Autoimmunity in Patients with Hyper IgM Syndrome

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Delavari Samaneh; Moeini Shad Tannaz; Pashangzadeh salar

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Journal Paper

Paper Information

Journal: Immunology and Genetics Journal Year:2019 | Volume:2 | Issue:4 Page(s): 42-51

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Information Journal Paper

Title

Autoimmunity in Patients with Hyper IgM Syndrome

Author(s)

Delavari Samaneh | Moeini Shad Tannaz | Pashangzadeh salar | Issue Writer Certificate

Keywords

Hyper IgM Syndrome

Autoimmunity

Immune thrombocytopenia purpura

CSR defects

Abstract

Background/objectives: hyper-IgM (HIGM) syndrome is characterized by normal to increased serum IgM, as well as very low or undetectable IgG, IgA, and IgE. HIGM (also known as class-switch recombination (CSR) defects) patients indicate different clinical manifestations such as autoimmune disorders. The present study aimed to evaluate demographic data, clinical manifestation, and immunological findings in HIGM patients. Methods: Clinical features and immunological data were collected from medical records belonged to the 79 Iranian HIGM patients diagnosed in Children’ s Medical Center in Iran. To compare clinical records and laboratory data, all HIGM patients were classified into two different groups as follows: patients with autoimmune disease and patients without autoimmune diseases. Results: A total of 79 patients (60 male and 19 female) with median (IQR) age of 12 years old at the time of the study were enrolled (6-22. 45). Autoimmunity diseases were seen in 19 patients (23. 75%, 3 females and 16 males). Among the noninfectious manifestations, the hepatomegaly and spelenomegaly were significantly higher in the patients with Autoimmunity (p= 0. 006), compared to the patients without Autoimmunity (p=0. 006). The most common autoimmune presentations among HIGM patients were ITP (32%), juvenile rheumatoid arthritis (16%), autoimmune hemolytic anemia (11%), Sclerosing cholangitis (11%), Gullain-Barré syndrome, Evans syndrome, diabetes mellitus, and chrohn’ s disease. Conclusions: The relationship between HIGM syndrome and Autoimmunity disorders could lead to sever clinical complications. Therefore, we suggested that immunologists should be aware of this complications.

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APA: Copy

Delavari, Samaneh, Moeini Shad, Tannaz, & Pashangzadeh, salar. (2019). Autoimmunity in Patients with Hyper IgM Syndrome. IMMUNOLOGY AND GENETICS JOURNA, 2(4), 42-51. SID. https://sid.ir/paper/754012/en

Vancouver: Copy

Delavari Samaneh, Moeini Shad Tannaz, Pashangzadeh salar. Autoimmunity in Patients with Hyper IgM Syndrome. IMMUNOLOGY AND GENETICS JOURNA[Internet]. 2019;2(4):42-51. Available from: https://sid.ir/paper/754012/en

IEEE: Copy

Samaneh Delavari, Tannaz Moeini Shad, and salar Pashangzadeh, “Autoimmunity in Patients with Hyper IgM Syndrome,” IMMUNOLOGY AND GENETICS JOURNA, vol. 2, no. 4, pp. 42–51, 2019, [Online]. Available: https://sid.ir/paper/754012/en

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