Article Text

This article has a correction. Please see:

PDF
Hyperimmunoglobulinaemia D syndrome in India: report of two siblings with a novel mutation
  1. A Lawrence1,
  2. F Hol2,
  3. A Aggarwal1,
  4. J P H Drenth2
  1. 1Department of Immunology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, India
  2. 2Department of Medicine, Division of Gastroenterology and Hepatology, University Medical Center, St Radboud, Nijmegen, The Netherlands
  1. Correspondence to:
    Dr A Aggarwal
    Department of Immunology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow 226014, India; amita{at}sgpgi.ac.in

Statistics from Altmetric.com

Hyperimmunoglobulinaemia D syndrome (HIDS) is an autosomal recessive auto-inflammatory syndrome caused by mutation in the mevalonate kinase (MVK) gene.1 It presents with febrile episodes starting in infancy with increased serum immunoglobulin (Ig)D levels.2 It is mostly described in people of Dutch or North European descent.3 Isolated cases have been reported from Turkey,4 Japan5 and Qatar.6 No case of HIDS has been reported from South Asia.

A 15-year-old boy from Kerala, India, presented with history of febrile episodes lasting 3–7 days since the age of 3 months. The episodes of fever were variably associated with polyarthritis, abdominal pain, headache, vomiting, diarrhoea, pleuritic chest pain and erythematous papular rash. The episodes occurred at an interval of 1–6 months. He underwent a laparotomy at the age of 8 years, when …

View Full Text

Request permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.

Linked Articles

  • Correction
    BMJ Publishing Group Ltd and European League Against Rheumatism