Table 1

Description of the data requested in the study forms

Data typeRequested fields
For all included patients (mandatory form)Demographic data, verification of exclusion and inclusion criteria, SNN score, final retained diagnosis (SNN, CIDP, SFN, LDN or other), autoimmune context (SS, SLE, IBD or inflammatory rheumatism)
For anti-FGFR3-positive patients and ‘center-matched’ negative patients (detailed form)
 Clinical data at onsetDate, course of neuropathy, sensory symptoms, topography of symptoms (UL, LL, proximal or distal)
 Clinical data at the first clinical exam and at the last clinical examDate, symptoms as reported by the patient, distribution of sensory manifestations as reported by the patient and as observed on clinical examination (UL, LL, proximal or distal), presence of dysautonomia and/or ataxia, tendon reflexes, M-Rankin score and other clinical scores
 ENMGDate, copy of the reports, normal values of the ENMG laboratory
 Paraclinical dataAssociated diseases: diabetes, cancers, IBD, celiac disease, autoimmune hepatitis, inflammatory rheumatic disease, SLE, SS, Sharp syndrome, sclerodermia, sarcoidosis, other diseases
Laboratory investigations: blood biochemical analyses (glucose, CRP, HbA1c, creatinine, liver enzymes, TSH, protein electrophoresis), immunology (complement, research of autoantibodies: onconeuronal, antineuronal, anti-ganglioside, ANA, anti-dsDNA, anti-SSA/SSB, ANCA, anti-citrulline, anti-mitochondrial, anti-tissue transglutaminase, rheumatoid factor, cryoglobulins) and haematology (CBC), cerebrospinal fluid analyses (total protein, glucose), biopsies (nerve, skin or other)
Imaging examination: PETscan, MRI or other
 Immunomodulatory treatmentType, date of onset and end, posology, treatment efficiency (stabilisation, failure, improvement, not clear)
  • ANA, antinuclear antibody; ANCA, antineutrophil cytoplasmic antibodies; anti-dsDNA, anti-double stranded DNA antibodies; CBC, complete blood count; CIDP, chronic inflammatory demyelinating polyneuropathy; CRP, C reactive protein; ENMG, electroneuromyography;FGFR3, fibroblast growth factor receptor 3; HbA1c, glycosylated haemoglobin A1c; IBD, inflammatory bowel disease; LDN, length-dependent neuropathy; LL, lower limbs; M-Rankin, modified-Rankin; PETscan, positron emission tomography; SFN, small fibre neuropathy; SLE, systemic lupus erythematous; SNN, sensory neuronopathy; SS, Sjögren syndrome; SSA, Sjögren syndrome A;SSB, Sjögren syndrome B; TSH, thyroid-stimulating hormone; UL, upper limbs.