ArticleTherapeutic advances in neurological disorders2018
IVIg for apparently autoimmune small-fiber polyneuropathy: first analysis of efficacy and safety.
Article in Therapeutic advances in neurological disorders, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 36 papers, 1 of them a synthesis that pooled it.
What it found
Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.
The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
36 citing papers in PubMed, 1 synthesis or guideline pooled it, 96 citations in OpenAlex.
- Incidence and risk factors for intravenous immunoglobulin-related hemolysis: A systematic review of clinical trial and real-world populations.Transfusion · 2022Pooled it
- Intravenous Immunoglobulin Therapy in Patients With Painful Idiopathic Small Fiber Neuropathy.Neurology · 2021Trial
- Autoantibodies in small fiber neuropathy: frequency and clinical features associated with antibodies to the novel targets MX1 and DBNL.Journal of neurology · 2026Observational
- Interferon-induced GTP-binding protein MX1 drives hyperexcitability in peripheral nerves: a novel mechanism in small fiber neuropathy.Journal of neuroinflammation · 2025Article
- Article
- iSTAND trial of IVIG in POTS: a step in the right direction, but more studies are needed.Clinical autonomic research : official journal of the Clinical Autonomic Research Society · 2025Article
- Scleritis and episcleritis in patients with idiopathic small fiber neuropathy.American journal of ophthalmology case reports · 2024Article
- Relapsing-Remitting Immunotherapy Responsive Small-Fiber Neuropathy: Longitudinal Tracking Through 10 Years Including Pregnancies.Neurology(R) neuroimmunology & neuroinflammation · 2024Article
- Post-COVID Small Fiber Neuropathy, Implications of Innate Immunity, and Challenges on IVIG Therapy.Neurology(R) neuroimmunology & neuroinflammation · 2024Article
- Characterizing Acute-Onset Small Fiber Neuropathy.Neurology(R) neuroimmunology & neuroinflammation · 2024Article
- Advances in diagnosis and management of distal sensory polyneuropathies.Journal of neurology, neurosurgery, and psychiatry · 2023Review
- Review
- Long COVID, the Brain, Nerves, and Cognitive Function.Neurology international · 2023Review
- Corneal Sub-Basal Nerve Plexus in Non-Diabetic Small Fiber Polyneuropathies and the Diagnostic Role of In Vivo Corneal Confocal Microscopy.Journal of clinical medicine · 2023Review
- A case of erythromelalgia with gastrointestinal dysautonomia treated with immunoglobulin: A case report.SAGE open medical case reports · 2023Article
- Small fiber polyneuropathy: A new therapeutic target in patients with interstitial cystitis/bladder pain syndrome?Frontiers in urology · 2023Review
- A role for pathogenic autoantibodies in small fiber neuropathy?Frontiers in molecular neuroscience · 2023Review
- Immunoglobulin Use for the Management of Painful Peripheral Neuropathy: A Systematic Review and Meta-Analysis.Pain and therapy · 2022Article
- Peripheral Neuropathy Evaluations of Patients With Prolonged Long COVID.Neurology(R) neuroimmunology & neuroinflammation · 2022Article
- Inappropriate sinus tachycardia in long-COVID and other updates on recent autonomic research.Clinical autonomic research : official journal of the Clinical Autonomic Research Society · 2022Article
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
4 authors at 4 institutions in 3 countries.
Funding
Abstract
objectivesSmall-fiber polyneuropathy (SFPN) has various underlying causes, including associations with systemic autoimmune conditions. We have proposed a new cause; small-fiber-targeting autoimmune diseases akin to Guillain-Barré and chronic inflammatory demyelinating polyneuropathy (CIDP). There are no treatment studies yet for this 'apparently autoimmune SFPN' (aaSFPN), but intravenous immunoglobulin (IVIg), first-line for Guillain-Barré and CIDP, is prescribed off-label for aaSFPN despite very high cost. This project aimed to conduct the first systematic evaluation of IVIg's effectiveness for aaSFPN.
methodsWith IRB approval, we extracted all available paper and electronic medical records of qualifying patients. Inclusion required having objectively confirmed SFPN, autoimmune attribution and other potential causes excluded. IVIg needed to have been dosed at ⩾1 g/kg/4 weeks for ⩾3 months. We chose two primary outcomes - changes in composite autonomic function testing (AFT) reports of SFPN and in ratings of pain severity - to capture objective as well as patient-prioritized outcomes.
resultsAmong all 55 eligible patients, SFPN had been confirmed by 3/3 nerve biopsies, 62% of skin biopsies, and 89% of composite AFT. Evidence of autoimmunity included 27% of patients having systemic autoimmune disorders, 20% having prior organ-specific autoimmune illnesses and 80% having ⩾1/5 abnormal blood-test markers associated with autoimmunity. A total of 73% had apparent small-fiber-restricted autoimmunity. IVIg treatment duration averaged 28 ± 25 months. The proportion of AFTs interpreted as indicating SFPN dropped from 89% at baseline to 55% (
conclusionThese results provide Class IV, real-world, proof-of-concept evidence suggesting that IVIg is safe and effective for rigorously selected SFPN patients with apparent autoimmune causality. They provide rationale for prospective trials, inform trial design and indirectly support the discovery of small-fiber-targeting autoimmune/inflammatory illnesses.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.