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

Primary objective: Identify the predictors of quality of life impairment and costs of management of patients with Sjögren's syndrome and small fiber neuropathy (SFN).

Secondary objectives :

• Hospital, drug and city-care costs
• Distribution of cost items
• Part of costs attributable to pSS-associated SFN
• Loss of quality of life attributable to pSS-associated SFN
• Relationship between the domains of the EQ 5D questionnaire and the other specific quality of life questionnaires
• Association of the EQ 5D and other questionnaires with the costs (part of the costs variance that might be explained by the quality of life).

PATIENTS AND METHODS / Study population

Monocentric study in the Department of Internal Medicine of Lariboisière Fernand Widal Hospital.

All included patients fulfilled 2002-criteria of primary Sjogren syndrome.

Patients are classified into 2 arms:

Arm1: patients with Sjögren's syndrome and definite SFN

Arm2 ( Control Group) : patients with Sjögren's syndrome and WITHOUT clinical and paraclinical arguments for peripheral neuropathy

RESARCH PROCESS

This study will be based on the delivery and analysis of validated questionnaires (collection sheet) in chronic diseases, pains or Sjögren's syndrome:

• SF-36 for quality of life,
• DN4 questionnaire to estimate the probability of neuropathic pain
• EQ5D Health questionnaire
• ESSPRI (4 questions): Sjogren-patient questionnaire
• PROFAD-SSI (19 questions), to assess tiredness, discomfort, pain and dryness symptoms associated with Sjögren's syndrome.
• The economic evaluation will be done after the inclusion visit and will be based on the collection of care resources consumed by the patients during a period of 6 months

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.