Peripheral neuropathy in systemic vasculitis and other autoimmune diseases – a report of five cases emphasizing the importance of etiologic characterization

Abstract

Introduction Peripheral neuropathies may present in the context of systemic vasculitis and other autoimmune diseases. The etiologic characterization is crucial to define the treatment and prognosis in secondary vasculitis. The purpose of this study is to describe the pathway of etiologic investigation including the role of nerve biopsy. Methods Retrospective analysis of patients seen in the neuromuscular outpatient clinic during the last four years with peripheral neuropathy in the context of systemic vasculitis or other autoimmune diseases. Results We present five patients with stepwise progressive sensorimotor deficits of upper and lower limbs. All patients presented with systemic features and one of them had an established diagnosis of systemic vasculitis. They underwent an extended blood panel, including autoimmune and serologic tests. Electromyography and nerve conduction studies revealed asymmetric axonal sensorimotor polyneuropathies in four patients, and an axonal sensorimotor multiple mononeuropathy in one. Four patients underwent nerve biopsy and the other performed a skin biopsy, with findings suggestive of possible vasculitic processes. The etiologies identified included microscopic polyangiitis, HBV-related polyarteritis nodosa and two eosinophilic granulomatosis with polyangiitis. In the last patient a specific etiology could not be established. Conclusion This series reveals the etiologic and phenotypic diversity of peripheral neuropathies related with systemic vasculitis. The therapeutic approach and prognosis were distinct in each patient, emphasizing the importance of a prompt diagnosis and appropriate treatment.

Central and Peripheral Nervous System Complications of Vasculitis Syndromes from Pathology to Bedside: Part 2—Peripheral Nervous System

“…To summarize, based on these histological criteria, it is possible to identify a: (i) definite vasculitis — active: vessel wall infiltrated by inflammatory cell; disruption of endothelium and fragmentation of internal elastic lamina; fibrinoid necrosis; thrombus; (ii) definite vasculitis — chronic: intimal hyperplasia with luminal narrowing; neovascularization; hemosiderin deposition; (iii) probable vasculitis: perivascular inflammation, vascular deposits of complement, IgM or fibrinogen detectable with direct immunofluorescence, accumulation of hemosiderin detectable with Perls’ stain, endoneurial fibrosis indicating fiber loss, asymmetric nerve fiber loss with sectorial involvement, myofiber necrosis and regeneration or infarcts revealed by simultaneously muscle biopsy and not explained by underlying myopathy; (iv) possible vasculitis: absence of perivascular inflammation, nonuniform involvement of the fascicles with sectorial loss of fibers, according to the Collins’ criteria [ 5 ••, 13 ••, 16 , 17 ]. Tables 3 and 4 show the basic histological criteria of VN on biopsy, whereas Tables 5 and 6 summarize the clinical-pathological classification of the PNSV and the categorization of the main vasculitic processes according to the relevant histological findings [ 18 •, 19 •, 20 – 29 , 30 •].…”