The Clinicopathologic Spectrum of IgG4-Related Disease

Abstract

Immunoglobulin G4-related disease is a fibroinflammatory systemic disease that is characterized by focal or diffuse organ infiltration by immunoglobulin G4-bearing plasma cells. Immunoglobulin G4-related disease may affect any organ, and a high index of suspicion is necessary for early detection to avoid irreversible fibrosis, organ dysfunction, and death. Tumor-forming lesions are common radiological features of immunoglobulin G4-related disease that need to be differentiated from malignancies. The diagnostic approach requires the integration of clinical, biochemical, and radiographic manifestations with classic histopathologic features, which remain crucial to diagnosis. The histology of immunoglobulin G4-related disease is determined by a dense lymphoplasmacytic infiltrate, storiform fibrosis, and obliterative phlebitis in the presence of increased immunoglobulin G4-positve plasma cells. Although immunoglobulin G4-related disease forms a distinct, clinically independent disease category, many questions and problems remain unanswered, especially on its pathogenesis and the role of immunoglobulin G4. Advances in the understanding of immunoglobulin G4-related disease are likely to change the diagnostic approach in the future and create potential targets for therapeutic purposes. Here we describe the concept of immunoglobulin G4-related disease and the most recent knowledge in the clinico-pathological characteristics on this emerging disease. This study can guide clinicians in early diagnosis and prevent unnecessary surgical resections.

Keywords

• Autoimmune, diagnosis, IgG4, IgG4-related disease, pathology, treatment

Kaynakça

1. 1. Sarles H, Sarles JC, Muratore R, Guien C. Chronic inflammatory sclerosis of the pancreas--an autonomous pancreatic disease? Am J Dig Dis 1961;6:688-98.
2. 2. Comings DE, Skubi KB, Van Eyes J, Motulsky AG. Familial multifocal fibrosclerosis: Findings suggesting that retroperitoneal fibrosis, mediastinal fibrosis, sclerosing cholangitis, riedel’s thyroiditis, and pseudotumor of the orbit may be different manifestations of a single disease. Ann Intern Med 1967;66:884-92.
3. 3. Kamisawa T, Funata N, Hayashi Y, Tsuruta K, Okamoto A, Amemiya K, et al. Close relationship between autoimmune pancreatitis and multifocal fibrosclerosis. Gut 2003;52:683-7.
4. 4. Wallace ZS, Deshpande V, Mattoo H, Mahajan VS, Kulikova M, Pillai S, et al. IgG4- Related Disease: clinical and laboratory features in 125 patients. Arthritis Rheumatol 2015;67:2466-75.
5. 5. Kamisawa T, Zen Y, Pillai S, Stone JH. IgG4-related disease. Lancet 2015;385:1460-71.
6. 6. Stone JH, Zen Y, Deshpande V. IgG4-Related Disease. N Eng J Med 2012;366:539-51.
7. 7. Zen Y, Nakanuma Y. Pathogenesis of IgG4-related disease. Curr Opin Rheumatol 2011;23:114-8.
8. 8. Okazaki K, Umehara H. Current Concept of IgG4-Related Disease. Curr Top Microbiol Immunol 2017;401:1-17.

