ORBITAL POLYMYOSITIS AND GIANT-CELL MYOCARDITIS

Background: Orbital polymyositis associated with giant cell myocarditi s rarely has been reported in the literature. The authors report the c linical, neuroradiographic, and histopathologic features of the only p atient to survive this usually fatal syndrome after cardiac transplant ation. Findings: This 22-year-old white woman presented in 1991 with p eriorbital redness, swelling, and pain in both eyes that was unrespons ive to antibiotic therapy. Results of her examination were significant for limited extraocular movements, ptosis, erythema, edema, chemosis, and exophthalmos. Electrocardiogram and chest x-ray were normal. Orbi tal computed tomographic scan showed swelling of the extraocular muscl es up to and including their insertions. The patient was given the dia gnosis of orbital polymyositis and her condition improved clinically a nd radiographically while taking parenteral steroids. One month after discharge, the patient was in cardiogenic shock. Endomyocardial biopsy showed giant cell myocarditis, and the patient underwent emergent car diac transplantation. Despite a complicated postoperative course, the patient has done remarkably well. Conclusion: Although this disorder i s rare, this case suggests the need for a high index of suspicion for giant cell myocarditis in patients with inflammatory orbital polymyosi tis. In non-Craves orbital polymyositis the patient should be question ed and instructed concerning the signs and symptoms of congestive hear t failure. Chest x-ray, Hotter monitoring, and electrocardiogram also should be performed and be repeated with an echocardiogram if there ar e any cardiac symptoms. In addition, early endomyocardial biopsy shoul d be considered in the proper clinical setting, allowing timely diagno sis and expeditious cardiac transplantation.