RECRUITINGOBSERVATIONAL

Medico-economic and Quality of Life Impact of Sjogren-associated Small Fiber Neuropathy

The Medico-economic Impact and Quality of Life of the Small-fiber Neuropathy Associated by Using Validated Scales (SF36, DN4, PROFAD SSI, ESPRI)

About This Trial

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.

Who May Be Eligible (Plain English)

Who May Qualify: - 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) Who Should NOT Join This Trial: - Presence of other causes of peripheral neuropathy - Acquired: Diabetes, AL amyloidosis, Alcoholism, celiac disease, Drugs, toxic, HIV, Sarcoidosis, systemic vasculitis, Guillain-Barré syndrome. - Hereditary: Transthyretin hereditary amyloidosis (TTR), hereditary sensory and autonomic neuropathy (HSAN), Fabry's disease - Patients with impaired thermo-algic sensitivity and / or dysautonomia and / or pain with DN4 ≥ 4 AND normal diagnostic tests (normal neurophysiological tests AND normal skin biopsy) are excluded. Always talk to your doctor about whether this trial is right for you.

Original Eligibility Criteria

View original clinical language

Inclusion Criteria: * 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) Exclusion Criteria: * Presence of other causes of peripheral neuropathy * Acquired: Diabetes, AL amyloidosis, Alcoholism, celiac disease, Drugs, toxic, HIV, Sarcoidosis, systemic vasculitis, Guillain-Barré syndrome. * Hereditary: Transthyretin hereditary amyloidosis (TTR), hereditary sensory and autonomic neuropathy (HSAN), Fabry's disease * Patients with impaired thermo-algic sensitivity and / or dysautonomia and / or pain with DN4 ≥ 4 AND normal diagnostic tests (normal neurophysiological tests AND normal skin biopsy) are excluded.

Treatments Being Tested

OTHER

Collection sheet

Using EQ5D questionnaire and a collection of care resources consumed by the patients during a period of 6 months associated by using validated scales (SF36, DN4, PROFAD SSI, ESPRI) : collection sheet

Locations (1)

Département de Médecine Interne - Hôpital Lariboisière

Paris, France