THE SPECTRUM OF IGG4-RELATED DISEASES

IgG4-related disease (IgG4-RD) is now recognized as a worldwide disease. It is a rare systemic fibroinflammatory disorder. The evaluation for IgG4-RD should include a comprehensive clinical history, physical examination, and selected laboratory investigation, along with appropriate radiologic studi...

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Main Authors: Inimioara Mihaela Cojocaru, Manole Cojocaru
Format: Article
Language:English
Published: Amaltea Medical Publishing House 2018-09-01
Series:Romanian Journal of Medical Practice
Subjects:
Online Access:https://revistemedicale.amaltea.ro/Romanian_Journal_of_MEDICAL_PRACTICE/Practica_medicala-2018-Nr.3/RJMP_2018_3_Art-03.pdf
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author Inimioara Mihaela Cojocaru
Manole Cojocaru
author_facet Inimioara Mihaela Cojocaru
Manole Cojocaru
author_sort Inimioara Mihaela Cojocaru
collection DOAJ
description IgG4-related disease (IgG4-RD) is now recognized as a worldwide disease. It is a rare systemic fibroinflammatory disorder. The evaluation for IgG4-RD should include a comprehensive clinical history, physical examination, and selected laboratory investigation, along with appropriate radiologic studies. A wide variety of organs can be involved in IgG4-RD. Confirmation of the diagnosis by biopsy is important for the exclusion of malignancy and other disorders that may mimic IgG4-RD. Consequently, diagnostic criteria for IgG4-RD have been proposed recently. The hallmarks of IgG4-RD are lymphoplasmacytic tissue infiltration of mainly IgG4-positive plasma cells and small lymphocytes, which may be accompanied by fibrosis, obliterative phlebitis, and, in the majority of patients, elevated serum levels of IgG4. The serum IgG4 level is elevated above the upper limit of normal (>135 mg/dL). The serum IgG4 concentration tends to increase with the number of organs involved and usually decreases after treatment with glucocorticoids. Among several autoantibodies identified so far, autoantibodies against lactoferrin and carbonic anhydrase II are most frequently detected in serum of IgG4-disease patients. Glucocorticoids, azathioprine, micophenolate mofetil, methotrexate and rituximab are therapeutical options.
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spelling doaj.art-5a1cf1e47dec4d5c97d718afab683f8f2022-12-21T21:26:36ZengAmaltea Medical Publishing HouseRomanian Journal of Medical Practice1842-82582069-61082018-09-0113318919610.37897/RJMP.2018.3.3THE SPECTRUM OF IGG4-RELATED DISEASESInimioara Mihaela Cojocaru0Manole Cojocaru1“Carol Davila” University of Medicine and Pharmacy, Bucharest; Department of Neurology, “Colentina” Clinical Hospital, Bucharest“Titu Maiorescu” University, Faculty of Medicine, BucharestIgG4-related disease (IgG4-RD) is now recognized as a worldwide disease. It is a rare systemic fibroinflammatory disorder. The evaluation for IgG4-RD should include a comprehensive clinical history, physical examination, and selected laboratory investigation, along with appropriate radiologic studies. A wide variety of organs can be involved in IgG4-RD. Confirmation of the diagnosis by biopsy is important for the exclusion of malignancy and other disorders that may mimic IgG4-RD. Consequently, diagnostic criteria for IgG4-RD have been proposed recently. The hallmarks of IgG4-RD are lymphoplasmacytic tissue infiltration of mainly IgG4-positive plasma cells and small lymphocytes, which may be accompanied by fibrosis, obliterative phlebitis, and, in the majority of patients, elevated serum levels of IgG4. The serum IgG4 level is elevated above the upper limit of normal (>135 mg/dL). The serum IgG4 concentration tends to increase with the number of organs involved and usually decreases after treatment with glucocorticoids. Among several autoantibodies identified so far, autoantibodies against lactoferrin and carbonic anhydrase II are most frequently detected in serum of IgG4-disease patients. Glucocorticoids, azathioprine, micophenolate mofetil, methotrexate and rituximab are therapeutical options.https://revistemedicale.amaltea.ro/Romanian_Journal_of_MEDICAL_PRACTICE/Practica_medicala-2018-Nr.3/RJMP_2018_3_Art-03.pdfigg4igg4-related diseases(igg4-rd)diagnosis
spellingShingle Inimioara Mihaela Cojocaru
Manole Cojocaru
THE SPECTRUM OF IGG4-RELATED DISEASES
Romanian Journal of Medical Practice
igg4
igg4-related diseases
(igg4-rd)
diagnosis
title THE SPECTRUM OF IGG4-RELATED DISEASES
title_full THE SPECTRUM OF IGG4-RELATED DISEASES
title_fullStr THE SPECTRUM OF IGG4-RELATED DISEASES
title_full_unstemmed THE SPECTRUM OF IGG4-RELATED DISEASES
title_short THE SPECTRUM OF IGG4-RELATED DISEASES
title_sort spectrum of igg4 related diseases
topic igg4
igg4-related diseases
(igg4-rd)
diagnosis
url https://revistemedicale.amaltea.ro/Romanian_Journal_of_MEDICAL_PRACTICE/Practica_medicala-2018-Nr.3/RJMP_2018_3_Art-03.pdf
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