Experience of intravenous immunoglobulin therapy in neuropathy associated with primary Sjögren's syndrome: A national multicentric retrospective study

Abstract

Objective. Sjö gren'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. Conclusion. 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.

Immunotherapy Prospects for Painful Small-fiber Sensory Neuropathies and Ganglionopathies

“…Small case series support the use of IVIg for ataxic (large-fiber) sensory ganglionitis [22]. The best evidence comes from a French multicenter study documenting efficacy of IVIg 2 g/kg for SS neuropathy, including nonataxic (small-fiber) ganglionitis [23].…”

“…Various neuronal antigens have been implicated in Sjögren syndrome (SS) axonopathy, but verification has been elusive (reviewed in [83]). A French multicenter study documented efficacy of IVIg 2 g/kg for SS neuropathy, including sensorimotor axonopathy [23]. A woman with chronic pain, edema, and vasodysregulation in the left foot and lower leg following bunionectomy years prior.…”

Bilateral Tonic Pupils as the Initial Manifestation of Sjögren’s Syndrome

“…The use of intravenous immunoglobulin has been shown beneficial in patients with Sjö gren's syndrome-associated neuropathy, particularly sensorimotor neuropathy. 16 In this case, intravenous immunoglobulin was administered, resulting in improvement of her trigeminal neuropathy alone, with no clinical change in the underlying autonomic neuropathy. A previous case report using plasma exchange also showed recovery of the patient's sensorimotor neuropathy, but Holmes-Adie syndrome remained unchanged.…”

Safety of treatments for primary Sjögren’s syndrome

“…[122] A retrospective study in 19 pSS patients with peripheral neuropathy reported a beneficial effect of monthly courses of IVIG on SS-associated sensorimotor neuropathy and non-ataxic sensory neuropathy. [123] Tolerance of IVIG was good. During the median treatment duration of 7 months, only one withdrawal due to an AE (nausea) was reported and no serious AEs occurred.…”