İmmünglobülin G4- ilişkili sistemik hastalık

Year 2021, Volume: 54 Issue: 1, 135 - 144, 27.04.2021

Erdal Bodakçi , Reşit Yıldırım

https://doi.org/10.20492/aeahtd.809877

Abstract

İmmunglobulin-G4 (IgG4) ilişkili hastalık, esas olarak herhangi bir organı aynı anda veya farklı zamanlarda tutabilen fibro-inflamatuvar bir hastalıktır. Hastalık genellikle bir tümörü taklit eden ve gözyaşı bezlerini, orbitaları, büyük tükürük bezlerini, pankreası, safra kanallarını, retroperitoneal alanı, akciğerleri, böbrekleri, aortu, meninksleri ve tiroid bezini etkileyebilen organ büyümesi ile karşımıza çıkmaktadır. Bu yeni hastalığın arkasındaki immünopatogenez henüz açıklanamamıştır. Hastalığın histopatolojik ayırt edici özellikleri arasında IgG4 pozitif plazma hücrelerinin baskın olduğu yoğun lenfoplazmositik infiltrasyonlar, storiform fibrozis ve obliteratif flebit bulunmaktadır. IgG4-ilişkili hastalık tanısı önemli bir klinik sorundur ve basit bir tanısal test yoktur. Tanıdaki en önemli sorun, IgG4-ilişkili hastalığın sıklıkla hem klinik hem de radyolojik olarak maligniteyi taklit eden bulgularla ortaya çıkabilmesidir. Bu nedenle, yanlışlıkla malignite teşhisini önlemek için IgG4-ilişkili hastalığı, etkilenen organın malign bir tümöründen (kanser veya lenfoma) doğru ve zamanında ayırmak çok önemlidir. Doğru tedavinin verilebilmesi için IgG4-ilişkili hastalığını etkilenen organın benzer inflamatuvar hastalıklarından da ayırmak gereklidir. Pek çok tanı kriterleri önerilmiş ve nihayetinde IgG4-ilişkili hastalık için kapsamlı tanı kriterleri 2011 yılında oluşturulmuştur. Hastalık genellikle steroide iyi yanıt verir, ancak bazen özellikle yüksek risk faktörleri taşıyan hastalarda nüksler görülmektedir. Bu nedenle, aktif hastalığı olanlarda, idame tedavisi veya rituksimab gibi B hücre azaltıcı ajan gerekebilmektedir.

Keywords

İmmünglobülin G4-, IgG4 ilişkili hastalık, storiform fibrozis, lenfoplazmositik inflamasyon

Immunoglobulın G4-related systemic disease

Abstract

IgG4-related disease (IgG4-RD) is a fibro-inflammatory disease that can involve essentially any organ simultaneously or metachronously. The disease often presents with organ enlargement that mimics a tumour and can affect the lacrimal glands, orbits, major salivary glands, pancreas, bile ducts, retroperitoneum, lungs, kidneys, aorta, pachymeninges and thyroid gland. The immunopathogenesis behind this new disease has not yet been clarified. Histopathologic hallmarks of IgG4-RD include dense lymphoplasmacytic infiltrations with a predominance of IgG4-positive plasma cells, storiform fibrosis and obliterative phlebitis. The diagnosis of IgG4-RD is a significant clinical challenge, and there is no simple diagnostic test for IgG4-RD. One problem in its diagnosis is that IgG4-RD frequently presents both clinically and radiologically with findings that mimic malignancy. It is therefore critical to differentiate IgG4-RD from a malignant tumor (cancer or lymphoma) of the affected organ in an accurate and timely manner to avoid a misdiagnosis of malignancy. It is also necessary to differentiate IgG4-RD from similar inflammatory diseases of the affected organ for application of the appropriate therapy. Diagnostic criteria for several IgG4-RDs have been proposed, and comprehensive diagnostic criteria for IgG4-RD were established in 2011. The disease usually responds well to steroids, but sometimes relapses especially in patients carrying high risk factors. Highly active diseases may therefore require maintenance therapy or B-cell depletion agent such as rituximab.

Keywords

Immunoglobulin G4, IgG4-Related disease, storiform fibrosis, lymphoplasmacytic inflammation

