Cardiac involvement in granulomatosis with polyangiitis (Wegener granulomatosis).

A 38-year-old man was admitted to the emergency department with a 12-hour onset of shortness of breath and position-related chest pain. In the last 3 months, he had had 2 similar but less intense episodes, each of which lasted for approximately 1 week. Both episodes were associated with subfebrility, flulike symptoms (dry cough, nasal obstruction), and increased fatigue and resolved with nonsteroidal antiinflammatory medication. The patient was diagnosed 2 years earlier with limited Wegener granulomatosis with upper and lower respiratory tract involvement (positive for antineutrophil cytoplasmic antibodies [anti-proteinase 3], a positive sinus ethmoidalis biopsy, and no renal involvement) and was undergoing immunosuppressive therapy (azathioprine and methylprednisolone). On admission, the patient was stable and afebrile and tolerated decubitus. The clinical examination was unremarkable, with no respiratory or cardiac pathological findings on auscultation. The chest radiograph showed a normal cardiac silhouette, clear lungs, and a small left pleural effusion. The ECG performed on admission showed sinus rhythm, normal PR interval, and diffuse flattened T waves. Initial blood tests showed mild anemia (hemoglobin 12.2 mg/dL) and marked inflammation, with an elevated erythrocyte sedimentation rate (86 mm/h) and C-reactive protein levels (306 mg/L). Troponin I on admission was within normal limits (0.02 g/L). Given the clinical picture (nasal obstruction) and the laboratory results (high C-reactive protein and an increase in antiproteinase 3 compared with 2 months earlier, from 24 to 128 U/mL), there was a strong suspicion of relapsing disease. Transthoracic echocardiography showed a thickened pericardium with a circumferential, homogeneous pericardial effusion (up to 14 mm along the left ventricular [LV] lateral wall; Figure 1; online-only Data Supplement online-only Data Supplement Movie I) without a filling impairment (mitral valve: E inspiration 75 cm/s, E expiration 97 cm/s, 23% variation, E wave deceleration time 166 ms; Figure 2) and nondilated left and right chambers with normal biventricular systolic function. No valve abnormalities, pulmonary hypertension, or arguments for endocarditis were found. Thoracic computed tomography with contrast showed mild cardiomegaly, discrete left posterobasal pleural effusion, and thickened pericardium without calcifications (Figure 3). No lung infiltrates or adenopathies were present. For further characterization of the pericardial involvement and to exclude the presence of myocardial granulomatous infiltrates, cardiac magnetic resonance imaging was performed. Functional analysis by steady state free-precession cine sequences showed mildly dilated left and right ventricles (end-diastolic LV volume 124 mL/m [normal values 79 8.7 mL/m], right ventricular volume 109 mL/m [normal values 88 11.7 mL/m]), with mildly impaired LV systolic function (LV ejection fraction 50%) and normal right ventricular systolic function (online-only Data Supplement Movie II).