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VOLUME 65 , ISSUE 3 ( July-September, 2023 ) > List of Articles

BRIEF RESEARCH COMMUNICATION

Churg–Strauss Syndrome

Romman Fatima, Dharampal Bansal, Syed Taha Mahmood, Afra Fatima, Viquasuddin Mohammed

Keywords : Antineutrophilic cytoplasmic antibody, Corticosteroid, Hypoxia, Pulse steroid, Respiratory distress

Citation Information : Fatima R, Bansal D, Mahmood ST, Fatima A, Mohammed V. Churg–Strauss Syndrome. Indian J Chest Dis Allied Sci 2023; 65 (3):167-170.

DOI: 10.5005/jp-journals-11007-0086

License: CC BY-NC 4.0

Published Online: 05-02-2024

Copyright Statement:  Copyright © 2023; The Author(s).


Abstract

Churg–Strauss syndrome an antineutrophilic cytoplasmic antibody (ANCA)-mediated vasculitis has an estimated prevalence of around 10.7 cases per million adults with the median age of presentation being 40 years. It is an uncommon vasculitis to be seen in pediatric age groups which when found has a poorer prognosis due to cardiac involvement and associated manifestations. Its pathophysiology is based on the immune-mediated activation of eosinophils followed by the release of chemokines, such as eotaxin-3, CCL17, major basic protein and many other intermediates that cause tissue damage. The case described, presented with recurrent sinusitis, allergic manifestations with sudden onset purpuric ankle rash and marked hypoxia-induced respiratory distress. Workup revealed marked eosinophilia and very high IgE levels pointing towards ANCA-mediated vasculitis. The patient was managed with pulse dose steroid therapy for remission and maintenance treatment with long-term steroids. The patient was discharged in stable condition with a monthly follow-up with the primary.


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  1. Chakraborty RK, Aeddula NR. Churg-Strauss Syndrome. [Updated 2023 Mar 23]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2023.
  2. Hsieh SC, Yu HS, Cheng SH, et al. Anti-myeloperoxidase antibodies enhance phagocytosis, IL-8 production, and glucose uptake of polymorphonuclear neutrophils rather than anti-proteinase 3 antibodies leading to activation-induced cell death of the neutrophils. Clin Rheumatol 2007;26(2):216–224. DOI: 10.1007/s10067-006-0285-3.
  3. Zwerina J, Bach C, Martorana D, et al. Eotaxin-3 in Churg-Strauss syndrome: A clinical and immunogenetic study. Rheumatology (Oxford) 2011;50(10):1823–1827. DOI: 10.1093/rheumatology/keq445.
  4. Schönermarck U, Csernok E, Trabandt A, et al. Circulating cytokines and soluble CD23, CD26 and CD30 in ANCA-associated vasculitides. Clin Exp Rheumatol 2000;18(4):457–463. PMID: 10949720.
  5. Thiel J, Troilo A, Salzer U, et al. Rituximab as induction therapy in eosinophilic granulomatosis with polyangiitis refractory to conventional immunosuppressive treatment: A 36-month follow-up analysis. J Allergy Clin Immunol Pract 2017;5(6):1556–1563. DOI: 10.1016/j.jaip.2017.07.027.
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