Diagnosis and management of spinal cord sarcoidosis

Abstract

Background: Sarcoidosis of the spinal cord is very rare, affecting less than 1% of patients with sarcoidosis.

Methods: We retrospectively reviewed the charts of all patients with biopsy proven sarcoidosis and spinal cord involvement seen in our Interstitial Lung Disease and Sarcoidosis Clinic at the University of Cincinnati over a nineteen year period. Presentation of disease, method of diagnosis, and response to immunosuppressive therapy (methotrexate, azathioprine, or cyclophosphamide) were noted.

Results: Seventeen patients with spinal cord sarcoidosis were identified. All patients presented with insidious parethesias or weakness. Diagnosis was confirmed by biopsy of spinal cord lesions in two patients, dural biopsy in three patients, mediastinoscopy in eight patients, bronchoscopy in two patients, skin biopsy in one patient, and liver biopsy in one patient. Only four patients were diagnosed with sarcoidosis prior to spinal cord involvement. Cytotoxic therapy was initiated to reduce the daily dose of prednisone to less than 10 mg. Fourteen patients were evaluated at least six months after starting cytotoxic therapy. Of these evaluable patients, ten patients received methotrexate plus low dose prednisone and five responded. One patient received azathioprine plus low dose prednisone and responded. One patient received azathioprine, methotrexate, and low dose prednisone and responded. All seven patients that received cyclophosphamide responded.

Conclusions: Spinal cord sarcoidosis should be considered in any patient presenting with gradual onset of paresthesias or weakness regardless of the diagnosis of sarcoidosis. Prompt treatment is important in long term outcome. Cyclophosphamide was an effective, well tolerated corticosteroid-sparing agent for patients with spinal cord sarcoidosis.