Login Register Cart 0
Generic placeholder image

Current Rheumatology Reviews

Editor-in-Chief

ISSN (Print): 1573-3971
ISSN (Online): 1875-6360

Back
Journal Home About Journal Editorial Board Journal Insight Current Issue Volumes/Issues
Subscribe
Case Report

A Case of Eosinophilic Granulomatosis with Polyangiitis Presenting with Mononeuritis Multiplex

Author(s): Md. Adnanul Alam*, Md. Ismail Hossain, Abed Hussain khan and Shohael Mahmud Arafat

Volume 18, Issue 4, 2022

Published on: 10 May, 2022

Page: [368 - 372] Pages: 5

DOI: 10.2174/1573397118666220330005431

Price: $65

conference banner
Abstract

Background: Eosinophilic granulomatosis with polyangiitis (EGPA), also known as Churg Strauss syndrome, is an uncommon vasculitis associated with antineutrophil cytoplasmic antibody (ANCA). The hallmarks of the disease are asthma, eosinophilia, and systemic vasculitis with varying degrees of neurological, cutaneous, cardiac, gastrointestinal, and renal involvement. Diagnosis is often difficult since the symptoms are diverse, and a number of differentials need to be excluded.

Case Presentation: In this report, we describe a 60-year-old patient who presented with mononeuritis multiplex and a painful skin rash. A history of late-onset asthma, which was poorly controlled, led us to suspect EGPA. Laboratory data showed leukocytosis, eosinophilia (>10%), elevated ESR, CRP, and IgE, normal chest Xray, positive rheumatoid factor (RA), perinuclear anti-neutrophil cytoplasmic antibody (p-ANCA), and evidence of extravascular eosinophils in histopathology report of skin biopsy. She was treated with methylprednisolone and cyclophosphamide pulse therapy with a satisfactory response.

Conclusion: Diagnosis of EGPA requires a combination of clinical and histopathological findings to meet the diagnostic criteria. A history of poorly controlled or late-onset asthma may guide us to the diagnosis that is frequently overlooked. Due to the wide heterogeneity of EGPA patients' phenotypes, sharp, professional judgment is needed for early disease detection and treatment in order to avoid irreversible changes and poor outcomes.

Keywords: Eosinophilic granulomatosis with polyangiitis, Churg-Strauss syndrome, mononeuritis multiplex, asthama, leukocytosis, eosinophilia.

« Previous Next »
Graphical Abstract

[1]
Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised international chapel hill consensus conference nomenclature of vasculitides. Arthritis Rheum 2013; 65(1): 1-11.
[http://dx.doi.org/10.1002/art.37715] [PMID: 23045170]
[2]
Trivioli G, Terrier B, Vaglio A. Eosinophilic granulomatosis with polyangiitis: Understanding the disease and its management. Rheumatology (Oxford) 2020; 59 (Suppl. 3): iii84-94.
[http://dx.doi.org/10.1093/rheumatology/kez570] [PMID: 32348510]
[3]
Mouthon L, Dunogue B, Guillevin L. Diagnosis and classification of eosinophilic granulomatosis with polyangiitis (formerly named Churg-Strauss syndrome). J Autoimmun 2014; 48-49: 99-103.
[http://dx.doi.org/10.1016/j.jaut.2014.01.018] [PMID: 24530234]
[4]
Zhang Z, Liu S, Guo L, et al. Clinical characteristics of peripheral neuropathy in eosinophilic granulomatosis with polyangiitis: A retrospective single-center study in China. J Immunol Res 2020; 20203530768
[http://dx.doi.org/10.1155/2020/3530768] [PMID: 32714994]
[5]
Berti A, Boukhlal S, Groh M, Cornec D. Eosinophilic granulomatosis with polyangiitis: The multifaceted spectrum of clinical manifestations at different stages of the disease. Expert Rev Clin Immunol 2020; 16(1): 51-61.
[http://dx.doi.org/10.1080/1744666X.2019.1697678 ] [PMID: 31762336]
[6]
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(8): 1094-100.
[http://dx.doi.org/10.1002/art.1780330806] [PMID: 2202307]
[7]
Robson J, Grayson P, Ponte C, et al. 110. Classification criteria for the ANCA-associated vasculitides. Rheumatology 2019; 58 (Suppl. 2): kez058-.
[http://dx.doi.org/10.1093/rheumatology/kez058.050]
[8]
Vaglio A, Buzio C, Zwerina J. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss): State of the art. Allergy 2013; 68(3): 261-73.
[http://dx.doi.org/10.1111/all.12088] [PMID: 23330816]
[9]
Frumholtz L, Laurent-Roussel S, Aumaître O, et al. Clinical and pathological significance of cutaneous manifestations in ANCA-associated vasculitides. Autoimmun Rev 2017; 16(11): 1138-46.
[http://dx.doi.org/10.1016/j.autrev.2017.09.009] [PMID: 28918161]
[10]
Marques CC, Fernandes EL, Miquelin GM, Colferai MMT. Cutaneous manifestations of Churg-Strauss syndrome: Key to diagnosis. An Bras Dermatol 2017; 92(5) (Suppl. 1): 56-8.
[http://dx.doi.org/10.1590/abd1806-4841.20175522 ] [PMID: 29267447]
[11]
Kalinova D, Kukushev G, Kolarov Z, Rashkov R. Severe mononeuritis multiplex in a patient with eosinophilic granulomatosis with polyangiitis. Reumatologia 2019; 57(5): 288-91.
[http://dx.doi.org/10.5114/reum.2019.89522] [PMID: 31844342]
[12]
Marzano AV, Raimondo MG, Berti E, Meroni PL, Ingegnoli F. Cutaneous manifestations of ANCA-associated small vessels vasculitis. Clin Rev Allergy Immunol 2017; 53(3): 428-38.
[http://dx.doi.org/10.1007/s12016-017-8616-5] [PMID: 28578472]
[13]
Comarmond C, Pagnoux C, Khellaf M, et al. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss): Clinical characteristics and long-term followup of the 383 patients enrolled in the French Vasculitis Study Group cohort. Arthritis Rheum 2013; 65(1): 270-81.
[http://dx.doi.org/10.1002/art.37721] [PMID: 23044708]
[14]
Sulaiman W, Seung OP, Noor SM. Acute myocardial infarction as Eosinophilic granulomatosis with polyangiitis (formerly Churg Strauss syndrome) initial presentation. Rev Bras Reumatol 2014; 54(5): 393-6.
[http://dx.doi.org/10.1016/j.rbr.2013.03.002] [PMID: 25627304]

Rights & Permissions Print Cite
Article Metrics
25
2
Wayfinder Image
© 2024 Bentham Science Publishers | Privacy Policy