Wegener’s granulomatosis (WG) is a disease whose target organs are the upper and lower respiratory tracts and the kidney. Some reports have documented other organ involvement, including the heart. Cardiac involvement in WG is unusual but the most common cardiac manifestations are pericarditis and coronary arteritis.We describe a patient with Wegener’s and no history of heart disease who developed a cardiomyopathy resulting in cardiac failure.
50 y/old white woman who presented with increased shortness of breath, cough and hemoptysis for two weeks. She also had lower extremities edema. WG was diagnosed since 1989 by lung biopsy and she had 3-4 relapse since the diagnosis was made. She has been treated with methotrexate, prednisone, and bactrim(intermittently). Last round of medications include methotrexate 2-3 weeks prior to amission but she was changed to cytoxan 100mg and prednisone 20mg one week prior to admission due to increased dyspnea. Her history was significant for 12years smoking history. No cardiac history. Initial evaluation revealed a patient with mild tachypnea, afebrile. Cardiovascular exam was negative. Bilateral crackles and wheezes were noted. There was marked leg edema up to the thigh and erythematous macules with necrotic center on extremities. The rest of the physical exam was negative. Labs obtained showed leukocytosis and thrombocytopenia. Hgb, coagulation panel, cardiac enzymes and electrolytes were normal. C-ANCA was 1:160. EKG showed sinus tachycardia. Admission blood gas analysis showed PaO2 77mmHg sat 95% on RA. CXR revealed multiple bilateral lung cavitations with cardiomegaly. She had a spiral chest CT scan that showed nodules bilaterally, biventricular chamber enlargement with R atrial distention and evidence of intramural thrombi on R atrium and both ventricular chambers. An echocardiogram showed LV ejection fraction of 25% and thrombus in the left ventricle. On admission thrombophilia work up was obtained revealing only abnormal protein S(45). She received anticoagulation and diuretics improving her dyspnea initially. During the hospital stay her condition deteriorated and she became increasingly short of breath and hypoxic despite of treatment and subsequently she died. The autopsy revealed myocardial hypertrophy and ventricular dilatation, segmental pulmonary emboli, necrosis of pulmonary vascular walls and lung parenchyma, and severe hemorrhage.DISCUSSION: WG involving the heart has been described, ranging from 6 to 44% of cases, but significant cardiac complications during the course of the disease are rare. Forstot et al retrospectively analyzed cases of patients with cardiac involvement and they found about 50% had pericarditis, 50% had coronary arteritis, 25% focal myocarditis and 21% valvulitis or endocarditis, conduction system was involved in 17% and myocardial infarction in 11%. Myocarditis with granulomas had been described producing acute cardiac failure that may progress to cardiomyopathy. To our knowledge, intrinsic heart muscle involvement leading to cardiomyopathy and cardiac failure is very rare in WG and this has rarely been reported. On her autopsy the predominant pathology was found in the cardiopulmonary system. Clearly in the lungs there was vasculitis suggestive of Wegeners. The most evident finding in the heart was the presence of diffuse fibrosis of myocardium without inflammation or arteritis. The most likely explanation for the sequence of events is the combination of multiple factors: cardiomyopathy with poor systolic function and tendency to develop mural thrombi secondary to low flow state and second the possibility of thrombophilic state associated with Protein S deficiency.
This case shows that although the clinical manifestation of cardiac disease in WG is not usually encountered, it should be considered in patients with this clinical presentation. We think that the cardiac condition in this patient could be directly attributable to WG in association with a thrombophilic state.
M. Medina, None.