Sarcoidosis-Associated Small Fiber Neuropathy: Clinical Features and Response to Immunosuppression
María Paula Aguilera Peña1, Barney Stern2, Carlos Pardo-Villamizar3, Paula Barreras1
1Cedars-Sinai Medical Center, 2Johns Hopkins Outpatient Center, 3Johns Hopkins U, Med Dept of Neurology
Objective:
To describe a cohort of patients with sarcoidosis-associated small fiber neuropathy (S-SFN) and their response to immunosuppression.
Background:
Small fiber neuropathy (SFN) affects up to 40% of sarcoidosis patients, causing chronic pain and disability. Risk factors and responses to immunosuppression are not well understood.
Design/Methods:
Retrospective study of biopsy-proven S-SFN patients, excluding those with large fiber neuropathy. We compared clinical characteristics and treatment responses between two groups: those with sarcoidosis without other risks for SFN (group 1) and those with sarcoidosis and additional risk factors for SFN (group 2).
Results:
: Thirty-seven patients were included; 23 (62%) were female. Presenting symptoms included pain (22, 60%), paresthesias (20, 54%), and numbness (20, 54%). Other symptoms were dysautonomia (18, 49%), fatigue (15, 41%), and brain fog (10, 27%). Additional contributors to SFN were identified in 24 patients (65%) (group 2); with diabetes (18, 49%), chronic kidney disease (6, 16%), and vitamin B12 deficiency (4, 13%) being the most frequent. The median follow-up was 26 months (IQR 12–47). Despite treatment with corticosteroids (97%) and standard sarcoidosis therapies, most patients experienced worsening symptoms (40% in group 1 vs. 53% in group 2, p=0.79). At follow-up, 33% had a mRS score ≥3, with worsening in group 1 being more common than in group 2 (40% vs 6%, p-value: 0.02). Symptom improvement was reported with adalimumab (5 of 10, 50%), IVIG (2 of 4, 50%), infliximab (4 of 10, 40%), and tocilizumab (3 of 4, 75%), with the highest response to TNF-alpha inhibitors in group 1 (50% vs. 42%).
Conclusions:
S-SFN is associated with chronic symptoms, including pain, dysautonomia, fatigue, and brain fog, with many patients worsening over time. Immunotherapies show promise, particularly TNF alpha inhibitors, IVIG, and tocilizumab. Identifying contributing comorbidities and exploring novel treatment options is essential for improving outcomes.
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