Morning Report 10/5/17.

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Presentation transcript:

Morning Report 10/5/17

Neuropathy in vasculitis Ischemia tends to happen randomly along nerves, affecting longer nerves first and thus can present quite asymmetrically Mononeuritis multiplex is the most specific presentation for vasculitis; that said, sensorimotor polyneuropathy is also very common and can be asymmetric; it is difficult to distinguish the two without EMG! Of note, neuropathies in vasculitis tend to be painful

Microscopic polyangiitis Characterized by small vessel necrotizing arteritis, without formation of granulomas. Often associated with MPO-ANCA (AKA p-anca) Vessel wall injury is thought to be cell-mediated injury, not immune complex deposition. Gwathmey KG, Burns TM, Collins MP, Bonham Dyck PJ. Vasculitic Neuropathies. Lancet Neurology. 2014;13(1):67-82

Microscopic polyangiitis and neuropathy About 30-40% of microscopic polyangiitis cases will have evidence of nervous system involvement, typically peripheral neuropathy (can be mononeuritis multiplex or polyneuropathy). Rare that this is the initial presenting syndrome of the disease (9% of cases) Zhang W, Zhou G, Shi Q, Zhang X, Zeng F, Zhang C. Clinical analysis of nervous system involvement in the ANCA-associated systemic vasculitides. Clin Exp Rheumatol 2009; 27: S65-S69.

ANCA vasculitis-associated myopathy Mostly just described in case reports as a standalone entity When both muscle and nerve are biopsied (thought to increase diagnostic yield), 30% of cases both muscle and nerve are found to have evidence of disease, and 30% of time disease is only found in muscle (despite clinical neuropathy). So, probably there is quite a bit of subclinical myopathy in patients with ANCA- associated neuropathy Said G, Lacroix C. Primary and secondar vasculitic neuropathy. J Neurol. 2005; 252: 633-641 Said G, Lacroix C. Primary and secondary vasculitic neuropathy. J Neurol. 2005; 252: 633-641

Take home points Vasculitic neuropathies tend to be painful and asymmetric, with associated elevated inflammatory markers You will not always find mononeuritis multiplex as the clinical presentation. Prominent asymmetry should make you suspicious!