Non-Systemic Vasculitic Neuropathy: Presentation, Therapeutic Management and Outcome

Benjamin Terrier¹, Fleur Cohen², Aude Rigolet³, Jean-Emmanuel Kahn⁴, Alice Berezne⁵, Olivier Benveniste⁶, Alain Créange⁷, Thierry Maisonobe⁸, Zahir Amoura⁹, Luc Mouthon¹⁰ and Loïc Guillevin for the French Vasculitis Study Group¹⁰, ¹National Referral Center for Rare Systemic Autoimmune Diseases, Cochin Hospital, Paris, France, ²Internal Medicine, Pitié-Salpétrière, Paris, France, ³Department: inflammation, immunopathology and biotherapy (DHU i2B), Assistance Publique - Hôpitaux de Paris, Pitié-Salpêtrière University Hospital, Paris, France, ⁴Internal Medicine, Foch Hospital, Suresnes, France, ⁵Paris Descartes University, Internal Medicine department, Cochin Hospital, Paris, France, ⁶UMR 974, Sorbonne Universités, University Pierre et Marie-Curie-Paris 6, INSERM, Paris, France, ⁷Neurology, Henri Mondor, Créteil, France, ⁸Neuropathology, Pitie-Salpetriere Hospital, Paris, France, ⁹Internal medicine 2, French National Reference Center for Systemic Lupus and Antiphospholipid Syndrome, Pitié-Salpêtrière Hospital (AP-HP), Paris, France, ¹⁰National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, AP–HP, Université Paris Descartes, Paris, Paris, France

Meeting: 2014 ACR/ARHP Annual Meeting

Keywords: corticosteroids, neuropathy and vasculitis

Session Information

Title: Vasculitis

Session Type: Abstract Submissions (ACR)

Background/Purpose

Non-systemic vasculitic neuropathy (NSVN) is a small-to-medium–sized vessel vasculitis limited to the peripheral nervous system. It can be considered a single-organ vasculitis, as recently defined in the revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides in 2012. NSVN is probably an underdiagnosed entity that has rarely been reported. This descriptive study was undertaken to ascertain its presentation, therapeutic management and outcome.

Methods We retrospectively analyzed 18 patients with NSVN fulfilling criteria proposed by Collins in 2010, including: 1) peripheral neuropathy, 2) vasculitis histologically proven on neuromuscular biopsy, 3) possible constitutional symptoms, and 4) absence of systemic manifestations or laboratory markers suggestive of systemic vasculitis or connective tissue disease.

Results

Ten women and 8 men, median age 51 (range 27–83) years, were included. Neurological manifestations were characterized by acute onset (78%), mononeuritis multiplex (88%), polyneuropathy (12%), sensory impairment (100%) and motor impairment (88%). Neurological manifestations were painful (88%) and asymmetrical (78%). Nerve trunks involved were external popliteal (94%), internal popliteal (44%), ulnar (33%), radial (17%) and median (11%). General symptoms included asthenia and weight loss >10% (22% each), arthralgias (12%) and fever (6%). Only 2 patients had elevated inflammatory parameters. Neuromuscular biopsy showed, in muscle or nerve, medium-sized-vessel vasculitis (56%) or small-sized-vessel vasculitis (44%), with fibrinoid necrosis (88%). Corticosteroids were prescribed to 94%, and their NSVN regressed significantly (65%), stabilized (2%) or progressed (24%). Cyclophosphamide was added to corticosteroids for refractory patients. Relapsing patients received methotrexate or azathioprine, with 3/4 patients showing improvement. After median follow-up of 47 months, neurological sequelae were noted in 83%, including sensory sequelae in 15 patients and motor sequelae in 6.

Conclusion

The results of this study describing NSVN presentation, therapeutic management and outcome indicated that acute onset, painful and asymmetrical manifestations are suggestive of NSVN. Half of the patients required immunosuppressive agents because of relapsing and/or refractory disease. Sensory and motor sequelae were common, suggesting that early diagnosis and initiation of therapy are critical.

Disclosure:

B. Terrier,
None;

F. Cohen,
None;

A. Rigolet,
None;

J. E. Kahn,
None;

A. Berezne,
None;

O. Benveniste,
None;

A. Créange,
None;

T. Maisonobe,
None;

Z. Amoura,
None;

L. Mouthon,
None;

L. Guillevin for the French Vasculitis Study Group,
None.

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