Ankur Jindal: Large-Vessel Inflammation and Immune Dysregulation in Wiskott–Aldrich Syndrome
Ankur Jindal, Immunologist, Rheumatologist and Angioedemologist at Manipal Hospital, shared a post on LinkedIn about a recent article he and his colleagues co-authored, published in JHI, adding:
“Excited to share our recent publication on a rare but devastating complication of Wiskott–Aldrich Syndrome (WAS).
Our team reviewed 80 patients with WAS and identified four children who developed large-vessel vasculopathy involving the aorta and its major branches.
Two patients presented with a Takayasu arteritis-like phenotype and were diagnosed with WAS only after persistent thrombocytopenia prompted further evaluation.
Key findings:
- Large-vessel vasculopathy may be underrecognized in WAS.
- Associated EBV viremia was observed in most cases.
- Early recognition and timely HSCT remain critical to improving survival.
This study highlights the expanding spectrum of immune dysregulation in WAS and the importance of considering inborn errors of immunity in children presenting with atypical vasculitis.”
Title: Large vessel vasculopathy: An underrecognizedcomplication in Wiskott–Aldrich syndrome
Authors: Deepti Suri, Pallavi L. Nadig, Dev Desai, Yamini Sharma, Ahmed Jamal, Gayathri C. Vaitheeswaran, Himanshi Choudhary, Murugan Sudhakar, Ridhima Aggarwal, Suprit Basu, Pratibha Suku, Vibhu Joshi, Rashmi Rikhi, Anmol Bhatia, Akshay Saxena, Manphool Singhal, Manpreet Dhaliwal, Saniya Sharma, Rakesh Kumar Pilania, Ankur Kumar Jindal, Vignesh Pandiarajan, Amit Rawat, Anju Gupta, Surjit Singh
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