How We Treat Autoimmune Small Fiber Polyneuropathy with Immunoglobulin Therapy

Schofield, Jill R.; Chémali, Kamal R.

doi:10.1159/000498858

Cited by 20 publications

(24 citation statements)

References 26 publications

(28 reference statements)

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“…The results of this study are in contrast to open-label case series, many of whom had an immune-mediated underlying condition [31][32][33][35][36][37] and a retrospective study suggesting IVIg's efficacy in SFN. 31,32,38 However, based on those reports, SFN patients, without or without a clear autoimmune condition, are increasingly being treated with IVIg.…”

Section: Discussioncontrasting

confidence: 87%

Intravenous Immunoglobulin Therapy in Patients With Painful Idiopathic Small Fiber Neuropathy

et al. 2021

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Objective:This is the first double-blind, randomized, controlled trial evaluating the efficacy and safety of intravenous immunoglobulin (IVIg) versus placebo in patients with idiopathic small fiber neuropathy (I-SFN).Methods:Between July 2016 and November 2018, 60 Dutch patients with skin-biopsy proven idiopathic SFN randomly received a starting dose of IVIg (2 g/kg body weight) or matching placebo (0.9% saline). Subsequently, 3 additional infusions of IVIg (1 g/kg) or placebo were administered at 3-weekly intervals. The primary outcome was a 1-point change in Pain Intensity Numerical Rating Scale (PI-NRS) at 12 weeks compared to baseline.Results:Thirty patients received IVIg, and 30 received placebo. In both groups, 29 patients completed the trial. In 40% of patients receiving IVIg, the mean average pain was decreased with at least 1 point, compared to 30% of the patients receiving placebo (p-value 0.588, OR 1.56, 95%CI 0.53-4.53). No significant differences were found on any of the other pre-specified outcomes including general wellbeing, autonomic symptoms, and overall functioning and disability.Conclusions:This RCT showed that IVIg treatment had no significant effect on pain in patients with painful idiopathic SFN.

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Section: Discussioncontrasting

confidence: 87%

Intravenous Immunoglobulin Therapy in Patients With Painful Idiopathic Small Fiber Neuropathy

et al. 2021

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“…However, side effects could generally be reduced by reducing the infusion rate and by premedication with steroids and additional intravenous hydration. As an alternative, pretreatment with nonsteroidal anti‐inflammatory medication or antihistamines has been recommended in patients with immune‐mediated small fiber neuropathy receiving IVIG [15] and could also be evaluated in POTS patients with immune‐mediated dysautonomia. Furthermore, we recommend close monitoring of patients during immunomodulatory therapy, as in two patients of our patient group an adjustment of symptomatic treatment was necessary.…”

Section: Discussionmentioning

confidence: 99%

“…The patient group presented in this case series consistently described a with nonsteroidal anti-inflammatory medication or antihistamines has been recommended in patients with immune-mediated small fiber neuropathy receiving IVIG [15] and could also be evaluated in POTS patients with immune-mediated dysautonomia.…”

Section: Discussionmentioning

confidence: 99%

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Immunomodulatory treatment in postural tachycardia syndrome: A case series

Rodríguez

Hoepner

Salmen

et al. 2021

Euro J of Neurology

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Background and purpose Postural tachycardia syndrome (POTS) is a form of autonomic dysfunction characterized by symptoms of orthostatic intolerance, often accompanied by sudomotor dysfunction and gastrointestinal dysmotility. Recently, evidence has accumulated that in a subset of patients, the pathogenesis of dysautonomia may be immune‐mediated. The aim of the current report was to evaluate the use of intravenous immunoglobulin (IVIG) treatment in patients with progressive and/or refractory immune‐mediated POTS. Methods We retroactively assessed the effect and tolerance of monthly administered IVIG in six patients using autonomic function testing, standardized symptom questionnaires, and patients' symptom diaries both before and 6 months into IVIG treatment. Objective outcome measures included heart rate increase after 10 min of head‐up tilt as well as duration and anhidrotic area in a thermoregulatory sweat test. Subjective outcome measures were patient reports and symptom ratings from the symptom questionnaire. Results All patients responded to immunomodulatory treatment, regardless of disease duration. After 6 months of IVIG, symptom severity was reduced by nearly 40%. Autonomic function testing showed improved cardiovascular functioning by 50% and a reduction of anhidrotic areas by one third. Overall, tolerance of IVIG treatment was poor, but could be improved by a reduction in infusion rate, premedication with steroids, and additional intravenous hydration. Conclusions Using subjective but also standardized objective measures, the case series describes promising effects of IVIG treatment in POTS patients with immune‐mediated dysautonomia. By reducing the infusion rate, pretreatment with steroids, and intravenous hydration, tolerance could be improved, and no patient had to discontinue the treatment.

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“… 1 Biotherapeutics are used to treat a broad range of severe diseases, for instance the immune messenger cytokines interferon beta (IFNβ) and alpha (IFNα) products are used to treat multiple sclerosis (MS) 2 and viral diseases 3 respectively. Moreover monoclonal antibodies are used to treat a range of diseases: autoimmune diseases – such as MS 4 and Guillain-Barré syndrome 5 – chronic inflammatory diseases, such as Crohn's disease, 6 as well as numerous cancers. 7,8 One of the major challenges of producing, distributing and storing these protein therapeutics is the risk of aggregation.…”

Section: Introductionmentioning

confidence: 99%