REVIEW PAPER
Severe mononeuritis multiplex in a patient with eosinophilic granulomatosis with polyangiitis
1
Clinic of Rheumatology, UMHAT St. Ivan Rilski University Hospital, Sofia, Bulgaria
2
Department of Otolaryngology, Military Medical Academy, Sofia, Bulgaria
Submission date: 2019-08-19
Final revision date: 2019-10-14
Acceptance date: 2019-10-16
Online publication date: 2019-10-31
Publication date: 2019-11-19
Reumatologia 2019;57(5):288-291
KEYWORDS
TOPICS
ABSTRACT
Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic vasculitis characterised by bronchial asthma, hypereosinophilia, and systemic vasculitis. History of asthma with blood eosinophilia and multiorgan involvement are the important clues to suspect EGPA. In the original paper by Churg and Strauss cardiac, gastrointestinal tract, renal, and neurological involvement were noted more frequently. The pattern of neurological involvement may be mononeuritis multiplex, and symmetrical and asymmetrical polyneuropathy. Mononeuritis multiplex was present in 78.1% while cranial nerves were involved in only 4.1% of cases. Glucocorticosteroids and immunosuppressants, especially cyclophosphamide, have considerably improved the prognosis and overall survival rates in patients with systemic vasculitis, including eosinophilic granulomatosis with polyangiitis. The authors present a clinical case of eosinophilic granulomatosis with polyangiitis with severe mononeuritis multiplex. The case reflects the successful application of a cyclophosphamide regime as a remission inducer.
REFERENCES (18)
1.
Masi AT, Hunder GG, Lie JT, et al. The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis). Arthritis Rheum 1990; 33: 1094-1100.
2.
Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised International Chapel Hill Consensus Conference of vasculitides. Arthritis Rheum 2013; 65: 1-11.
3.
Mouthon L, Dunogué B, Guillevin L. Diagnosis and classification of eosinophilic granulomatosis with polyangiitis (formerly named Churg-Strauss syndrome). J Autoimmun 2014; 48-49: 99-103.
4.
Franco DL, Ruff K, Mertz L, et al. Eosinophilic granulomatosis with polyangiitis and diffuse gastrointestinal involvement. Case Rep Gastroenterol 2014; 8: 329-336.
5.
Groh M, Pagnoux C, Baldini C, et al. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA) Consensus Task Force recommendations for evaluation and management. Eur J Intern Med 2015; 26: 545-553.
6.
Churg A. Recent advances in the diagnosis of Churg-Strauss syndrome. Mod Pathol 2001; 14: 1284-1293.
7.
Mohammad N, Wan Ghazali WS. Eosinophilic granulomatosis with polyangiitis and mononeuritis multiplex responded to induction cyclophosphamide. BMJ Case Reports 2017; 2017: bcr-2016-218252.
8.
Churg J, Strauss L. Allergic granulomatosis, allergic angiitis and polyarteritis nodosa. Am J Pathol 1951; 27: 277-301.
9.
Guillevin L, Cohen P, Gayraud M, et al. Churg-Strauss syndrome. Clinical study and long-term follow-up of 96 patients. Medicine (Baltimore) 1999; 78: 26-37.
10.
Kararizou E, Davaki P, Spengos K, et al. Churg-Strauss syndrome complicated by neuropathy: a clinicopathological study of nine cases. Clin Neuropathol 2011; 30: 11-17.
11.
Jeong YJ, Kim KI, Seo IJ, et al. Eosinophilic lung disease: a clinical radiologic and pathologic overview. Radiographics 2007; 27: 617-637.
12.
Francescutti V, Ellis AK, Bourgeois JM, Ward C. Acute acalculous cholecystitis: An usual presenting feature of Churg-Strauss vasculitis. Can J Surg 2008; 51: E129-130.
13.
Sinico RA, Di Toma L, Maggiore M, et al. Prevalence and clinical significance of antineutrophilic cytoplasmic antibodies in Churg-Strauss syndrome. Arthritis Rheum 2005; 52: 2926-2935.
14.
Cohen P, Pagnoux C, Mahr A, et al. Churg-Strauss syndrome with poor-prognosis factors: A prospective multicenter trial comparing glucocorticosteroids and six or twelve cyclophosphamide pulses in forty-eight patients. Arthritis Rheum 2007; 57: 686-693.
15.
Specks U, Merkel PA, Seo P, et al. Efficacy of remission-induction regimens for ANCA-associated vasculitis. N Engl J Med 2013; 369: 417-427.
16.
Bosch X, Guilabert A, Espinosa G, Mirapeix E. Treatment of antineutrophil cytoplasmic antibody associated vasculitis: a systematic review. JAMA 2007; 298: 655-669.
17.
Guillevin L. Vasculitis: Mepolizumab for eosinophilic granulomatosis with polyangiitis. Nat Rev Rheumatol 2017; 13: 518-529.
18.
Yates M, Watts RA, Bajema IM, et al. EULAR/ERA-EDTA recommendations for the management of ANCA-associated vasculitis. Ann Rheum Dis 2016; 75: 1583-1596.
Copyright: © Narodowy Instytut Geriatrii, Reumatologii i Rehabilitacji w Warszawie. This is an Open Access journal, all articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International (CC BY-NC-SA 4.0) License (https://creativecommons.org/licenses/by-nc-sa/4.0/), allowing third parties to copy and redistribute the material in any medium or format and to remix, transform, and build upon the material, provided the original work is properly cited and states its license.
Share
RELATED ARTICLE
| eISSN: | 2084-9834 |
| ISSN: | 0034-6233 |
We process personal data collected when visiting the website. The function of obtaining information about users and their behavior is carried out by voluntarily entered information in forms and saving cookies in end devices. Data, including cookies, are used to provide services, improve the user experience and to analyze the traffic in accordance with the Privacy policy. Data are also collected and processed by Google Analytics tool (more).
You can change cookies settings in your browser. Restricted use of cookies in the browser configuration may affect some functionalities of the website.
You can change cookies settings in your browser. Restricted use of cookies in the browser configuration may affect some functionalities of the website.
