Infliximab treatment in pathology-confirmed neurosarcoidosis

Abstract

Objective: To assess the efficacy and risks of treatment with infliximab (anti-tumor necrosis factor alpha) in pathology-confirmed neurosarcoidosis.

Methods: In a retrospective study in 2 tertiary referral centers in the Netherlands, we analyzed clinical characteristics, complications, and outcome of patients with neurosarcoidosis treated with infliximab.

Results: Twenty-eight patients were identified with a mean age of 42 years. Neurosarcoidosis presented with a cerebral parenchymal localization in 16 (59%), pituitary gland/hypothalamic sarcoidosis in 15 (54%), peripheral nerve involvement in 12 (43%), and chronic meningitis in 11 patients (41%). Initial treatment response after the start of infliximab was complete remission in 6 (21%) and improvement in 14 (50%), whereas 7 patients had stable disease (25%), and 1 (4%) deteriorated and died. At the end of follow-up, with a median of 32 months, 5 patients (18%) had died, and 2 (40%) were using infliximab at the time of death. Tapering or discontinuation of corticosteroids without a relapse was achieved in 19 of 28 patients (68%). In patients with decreasing dosing or discontinuation of infliximab, a relapse occurred in 5 of 19 patients (26%). Complications of infliximab were reported in 10 of 28 patients (36%) and mainly consisted of infections in 8 (29%).

Conclusion: Infliximab is an effective treatment in neurosarcoidosis leading to remission or improvement in 70%. The mortality rate in infliximab-treated patients was substantial, indicating the severity of disease and treatment-associated complications.

Classification of evidence: This study provides Class IV evidence that in people with pathology-confirmed neurosarcoidosis, infliximab is beneficial.

Figure 1. Serum soluble interleukin 2 receptor and angiotensin-converting enzyme values before and after infliximab

The dotted line indicates the upper limit of the normal value. p Values were calculated using the Mann-Whitney U test. (A) Serum sIL2r values before and after the start of infliximab. (B) Serum ACE values before and after the start of infliximab. ACE = angiotensin-converting enzyme; sIL2r = soluble interleukin 2 receptor.

Figure 2. Modified Rankin Scale score change of follow-up compared with baseline

0: asymptomatic, 1: complaints without functional disability, 2: complaints with slight functional disability, 3: complaints with moderate disability, 4: complaints with moderate-to-severe functional disability, 5: complaints with severe disability, and 6: death. mRS = modified Rankin Scale.