Central nervous system damage in IgG4-related disease: review
https://doi.org/10.17650/1683-3295-2020-22-3-84-101
Abstract
IgG4-related disease is a new and recently described and determined disease involving multiple organs. Its etiology and pathogenesis are still not fully understood. However according to multiple studies this disease should be considered as a unique pathological autoimmune unity involving various organs and tissues. IgG4-reladted disease was first considered as a form of pancreatic inflammatory process and diagnosed as IgG4-related autoimmune pancreatitis. However current data demonstrate the potential involvement of almost all tissues and organs including central nervous system. Diagnostic criteria are being reviewed annually resulting in IgG4-related disease diagnosis in many considered differently cases. This review presents current literature data related to IgG4-related disease etiology and pathogenesis, diagnostic criteria and potential forms of central nervous system IgG4-related injury.
About the Authors
V. A. Smirnov
N.V. Sklifosovsky Research Institute for Emergency Medicine, Moscow Healthcare Department
Russian Federation
Russian Federation
3 Bolshaya Sukharevskaya Sq., Moscow 129090
A. A. Grin
N.V. Sklifosovsky Research Institute for Emergency Medicine, Moscow Healthcare Department
Russian Federation
Russian Federation
3 Bolshaya Sukharevskaya Sq., Moscow 129090
References
1. Buyeverov A.O., Kucheryavy Yu.A. IgG4-associated disease. Moscow: Forte Print, 2014. 128 p. (In Russ.).
2. Okamoto T., Kamisawa A. Autoimmune pancreatitis: proposal of IgG4-related sclerosis disease. J Gastroenterol 2006;41:613–25.
3. Yamamoto M., Takahashi H., Ohara M. et al. A new conceptualization for Mikulicz’s disease as an IgG4-related plasmacytic disease. Mod Rheumatol 2006;16(6):335–40.
4. Masaki Y., Dong L., Kurose N. et al. Proposal for a new clinical entity, IgG4-positive multi-organ lymphoproliferative syndrome: analysis of 64 cases of IgG4-related disorders. Ann Rheum Dis 2009;68(8):1310–5.
5. Okazaki K., Uchida K., Ikeura T., Takaoka M. Current concept and diagnosis of IgG4-related disease in the hepato-bilio-pancreatic system. J Gastroenterol 2013;48(3):303–14.
6. Mikulicz J. Uber eine eigenartige symmetrishe Erkrankung der Tranen und Mundspeicheldrusen. Stuttgart: Beitr z Chir Fesrschr f Theodor Billroth, 1892. Pp. 610–630.
7. Küttner H. Über entzundiche Tumoren der Submanxillarspeicheldruse. Bruns Beitr Klin Chir 1896;(15):815–34.
8. Thal A.P., Murray M.J., