کد مقاله کد نشریه سال انتشار مقاله انگلیسی ترجمه فارسی نسخه تمام متن
5654881 1589410 2017 3 صفحه PDF سفارش دهید دانلود کنید
عنوان انگلیسی مقاله ISI
IgG4-related disease in autoimmune lymphoproliferative syndrome
موضوعات مرتبط
علوم زیستی و بیوفناوری ایمنی شناسی و میکروب شناسی ایمونولوژی
پیش نمایش صفحه اول مقاله
IgG4-related disease in autoimmune lymphoproliferative syndrome
چکیده انگلیسی


- IgG4-related disease should be considered in ALPS patients.
- Hypergammaglobulinemia is common in ALPS and may mask IgG4-related disease.
- Lymphocytic organ infiltration in ALPS may suggest IgG4-related disease.
- IgG4-related disease should be considered in ALPS patients refractory to rapamycin.

A patient with autoimmune lymphoproliferative disorder (ALPS) developed IgG4-related disease. In retrospect, he had high levels of serum IgG4 for several years prior to presenting with IgG4-related pancreatitis. These high IgG4 levels were masked by hypergammaglobulinemia, a common feature of ALPS. We next screened 18 ALPS patients; four of them displayed increased levels of IgG4. Hence, IgG4-related disease should be considered in ALPS patients, especially in those manifesting lymphocytic organ infiltration or excessive hypergammaglobulinaemia. Screening of IgG4-related disease patients for ALPS-associated mutations would provide further information on whether this disease could be a late-onset atypical presentation of ALPS.

ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Clinical Immunology - Volume 180, July 2017, Pages 97-99
نویسندگان
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