Myocardial Sarcoidosis Unresponsive to Steroids

Myocardial Sarcoidosis Unresponsive to SteroidsTreatment with Cyclophosphamide
Corticosteroids are the mainstay in the treatment of chronic sarcoidosis. Only recently have immune-alter-ing medications been used, usually in patients who have been shown to have disease unresponsive to steroid therapy. The following case exemplifies this unusual aspect of sarcoidosis.
Case Report
A 37-year-old black woman presented in January, 1979 with shortness of breath and bilateral hilar lymphodenopathy. Sarcoidosis was diagnosed by mediastinoscopy and biopsy. Prednisone was prescribed, but she discontinued its use after two months due to Cushingoid side effects. In August, 1979, she presented with a massive left pleural efiusion. An exhaustive workup revealed only sarcoidosis. Prednisone was started at 30 mg per day. The pleural efiusion initially resolved but gradually recurred. After eight months of steroid treatment consisting of at least 30 mg/day, a massive left and small right pleural efiusion were seen on the chest x-ray film. Cardiomegaly was also seen and an echocardiogram demonstrated gross pericardial efiusion. Therapy with high-dose prednisone (60-100 mg/day) was associated with an immediate reduction in the pleural and pericardial effusions. These findings later recurred and by August, 1980, the physical examination revealed anasarca.

Throughout her course, all biochemical and immunologic parameters for sarcoidosis were either negative or normal. These tests included serum and urine for calcium, urine for hydroxyproline, SACE, immunoglobulin and complement levels, and tests for circulating immune complexes. The PPD result was negative and an anergy battery was positive. All studies of other organ systems were equally unrewarding, with the exception of a gallium scan which showed diffuse uptake in the region of the pulmonary hila and a thallium scan of the heart which was consistent with cardiomyopathy.
A 24-hour Holter monitor revealed one PVC in a 24-hour period. Pericardiectomy was performed due to the features of constrictive pericarditis which was unresponsive to conventional therapy. At surgery, the myocardium was noted to be almost completely covered with white studs believed to be involvement with sarcoidosis (Fig 1). The pericardial tissue revealed granulomatous inflammation consistent with sarcoidosis. She was discharged home in an improved condition on therapy with 40 mg of prednisone per day.
The left pleural efiusion never disappeared completely, and by April, 1981, it had achieved massive proportions which again caused dyspnea. Prednisone therapy was gradually increased, and despite doses up to 100 mg per day, the efiusion persisted. Cyclophosphamide therapy was empirically started in June, 1981 at a dose of 25 mg bid. There was rapid diminution in her dyspnea and fatigue and a sense of well-being returned. Prednisone therapy was gradually tapered and subsequently discontinued. The gallium scan reverted to negative and has remained so. Over the years, attempts were made to reduce the dose of cyclophosphamide to 25 mg per day. On each attempt, her sarcoidosis again became active either clinically (pleuritic chest pain), electrocardiographically (arrhythmias), or radiographically (chest nodules which were biopsied and showed non-caseating granulomas). She is now maintained on 50 mg per day.

Figure 1. Myocardial surface at surgery. Note the whitish areas (as opposed to the flash reflection) indicating gross involvement with sarcoidosis.

Figure 1. Myocardial surface at surgery. Note the whitish areas (as opposed to the flash reflection) indicating gross involvement with sarcoidosis.

Category: Myocardial Sarcoidosis

Tags: cyclophosphamide, myocardial sarcoidosis, steroids