Iranian Journal of Allergy, Asthma and Immunology (Aug 2019)

Lupus Erythematosus and Chronic Granulomatous Disease: Report of Four Iranian Patients with AR-CGD and One XL-CGD

  • Marzieh Maddah,
  • Mohammad Reza Fazlollahi,
  • Reza Shiari,
  • Farhad Shahram,
  • Setareh Mamishi,
  • Delara Babaie,
  • Maryam Monajemzadeh,
  • Soheila Sotudeh,
  • Amir Ali Hamidieh,
  • Mohsen Badalzadeh,
  • Shaghayegh Tajik,
  • Leila Sedighipour,
  • Zahra Pourpak

DOI
https://doi.org/10.18502/ijaai.v18i4.1426
Journal volume & issue
Vol. 18, no. 4

Abstract

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Chronic granulomatous disease (CGD) is a rare genetic disorder of neutrophil activity, resulting in increased rate of recurrent infections with catalase–positive bacteria and fungi, as well as various autoimmune diseases such as sarcoidosis, rheumatoid arthritis, and discoid and/or systemic lupus erythematosus. Few reports have reported lupus erythematosus (LE) in patients with X–linked CGD (XL-CGD) and carriers, and very few in autosomal recessive CGD (AR-CGD). Here, we present 5 patients with CGD developing LE at different ages to emphasize on the importance of appropriate follow–up and treatment in patients with CGD with clinical signs and symptoms of autoimmune diseases and even in those with negative serologic results.

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