References

• 1. Stone JH, Zen Y, Deshpande V. IgG4-related disease. N Engl J Med. 2012;366(6):539-551. doi:10.1056/NEJMra1104650
• 2. Terao C, Ota M, Iwasaki T, et al. IgG4-related disease in the Japanese population: a genome-wide association study. Lancet Rheumatol 2019;1:e14-22. doi:10.1016/S2665-9913(19)30006-2
• 3. Wang L, Zhang P, Zhang X, et al. Sex disparities in clinical characteristics and prognosis of immunoglobulin G4-related disease: a prospective study of 403 patients. Rheumatology(Oxford).2019;58(5):820-830. doi:10.1093/rheumatology/key397
• 4. Karim F, Loeffen J, Bramer W, et al. IgG4-related disease: a systematic review of this unrecognized disease in pediatrics. Pediatr Rheumatol Online J. 2016;14(1):18. Published 2016 Mar 25. doi:10.1186/s12969-016-0079-3
• 5. Maehara T, Moriyama M, Nakamura S. Pathogenesis of IgG4-related disease: a critical review. Odontology. 2019;107(2):127-132. doi:10.1007/s10266-018-0377-y
• 6. Stone JH, Khosroshahi A, Deshpande V, et al. Recommendations for the nomenclature of IgG4-related disease and its individual organ system manifestations. Arthritis Rheum 2012; 64:3061. doi: 10.1002/art.34593
• 7. Kuruma S, Kamisawa T, Tabata T, et al. Allergen-specific IgE antibody serologic assays in patients with autoimmune pancreatitis. Intern Med. 2014;53:541–3. doi.org/10.2169/internalmedicine.53.0963
• 8. Inoue D, Yoshida K, Yoneda N, et al. IgG4-related disease: dataset of 235 consecutive patients. Medicine (Baltimore).2015;94:e680. doi:  10.1097/ MD.0000000000000680
• 9. Wallace ZS, Zhang Y, Perugino CA, et al. Clinical phenotypes of IgG4-related disease: an analysis of two international cross-sectional cohorts. Ann Rheum Dis 2019; 78:406. doi.org/10.1136/annrheumdis-2018-214603
• 10. Cheuk W, Yuen HK, Chu SY, et al. Lymphadenopathy of IgG4-related sclerosing disease. Am J Surg Pathol 2008; 32:671. doi: 10.1097 / PAS.0b013e318157c068.
• 11. Saito Y, Kojima M, Tahata K, et al. Systemic IgG4-related lymphadenopathy: a clinical and pathologic comparison to multicentric Castleman's disease. Mod Pathol. 2009;22:589–99. doi.org/10.1038/modpathol.2009.17
• 12. Sah RP, Chari ST, Pannala R, et al. Differences in clinical profile and relapse rate of type 1 versus type 2 autoimmune pancreatitis. Gastroenterology 2010; 139:140. doi.org/10.1053/j.gastro.2010.03.054
• 13. Hart PA, Levy MJ, Smyrk TC, et al. Clinical profiles and outcomes in idiopathic duct-centric chronic pancreatitis (type 2 autoimmune pancreatitis): the Mayo Clinic experience. Gut 2016; 65:1702. doi.org/10.1136/gutjnl-2015-309275
• 14. Nishimori I, Tamakoshi A, Otsuki M, Research Committee on Intractable Diseases of the Pancreas, Ministry of Health, Labour, and Welfare of Japan. Prevalence of autoimmune pancreatitis in Japan from a nationwide survey in 2002. J Gastroenterol 2007; 42 Suppl 18:6. doi.org/10.1007/s00535-007-2043-y
• 15. Kanno A, Masamune A, Okazaki K, et al. Nationwide epidemiological survey of autoimmune pancreatitis in Japan in 2011. Pancreas. 2015;44:535–9. doi: 10.1097 / MPA.0b013e3182480c99
• 16. Kamisawa T, Zen Y, Pillai S, et al. IgG4-related disease. Lancet. 2015;385(9976):1460–71. doi.org/10.1016/S0140-6736(14)60720-0
• 17 .Koizumi S, Kamisawa T, Kuruma S, et al. Clinical features of IgG4-related dacryoadenitis. Graefes Arch Clin Exp Ophthalmol. 2014;252:491–7. doi.org/10.1007/s00417-013-2541-y
• 18. Masaki Y, Dong L, Kurose N, et al. Proposal for a new clinical entity, IgG4-positive multiorgan lymphoproliferative syndrome: analysis of 64 cases of IgG4-related disorders. Ann Rheum Dis 2009; 68:1310. doi.org/10.1136/ard.2008.089169
• 19. Akiyama M, Kaneko Y, Hayashi Y, Takeuchi T. IgG4-related disease involving vital organs diagnosed with lip biopsy: A case report and literature review. Medicine (Baltimore) 2016; 95:e3970. doi.org/10.1097 / MD.0000000000003970
• 20. Sato Y, Ohshima K, Ichimura K, et al. Ocular adnexal IgG4-related disease has uniform clinicopathology. Pathol Int 2008; 58:465. doi.org/10.1111/j.1440-1827.2008.02257.x
