complicated by sepsis due to Escherichia coli, and both prostheses were removed. Prednisolone was stopped, and maintenance treatment with oral ciprofloxacin was prescribed.
On presentation, the physical examination revealed a body temperature of 37.4°C, painful neck and shoulder region, and crackles over the left lung. Laboratory investigation showed normocytic anemia (hemoglobin, 9.0 g/dL) and a markedly increased erythrocyte sedimentation rate (>120 mm/h) and C-reactive protein level (27.7 mg/dL). Chest radiography showed bilateral convex heart enlargement (Figure), pleural effusion, and a pulmonary infiltrate. Pulmonary emboli were unlikely after a normal perfusion scan. Under the assumption of atypical pneumonia, oral clarithromycin was added, but was ineffective. Echocardiography showed 30 mm of circular pericardial effusion, and 1300 mL of exudate was aspirated (leukocyte count, 2.4 × 103/μL). Pleural puncture revealed similar findings. Microbiological cultures remained sterile, including negative results for tuberculosis by polymerase chain reaction.
The most likely diagnosis was pleuropericarditis due to relapsed giant cell arteritis. Prednisolone was restarted at 60 mg daily, which resulted in rapid clinical improvement and normalization of all laboratory parameters. Prednisolone was gradually tapered. Echocardiography and chest radiography 7 months later confirmed resolution of both pericardial and pleural effusion.