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Deficiency of adenosine deaminase 2 (DADA2): two cases of multisystem vasculitis managed in a South Indian tertiary care centre
  1. Avanish Jha,
  2. Daisy Doley,
  3. John Mathew and
  4. Arvind G Hegde
  1. Clinical Immunology and Rheumatology, Christian Medical College Vellore, Vellore, Tamilnadu, India
  1. Correspondence to Dr John Mathew; johnmathew{at}cmcvellore.ac.in

Abstract

Deficiency of adenosine deaminase 2 (DADA2) is a newly described entity of monogenic vasculitis with multisystem involvement and prominent neurological features. With this report, we are adding to the growing spectrum of cases of DADA2 with two adult cases of early-onset recurrent cerebrovascular events with multisystem involvement. These cases highlight the need for high suspicion of this diagnosis in adults presenting late with symptoms compatible with DADA2. We further report the futility and probable harm of antiplatelet agents in DADA2. Tumour necrosis factor inhibitors have been shown to be beneficial for the treatment and prevention of severe manifestations of this condition.

  • immunology
  • vasculitis
  • biological agents

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Footnotes

  • Contributors AJ was involved in consent and data acquisition, literature search, manuscript writing, review and editing of the manuscript. DD was involved in data acquisition, literature search, and review and editing of the manuscript. JM was involved in the concept, research design, editing and review of the manuscript, and is the guarantor. He is also in charge of intellectual property assignment. AGH was involved in the preparation, editing and review of the manuscript.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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