TY - JOUR
T1 - Infliximab treatment in pathology-confirmed neurosarcoidosis
AU - Fritz, Daan
AU - Timmermans, Wilhelmina M. C.
AU - van Laar, Jan A. M.
AU - van Hagen, P. Martin
AU - Siepman, Theodora A. M.
AU - van de Beek, Diederik
AU - Brouwer, Matthijs C.
PY - 2020/9/1
Y1 - 2020/9/1
N2 - 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.
AB - 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.
UR - http://www.scopus.com/inward/record.url?scp=85088812036&partnerID=8YFLogxK
U2 - https://doi.org/10.1212/NXI.0000000000000847
DO - https://doi.org/10.1212/NXI.0000000000000847
M3 - Article
C2 - 32718952
SN - 2332-7812
VL - 7
JO - Neurology® neuroimmunology & neuroinflammation
JF - Neurology® neuroimmunology & neuroinflammation
IS - 5
ER -