Objectives: Central nervous system manifestations of sarcoidosis occur in approximately 5% of patients with sarcoidosis, often lead to substantial morbidity, and therefore require immediate treatment. Spinal cord involvement is exceptionally rare. The tumor necrosis factor-α inhibitor infliximab seems to be an effective alternative in severe cases, refractory to other therapies, but a standard concept of treatment is lacking.
Methods: We presented a case of severe corticosteroid-refractory spinal cord sarcoidosis with immediate and dramatic response to infliximab. In addition, we reviewed the literature on infliximab therapy in neurosarcoidosis and drew parallels to other medical fields in order to have a basis for decision making in the initiation and discontinuation of treatment.
Results: We identified a total of 34 case reports on effective infliximab treatment of therapy-resistant neurosarcoidosis through PubMed search. Nineteen of the 34 cases reported the duration until treatment response. In accordance with our patient, 14 of the 34 case reports showed improvement between first and third infusion. Eight of the 34 cases reported sustained remission after cessation of infliximab. No definite treatment regimen was used.
Conclusions: Infliximab seems to be a fast-acting and effective drug for severe neurosarcoidosis. No systematic treatment strategy is available because of lack of controlled trials. Until then, therapy regimens may be adapted to those used in other medical fields where infliximab treatment is well established.