Symptom Severity and Quality of Life in Sarcoidosis-associated Small Fiber Neuropathy
Sofia Ramirez Guerrero1, María Paula Aguilera Peña2, Elizabeth Frame3, Bobak Akhavan4, Evan Kransdorf5, Paula Barreras1
1Department of Neurology, Cedars-Sinai Medical Center, 2Department of Neurology, Tulane School of Medicine, 3Division of Rheumatology, Cedars-Sinai Medical Center, 4Division of Pulmonary and Critical Care, Cedars-Sinai Medical Center, 5Smidt Heart Institute, Cedars-Sinai Medical Center
Objective:
To explore differences in symptoms, quality of life, and disease severity in patients with sarcoidosis with and without Small Fiber Neuropathy (SFN).
Background:
SFN occurs frequently in patients with systemic sarcoidosis and can affect quality of life. It is uncertain if this is due to inflammatory disease activity.
Design/Methods:
Cross-sectional study including subjects with biopsy-proven systemic sarcoidosis with and without SFN and non-sarcoidosis SFN. Questionnaires were used to assess symptoms and quality of life: Pain Effects Scale (PES), King’s Sarcoidosis Questionnaire (KSQ), Modified Fatigue Impact Scale (MFIS), SFN Screening List (SFNSL), Rasch-Transformed SFN-Symptoms Inventory Questionnaire (RT-SFN-SIQ). Sarcoidosis organ involvement and disease activity markers were recorded.
Results:
16 subjects had systemic sarcoidosis alone (SS), 8 had sarcoidosis-associated SFN (S-SFN), and 6 had non-sarcoidosis SFN. Compared to the SS group, the S-SFN group had more severe pain (PES 20.5 vs. 6.0, p<0.05), fatigue (MFIS 15.0 vs. 7.0, p<0.05), dysautonomia symptoms per RT-SFN-SIQ (13.9 vs. 5.9, p<0.05) and SFNSL (33.9 vs. 9.3, p<0.05), and worse sarcoidosis-related disability per KSQ score (79.3 vs. 59.3, p=0.08). No significant differences were found in these scores between S-SFN and non-sarcoidosis SFN. None of the S-SFN cases had diabetes, alcohol use disorder, or vitamin deficiencies. There was no age or sex difference between SS and S-SFN. Patients with S-SFN were older than patients with non-sarcoidosis SFN (59.0 vs. 48.3 y/o, p=0.13). Extrapulmonary involvement was common in both the SS (n=15/16) and the S-SFN group (n=6/8). The SS group had higher levels of Vit-D-1,25-OH D (59.7 vs. 43.3 pg/ml, p<0.05) and serum sIL2R (1719 vs.1477 pg/ml,p=0.29) than the S-SFN group.
Conclusions:
Patients with sarcoidosis who develop SFN have worse pain, fatigue, dysautonomia, and quality of life than patients without SFN. Proposed markers of sarcoidosis disease activity sIL2-R and Vit-D-1,25-OH may not be relevant in the assessment of S-SFN, though larger studies are needed.
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