SUMMARY
Zwolak Agnieszka, Malicka Joanna, Zwolak Robert, Dudzinska Marta, Swirska Joanna, Daniluk Jadwiga, Tarach Jerzy S., Luczyk Robert. Orbitopatia jako manifestacja choroby IgG4-zaleznej - opis przypadków = Orbitopathy as a manifestation of IgG4-related disease - case report. Journal of Education, Health and Sport. 2016;6(12):475-487. eISSN 2391-8306. DOI http://dx.doi.org/10.5281/zenodo.208373http://ojs.ukw.edu.pl/index.php/johs/article/view/4078 The journal has had 7 points in Ministry of Science and Higher Education parametric evaluation. Part B item 754 (09.12.2016).754 Journal of Education, Health and Sport eISSN 2391-8306 7© The Author (s) 2016;This article is published with open access at Licensee Open Journal Systems of Kazimierz Wielki University in Bydgoszcz, PolandOpen Access. This article is distributed under the terms of the Creative Commons Attribution Noncommercial License which permits any noncommercial use, distribution, and reproduction in any medium,provided the original author(s) and source are credited. This is an open access article licensed under the terms of the Creative Commons Attribution Non Commercial License(http://creativecommons.org/licenses/by-nc/4.0/) which permits unrestricted, non commercial use, distribution and reproduction in any medium, provided the work is properly cited.This is an open access article licensed under the terms of the Creative Commons Attribution Non Commercial License (http://creativecommons.org/licenses/by-nc/4.0/) which permits unrestricted, non commercialuse, distribution and reproduction in any medium, provided the work is properly cited.The authors declare that there is no conflict of interests regarding the publication of this paper.Received: 01.12.2016. Revised 12.12.2016. Accepted: 19.12.2016. Orbitopatia jako manifestacja choroby IgG4-zaleznej - opis przypadkówOrbitopathy as a manifestation of IgG4-related disease - case report Agnieszka Zwolak1,2, Joanna Malicka2, Robert Zwolak3, Marta Dudzinska1, Joanna Swirska1,2, Jadwiga Daniluk1,4, Jerzy S. Tarach2, Robert Luczyk1 1Katedra Interny z Zakladem Pielegniarstwa Internistycznego, Uniwersytet Medyczny w Lublinie1Chair of Internal Medicine and Department of Internal Medicine in Nursing, Medical University of Lublin2Klinika Endokrynologii, Uniwersytet Medyczny w Lublinie2Department of Endocrinology, Medical University of Lublin3Klinika Reumatologii i Ukladowych Chorób Tkanki Lacznej3Department of Rheumatology and Connective Tissue Diseases4Instytut Zdrowia, Panstwowa Szkola Wyzsza im Papieza Jana Pawla II w Bialej Podlaskiej4Instiutute of Public Health, State School of Higher Education in Biala Podlaska StreszczenieWstep: Choroby IgG4-zalezne stanowia multidyscyplinarny problem, ich istota sa nacieki z komórek plazmatycznych z towarzyszacym wlóknieniem zajetych tkanek, a tym tkanek oczodolu. Celem badania jest przedstawienie dwóch przypadków pacjentów z orbitopatia IgG4.Material i metody: Analizy dokonano w oparciu o dokumentacje medyczna pacjentówPrzypadek 1: 68-mezczyzna zostal przyjety do poradni endokrynologicznej z powodu narastajacego wytrzeszczu obu galek ocznych. Badanie MRI potwierdzilo powiekszenie i obrzek wszystkich miesni okoruchowych. Inne choroby, w przebiegu których dochodzic moze do orbitopatii, w tym choroba Graves’a, zostaly wykluczone. W oparciu o podwyzszony poziom przeciwcial IgG4 w surowicy krwi, rozpoznano chorobe IgG4-zalezna. Rozpoczeto leczenie glikokortykosteroidami, w wyniku czego doszlo do znacznej regresji zmian w oczodolach. Leczenie powtórzono po uplywie roku z powodu nawrotu objawów, z nastepowym leczeniem metotreksatem. Do chwili obecnej pacjent pozostaje w calkowitej remisji klinicznej, kontynuuje leczenie metotreksatem z dobra tolerancja.Przypadek 2: 62-letnia kobieta, z choroba Graves’a, po operacji usuniecia guza z prawego oczodolu (hist-pat: sclerosing inflammatory pseudotumor), zostala przyjeta do Kliniki Endokrynologii z powodu nawrotu wytrzeszczu oka prawego. Wykluczono orbitopatie w przebiegu choroby Graves’a. Na podstawie podwyzszonego poziomu przeciwcial IgG4 w surowicy oraz obrazu histopatologicznego usunietej uprzednio zmiany oczodolu rozpoznano chorobe IgG4-zalezna. Rozpoczeto leczenie glikokortykosteroidami, z nastepowa terapia metotrexatem oraz azatiopryna. Pacjentka do chwili obecnej pozostaje w remisji choroby.Wnioski: W przypadku cech orbitopatii w diagnostyce róznicowej nalezy brac pod uwage mozliwosc rzadko wystepujacej patologii, jaka sa choroby IgG4- zalezne. Slowa kluczowe: choroby IgG4-zalezne, orbitopatia, glikokortykosteroidy Abstract Introduction: Immunoglobulin-G4-related disease (IgG4-RD) is a multidisciplinary problem due to plasmatic cells infiltration and areas of fibrosis in the affected tissues, including the eye.The aim of the study was to present two patients with orbitopathy due to IgG4-related eye disease.Material and methods: Analysis of medical records of patients.Case 1: A 68-year old man, was admitted to the endocrinology clinic because of increased protrusion of both eyeballs. The MRI scans revealed enlargement and swallowing of all periorbital muscles. Common diseases of the eyeballs, including Graves disease were excluded. Based on elevated serum IgG4, IgG4-related disease was stated. The glicocorticosteroid therapy was introduced. Evident regression of protrusion of eyeballs was observed. The therapy was repeated one year later because of recurrence of symptoms and methotrexate therapy was added to this. The patient continues now methotrexate therapy only and does not present any symptoms of orbitopathy. Case 2: A 62-years old woman with Graves disease, after surgery of the tumor of the right orbit, was admitted to the Endocrinology Clinic because of right eye protrusion rebound. The Graves orbitopathy was ruled out. Since IgG4 level in plasma was elevated and considering histopathological recognition of the removed tumor of the orbit (sclerosing inflammatory pseudotumor), IgG4-related disease of the eye was stated. Glucocorticosteroids were introduced, followed by methotrexate and azathioprine therapy. The patient remains in remission now.Conclusion: Presented cases highlight the importance of considering IgG4-RD in the differential diagnosis of orbitopathy. Key word: IgG4-related disease, orbitopathy, glucocorticosteroids