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Lupus Erythematosus and Chronic Granulomatous Disease: Report of Four Iranian Patients With Ar-Cgd and One Xl-Cgd Publisher Pubmed



Maddah M1 ; Fazlollahi MR1 ; Shiari R2 ; Shahram F3 ; Mamishi S4 ; Babaie D5 ; Monajemzadeh M6 ; Sotoudeh S7 ; Hamidieh AA8 ; Badalzadeh M1 ; Tajik S1 ; Sedighipour L1 ; Pourpak Z1
Authors
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Authors Affiliations
  1. 1. Immunology, Asthma and Allergy Research Institute, Tehran University of Medical Sciences, P.O.Box: 14185-863, Tehran, 14194, Iran
  2. 2. Department of Pediatric Rheumatology, Mofid Children's Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran
  3. 3. Rheumatology Research Center, Shariati Hospital, Tehran University of Medical Sciences, Tehran, Iran
  4. 4. Department of Infectious Diseases, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran
  5. 5. Department of Allergy and Clinical Immunology, Mofid Children's Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran
  6. 6. Department of Pathology, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran
  7. 7. Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran
  8. 8. Department of Pediatric Stem Cell Transplantation, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran

Source: Iranian Journal of Allergy# Asthma and Immunology Published:2019


Abstract

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. © August 2019, Iran J Allergy Asthma Immunol.
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