کد مقاله | کد نشریه | سال انتشار | مقاله انگلیسی | ترجمه فارسی | نسخه تمام متن |
---|---|---|---|---|---|
5654881 | 1407298 | 2017 | 3 صفحه PDF | ندارد | دانلود کنید |

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