The Medico-economic Impact and Quality of Life of the Small-fiber Neuropathy Associated by Using Validated Scales (SF36, DN4, PROFAD SSI, ESPRI)
BACKGROUND Sjögren's syndrome is an autoimmune disease whose prevalence is estimated between 200 and 500 patients per 100,000 persons in France (120 to 500,000 patients). It affects women (90%) between 40 and 60 years of age and main manifestations are generalized sicca syndrome (ocular, oral, cutaneous) and arthralgia. In 20% of cases, Sjögren's syndrome is associated with peripheral neuropathies, and the most common form is painful small fiber neuropathy (SFN). SFNs are mainly featured by neuropathic pain including burns (90%), numbness (87.5%), tingling (72.5%), electric shocks (70%) and tingling (82.5%) and also autonomic disorders (50 to 70%). However, there are still important issues that deserve to be investigated by clinical and basic research. Among these issues, this study will focus on: * The impact of SFN on the quality of life of patients with Sjögren's syndrome. * The medico-economic impact of the SFN taking into account the repercussions on the quality of life, including professional life, usual care cost (analgesics, medical and paramedical consultations, hospitalizations or emergency). EXPECTED RESULTS * Confirmation of the major impairment in the quality of life of patients with Sjogren-associated SFN * Analysis of correlations to highlight or not clinical or biological factors associated with quality of life impairment. * Evaluation of the cost attributed to the presence of an SFN in patients with Sjögren's syndrome and the pharmaco-economic interest of conventional therapeutic management (analgesic treatment, consultation pain) compared to the cost of more aggressive immunomodulatory treatments.
• Definite primary Sjögren syndrome
• Age over 18 years
• No biologics nor immunoglobulin therapy during the 6 months before study onset
⁃ Arm 1: patients with a small fiber neuropathy defined by the presence of a clinical AND one paraclinical abnormality
• (i) Clinical signs of small fibers involvement: thermo-algic sensory deficit or autonomic dysfunction or neuropathic pain with DN4 ≥4;
• AND
• (ii) Small fibers neurophysiological abnormalities (QST, laser evoked potentials, autonomic nervous system tests (sympathetic skin response test or Sudoscan®)
• OR
• (iii) abnormal intraepidermal nerve fiber density (skin biopsy)
⁃ Arm2 (control group): patients without signs of peripheral neuropathy (small or large fiber)