Abstract
The objective of this study is to determine if the nerve pathology in patients with POEMS syndrome is different from CIDP. We hypothesized that nerve biopsies from patients with POEMS syndrome would have more small vessels and axonal degeneration but less inflammation than CIDP.We performed a retrospective analysis of nerve biopsies performed on “classic” CIDP and POEMS cases. Nerve biopsies were blinded and reviewed by two of the authors (EAP, PJBD). Teased fibers, paraffin-embedded sections, semithin sections and immunostains were analyzed. Small endoneurial and epineurial vessels were counted on paraffin sections with smooth muscle actin (SMACTIN) preparation to judge for neovascularization.A total of 61 cases (35-POEMS, 26-CIDP) were included. The POEMS-group had significantly higher axonal degeneration and fewer normal myelinated fibers on teased fiber preparations. The CIDP-group had significantly more endoneurial mononuclear inflammation on paraffin sections and immunostains. Large onion-bulbs were present only in CIDP cases. A significantly higher number of epineurial vessels was present in POEMS biopsies, with a total count of 120 epineurial vessels predicted as best cutoff to differentiate both conditions (77 % specific and 54 % sensitive).In conclusion, nerve biopsy can be helpful in distinguishing POEMS syndrome from CIDP. POEMS syndrome demonstrates more axonal degeneration and epineurial neovascularization whereas CIDP has greater endoneurial inflammation and onion-bulb formation. These findings support the idea that there are differing underlying mechanisms for these disorders, POEMS being related to paraneoplastic vasculopathy associated with angiogenic factors and CIDP related to inflammatory demyelination.
Highlights
The peripheral neuropathy of POEMS Syndrome has great similarities with that of Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP)
We conducted a large retrospective review of nerve biopsies performed at our institution from patients with POEMS syndrome and Piccione et al Acta Neuropathologica Communications (2016) 4:116 from patients with CIDP and regraded them in a blinded fashion to determine if numbers of small epineurial vessels and findings of inflammatory demyelination were useful in separating these disorders
There was a male predominance in the POEMS group (22 males and females, M/F ratio of 1.7:1) and a slight female predominance in the CIDP group (12 males and females, M/F ratio of 1:1.2)
Summary
The peripheral neuropathy of POEMS Syndrome (polyneuropathy, organomegaly, endocrinopathy, Mprotein, and skin changes) has great similarities with that of CIDP (chronic inflammatory demyelinating polyradiculoneuropathy). Both neuropathies cause a motor and sensory, chronic and progressive neuropathy, with polyradicular features (distal and proximal involvement), and with demyelinating electrophysiological features. Because both conditions are treatable and because the treatments for them are different, it is important to get the diagnosis correct as early as possible to ensure good treatment outcome. We conducted a large retrospective review of nerve biopsies performed at our institution from patients with POEMS syndrome and Piccione et al Acta Neuropathologica Communications (2016) 4:116 from patients with CIDP and regraded them in a blinded fashion to determine if numbers of small epineurial vessels and findings of inflammatory demyelination were useful in separating these disorders
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