IVIg for apparently autoimmune small -fiber polyneuropathy: First analysis of efficacy and safety, Oaklander et al, 2017

Andy · Dec 10, 2017

Abstract

Objectives
Small-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.

Methods
With IRB approval, we extracted all available paper and electronic medical records of qualifying patients. Inclusion required having objectively confirmed SFPN, auto immune attribution, and
other potential causes excluded. IVIg needed to have been dosed at ≥1gram/kg/4 weeks for
≥3 months. We chose two primary outcomes – changes in composite autonomic function testing (AFT) reports of SFPN and in pain severity–to capture objective as well as patient-prioritized outcomes.

Results
Among all 55 eligible patients, SFPN had been confirmed by 3/3 nerve biopsies, 62% of skin biopsies, and 89% of composite autonomic function testing (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 of autoimmunity. 73% had apparent small-fiber-restricted autoimmunity. IVIg treatment duration averaged 28 ± 2 5 months. The proportion of AFTs interpreted as indicating SFPN dropped from 89% at baseline to 55% (p ≤ 0.001). Sweat production normalized (p=0.039) and all the other 4 domains trended towards improvement. Among patients with pre-treatment pain ≥ 3/10, severity averaging 6.3±1.7 dropped to 5.2±2.1 (p=0.007). 74% of patients rated themselves “improved” and their neurologists labeled 77% as “IVIg responders”. 16% entered remissions that were sustained after IVIg withdrawal.

All adverse events were expected; most were typical infusion reactions. The two moderate complications (3.6%) were vein thromboses not requiring discontinuation. The one severe event (1.8%), hemolytic anemia, remitted after IVIg discontinuation.

Conclusion
These 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 about trial design, and indirectly support the discovery of small-fiber-targeted autoimmune illnesses.
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Full text in PDF format - https://neuropathycommons.org/sites/default/files/IVIG TAND-in press version 11-5-17.pdf

Seen from this Facebook post
Code:
https://www.facebook.com/DysautonomiaInternational/posts/1490688391030035

Dolphin · Dec 11, 2017

‘BAMBOOZLING THE IMMUNE SYSTEM’
Her Chronic Pain Was a Medical Mystery. Was It an Unexplained Condition?
Leslie Levine’s pain felt ‘like my legs were being dipped in boiling oil 24/7.’ A neurologist’s creative solution helped.
KAREN WEINTRAUB
12.06.17 9:00 AM ET

https://www.thedailybeast.com/her-chronic-pain-was-a-medical-mystery-was-it-an-unexplained-condition
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Andy · Feb 11, 2018

Now available open access at https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5791555/

Sasha · Feb 11, 2018

Is there any evidence for this claim (from the Dysautonomia International FB post)?: 'SFPN occurs in the majority of patients with POTS, CFS, EDS, CRPS, fibromyalgia, and many other "syndrome" disorders. '

Andy · Feb 11, 2018

Sasha said: ↑

Is there any evidence for this claim (from the Dysautonomia International FB post)?: 'SFPN occurs in the majority of patients with POTS, CFS, EDS, CRPS, fibromyalgia, and many other "syndrome" disorders. '
Click to expand...

Had a quick scan through the paper and it doesn't seem to be from there.

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IVIg for apparently autoimmune small -fiber polyneuropathy: First analysis of efficacy and safety, Oaklander et al, 2017

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IVIg for apparently autoimmune small -fiber polyneuropathy: First analysis of efficacy and safety, Oaklander et al, 2017

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