9. 9. Deshpande V, Zen Y, Chan JK, Yi EE, Sato Y, Yoshino T, et al. Consensus statement on the pathology of IgG4-related disease. Mod pathol 2012;25:1181-92.
10. 10. Aalberse RC, Stapel SO, Schuurman J, Rispens T. Immunoglobulin G4: an odd antibody. Clin exp allerg 2009;39:469-77.
11. 11. Durham SR, Emminger W, Kapp A, Colombo G, de Monchy JG, Rak S, et al. Longterm clinical efficacy in grass pollen-induced rhinoconjunctivitis after treatment with SQ-standardized grass allergy immunotherapy tablet. J Allergy Clin Immunol 2010;125:131-8.
12. 12. Mattoo H, Della-Torre E, Mahajan VS, Stone JH, Pillai S. Circulating Th2 memory cells in IgG4-related disease are restricted to a defined subset of subjects with atopy. Allergy 2014;69:399-402.
13. 13. Kamisawa T, Anjiki H, Egawa N, Kubota N. Allergic manifestations in autoimmune pancreatitis. Eur J Gastroenterol Hepatol 2009;21:1136-9.
14. 14. Zen Y, Fujii T, Harada K, Kawano M, Yamada K, Takahira M, et al. Th2 and regulatory immune reactions are increased in immunoglobin G4-related sclerosing pancreatitis and cholangitis. Hepatology 2007;45:1538-46.
15. 15. Jeannin P, Lecoanet S, Delneste Y, Gauchat JF, Bonnefoy JY. IgE versus IgG4 production can be differentially regulated by IL-10. J Immunol 1998;160:3555-61.
16. 16. Yamamoto M, Takahashi H, Shinomura Y. Mechanisms and assessment of IgG4- related disease: lessons for the rheumatologist. Nat Rev Rheumatol 2013;10:148-59.
17. 17. Della Torre E, Mattoo H, Mahajan VS, Carruthers M, Pillai S, Stone JH. Prevalence of atopy, eosinophilia, and IgE elevation in IgG4-related disease. Allergy 2014;69:269-72.
18. 18. Chen H, Lin W, Wang Q, Wu Q, Wang L, Fei Y, et al. IgG4-related disease in a Chinese cohort: a prospective study. Scand J Rheumatol 2014;43:70-4.
19. 19. Martinez-Valle F, Fernandez-Codina A, Pinal-Fernandez I, Orozco-Galvez O, Vilardell-Tarres M. IgG4-related disease: Evidence from six recent cohorts. Autoimmun Rev 2017;16:168-72.
20. 20. Haldar D, Cockwell P, Richter AG, Roberts KJ, Hirschfield GM. An overview of the diagnosis and management of immunoglobulin G4-related disease. CMAJ 2016;188:953-61.
21. 21. Zhang J, Chen H, Ma Y, Xiao Y, Niu N, Lin W, et al. Characterizing IgG4-related disease with 18F-FDG PET/CT: a prospective cohort study. Eur J Nucl Med Mol Imag 2014;41:1624-34.
22. 22. Vasaitis L. IgG4-related disease: A relatively new concept for clinicians. Eur J Int Med 2016;27:1-9.
23. 23. Toyoda K, Oba H, Kutomi K, Furui S, Oohara A, Mori H, et al. MR imaging of IgG4-related disease in the head and neck and brain. AJNR Am J Neuroradiol 2012;33:2136-9.
24. 24. Umehara H, Okazaki K, Masaki Y, Kawano M, Yamamoto M, Saeki T, et al. Comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD), 2011. Mod Rheumatol 2012;22:21-30.
25. 25. Hamano H, Kawa S, Horiuchi A, Unno H, Furuya N, Akamatsu T, et al. High serum IgG4 concentrations in patients with sclerosing pancreatitis. N Eng J Med 2001;344:732-8.
26. 26. Sah RP, Chari ST, Pannala R, Sugumar A, Clain JE, Levy MJ, et al. Differences in clinical profile and relapse rate of type 1 versus type 2 autoimmune pancreatitis. Gastroenterol 2010;139:140-8.
27. 27. Hart PA, Kamisawa T, Brugge WR, Chung JB, Culver EL, Czako L, et al. Longterm outcomes of autoimmune pancreatitis: a multicentre, international analysis. Gut 2013;62:1771-6.
28. 28. Sugumar A, Chari ST. Autoimmune pancreatitis. J Gastroenterol Hepatol. 2011;26:1368-73.
29. 29. Majumder S, Takahashi N, Chari ST. Autoimmune Pancreatitis. Dig Dis Sci 2017;62:1762-9.
30. 30. Kamisawa T, Egawa N, Inokuma S, Tsuruta K, Okamoto A, Kamata N, et al. Pancreatic endocrine and exocrine function and salivary gland function in autoimmune pancreatitis before and after steroid therapy. Pancreas 2003;27:235-8.
31. 31. Nakazawa T, Naitoh I, Hayashi K, Okumura F, Miyabe K, Yoshida M, et al. Diagnostic criteria for IgG4-related sclerosing cholangitis based on cholangiographic classification. J Gastroenterol 2012;47:79-87.
32. 32. Mendes FD, Jorgensen R, Keach J, Katzmann JA, Smyrk T, Donlinger J, et al. Elevated serum IgG4 concentration in patients with primary sclerosing cholangitis. Am J Gastroenterol 2006;101:2070-5.
33. 33. Oseini AM, Chaiteerakij R, Shire AM, Ghazale A, Kaiya J, Moser CD, et al. Utility of serum immunoglobulin G4 in distinguishing immunoglobulin G4-associated cholangitis from cholangiocarcinoma. Hepatology 2011;54:940-8.
34. 34. Kalaitzakis E, Levy M, Kamisawa T, Johnson GJ, Baron TH, Topazian MD, et al. Endoscopic retrograde cholangiography does not reliably distinguish IgG4-associated cholangitis from PSC or cholangiocarcinoma. Clin Gastroenterol Hepatol 2011;9:800-3.