Chest
Volume 124, Issue 5, November 2003, Pages 2023-2026
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Selected Reports
Treatment of Corticosteroid-Resistant Neurosarcoidosis With a Short-Course Cyclophosphamide Regimen

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Background/objectives

Many patients with neurosarcoidosis have disease that is refractory to corticosteroids or they are unable to tolerate high-dose corticosteroids because of detrimental side effects. We examined a short-course, pulse-dose regimen using cyclophosphamide to treat such patients.

Methods

We identified a population of patients with neurosarcoidosis refractory to standard therapy with corticosteroids. Patients who were unable to tolerate corticosteroid therapy due to side effects were also included. Alternative therapy for these patients was initiated using IV cyclophosphamide.

Results

Seven patients were identified for treatment with our cyclophosphamide regimen. The mean duration of therapy was 5.4 months. Four of the seven patients reported symptomatic improvement on therapy, and all seven patients demonstrated objective improvement in either MRI or cerebrospinal fluid abnormalities. Mean corticosteroid dose of the group was reduced from 42 mg/d before therapy to 18 mg/d after therapy. Relapse of neurologic symptoms was noted in one patient after the completion of therapy. One patient acquired an opportunistic infection, and a second patient required hospitalization for a central venous catheter infection.

Conclusion

Short-course cyclophosphamide appears to be a reasonable, steroid-sparing treatment option for patients with corticosteroid-refractory neurosarcoidosis.

Section snippets

Patients and Methods

Patients were selected from those followed up at the Sarcoidosis Center at the Medical University of South Carolina. Between September 1998 and June 2002, 451 patients were seen at our center. Thirty-seven patients met either definite or probable criteria by the Case Control Etiologic Study of Sarcoidosis9 instrument for the diagnosis of neurosarcoidosis. We identified patients whose neurosarcoidosis was refractory to conventional therapy with corticosteroids or who were unable to tolerate

Results

Seven patients with refractory neurosarcoidosis were selected for cyclophosphamide therapy. The baseline characteristics of the patients receiving cyclophosphamide are summarized in Table 1. Table 2 describes the diagnostic criteria met by each patient and the specific neurologic manifestations.

The clinical course is summarized in Table 3. All patients were receiving corticosteroids at the time of cyclophosphamide initiation, with an average dose of 42 mg/d. The duration of therapy with

Discussion

Traditionally, high-dose corticosteroids have been the standard of care for treatment of neurosarcoidosis. Unfortunately, a significant percentage of patients with neurosarcoidosis have disease refractory to corticosteroids or are unable to tolerate the high doses that are often required for effective treatment.2 In recent years, attention has been turned to the use of cytotoxic and noncytotoxic agents as corticosteroid-sparing agents. Methotrexate, cyclosporine, chlorambucil, azathioprine,

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