|کد مقاله||کد نشریه||سال انتشار||مقاله انگلیسی||ترجمه فارسی||نسخه تمام متن|
|5654881||1589410||2017||3 صفحه PDF||سفارش دهید||دانلود کنید|
- 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.
Journal: Clinical Immunology - Volume 180, July 2017, Pages 97-99