• 21. Ebbo M, Patient M, Grados A, et al. Ophthalmic manifestations in IgG4-related disease: Clinical presentation and response to treatment in a French case-series. Medicine (Baltimore) 2017; 96:e6205. doi:  10.1097 / MD.0000000000006205
• 22. Andrew NH, Sladden N, Kearney DJ, Selva D. An analysis of IgG4-related disease (IgG4-RD) among idiopathic orbital inflammations and benign lymphoid hyperplasias using two consensus-based diagnostic criteria for IgG4-RD. Br J Ophthalmol 2015; 99:376. .doi.org/10.1136/bjophthalmol-2014-305545
• 23. Wallace ZS, Khosroshahi A, Jakobiec FA, et al. IgG4-related systemic disease as a cause of "idiopathic" orbital inflammation, including orbital myositis, and trigeminal nerve involvement. Surv Ophthalmol 2012; 57:26. doi.org/10.1016/j.survophthal.2011.07.004.
• 24. Van Bommel EF, Jansen I, Hendriksz TR, et al. Idiopathic retroperitoneal fibrosis: prospective evaluation of incidence and clinicoradiologic presentation. Medicine (Baltimore). 2009;88:193–201. doi: 10.1097 / MD.0b013e3181afc420
• 25. Khosroshahi A, Carruthers MN, Stone JH, et al. Rethinking Ormond’s disease: “idiopathic” retroperitoneal fibrosis in the era of IgG4-related disease. Medicine (Baltimore). 2013;92:82–91. doi:  10.1097 / MD.0b013e318289610f
• 26. Zen Y, Inoue D, Kitao A, et al. IgG4-related lung and pleural disease: a clinicopathologic study of 21 cases. Am J Surg Pathol. 2009;33:1886–93. doi: 10.1097 / PAS.0b013e3181bd535b
• 27. Kawano M, Saeki T, Nakashima H, et al. Proposal for diagnostic criteria for IgG4-related kidney disease. Clin Exp Nephrol. 2011;15:615–26. doi.org/10.1007/s10157-011-0521-2
• 28. Umehara H, Okazaki K, Masaki Y, et al. Comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD),2011.Mod Rheumatol 2012;22:21–30. doi.org/10.1007/s10165-011-0571-z.
• 29. Brito-Zerón P, Ramos-Casals M, Bosch X, et al. The clinical spectrum of IgG4-related disease. Autoimmun Rev. 2014;13:1203–10. doi.org/10.1016/j.autrev.2014.08.013.
• 30. Guma, Monica, and Gary S. Firestein. "IgG4-related diseases." Best Practice & Research Clinical Rheumatology 26.4(2012):425-438. doi.org/10.1016/j.berh.2012.07.001.
• 31. Khosroshahi A, Wallace ZS, Crowe JL, et al. International Consensus Guidance Statement on the Management and Treatment of IgG4-Related Disease. Arthritis Rheumatol. 2015;67(7):1688-1699. doi:10.1002/art.39132
• 32. Brito-Zerón P, Kostov B, Bosch X, Acar-Denizli N, Ramos-Casals M, Stone JH. Therapeutic approach to IgG4-related disease: A systematic review. Medicine (Baltimore). 2016;95(26):e4002. doi:10.1097/MD.0000000000004002
• 33. Khosroshahi A, Stone JH. Treatment approaches to IgG4-related systemic disease. Curr Opin Rheumatol. 2011;23(1):67-71. doi:10.1097 / BOR. 0b013e328341a240
• 34. Della-Torre E, Stone JH. "How I manage" IgG4-Related Disease. J Clin Immunol. 2016;36(8):754-763. doi:10.1007/s10875-016-0331-0
• 35. Wang L, Zhang P, Wang M, et al. Failure of remission induction by glucocorticoids alone or in combination with immunosuppressive agents in IgG4-related disease: a prospective study of 215 patients. Arthritis Res Ther. 2018;20(1):65. Published 2018 Apr 10. doi:10.1186/s13075-018-1567-2
• 36. Yunyun F, Yu P, Panpan Z, et al. Efficacy and safety of low dose Mycophenolate mofetil treatment for immunoglobulin G4-related disease: a randomized clinical trial. Rheumatology (Oxford). 2019;58(1):52-60. doi:10.1093 / rheumatology / key227
• 37. Ebbo M, Grados A, Samson M, et al. Long-term efficacy and safety of rituximab in IgG4-related disease: Data from a French nationwide study of thirty-three patients. PLoS One. 2017;12(9): e0183844. Published 2017 Sep 15. doi:10.1371 / journal. pone. 0183844
• 38. Kubo K, Yamamoto K. IgG4-related disease. Int J Rheum Dis. 2016;19(8):747-762. doi:10.1111/1756-185X.12586
• 39. Hirano K, Tada M, Sasahira N, et al. Incidence of malignancies in patients with IgG4-related disease. Intern Med. 2014;53(3):171-176 doi:10.2169/internalmedicine.53.1342.
• 40. Kawano M, Saeki T. IgG4-related kidney disease-an update. Curr Opin Nephrol Hypertens.2015;24:193–201. doi:  10.1097 / MNH.0000000000000102