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