title: Experience of intravenous immunoglobulin therapy in neuropathy associated with primary Sjögren's syndrome: a national multicentric retrospective study.
abstract: OBJECTIVE
  Sjögren's syndrome (SS)-related peripheral neuropathy is responsible for disability, but no treatment has been shown to improve its outcome. In some cases, intravenous immunoglobulin (IVIG) therapy has been associated with some benefit. In this study, we investigated the effectiveness of IVIG in SS-related peripheral neuropathy.
  
  
  METHODS
  We assessed the efficacy and tolerance of IVIG in 19 patients with primary SS-related neuropathy without any necrotizing vasculitis in a retrospective national multicentric study. The evaluation of the response was assessed using the disability Modified Rankin Scale (MRS) and a global evaluation by the practitioner.
  
  
  RESULTS
  Eight patients (42%) exhibited a decrease of the MRS score corresponding to a clinical improvement, 10 patients (52%) exhibited a stable MRS score, and 1 patient (6%) showed an increase of MRS score. According to the global evaluation by the practitioner, 9 (47%) of the 19 patients were improved, 6 patients (31%) were stable, and 4 patients (21%) worsened. All the patients with sensorimotor (n = 5) or nonataxic sensory neuropathy (n = 4) were improved or stabilized. However, among the patients with ataxic neuropathy (n = 9), only 2 improved and 4 worsened. Ten of the 13 patients treated with corticosteroids could have had the prednisone dosage decreased from 15 mg/day (range 7.5-60) to 10 mg/day (range 5-20) with IVIG. Only 1 patient stopped the treatment after 1 dose because of a minor side effect and lack of initial efficacy.
  
  
  CONCLUSIONS
  IVIG may be useful in the treatment of SS-associated sensorimotor neuropathies or nonataxic sensory neuropathy without any necrotizing vasculitis. The benefit of such therapy in the SS-related ataxic neuropathy seems less clear.
authors.affiliation: Hôpital de la Source, Orléans, France.
authors.forename: Stéphanie
authors.initials: S
authors.lastname: Rist
authors.forename: Jérémie
authors.initials: J
authors.lastname: Sellam
authors.forename: Eric
authors.initials: E
authors.lastname: Hachulla
authors.forename: Christelle
authors.initials: C
authors.lastname: Sordet
authors.forename: Xavier
authors.initials: X
authors.lastname: Puéchal
authors.forename: Pierre-yves
authors.initials: PY
authors.lastname: Hatron
authors.forename: Claude-laurent
authors.initials: CL
authors.lastname: Benhamou
authors.forename: Jean
authors.initials: J
authors.lastname: Sibilia
authors.forename: Xavier
authors.initials: X
authors.lastname: Mariette
mesh_terms: D000305:Adrenal Cortex Hormones / Q000008:administration & dosage; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D004185:Disability Evaluation; D005260:Female; D005602:France; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000008:administration & dosage* / Q000009:adverse effects; D007166:Immunosuppressive Agents / Q000008:administration & dosage* / Q000009:adverse effects; D008297:Male; D008875:Middle Aged; D010523:Peripheral Nervous System Diseases / Q000175:diagnosis / Q000188:drug therapy* / Q000209:etiology; D012189:Retrospective Studies; D012859:Sjogren's Syndrome / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy*; D016896:Treatment Outcome
chemical_list: D000305:Adrenal Cortex Hormones; D016756:Immunoglobulins, Intravenous; D007166:Immunosuppressive Agents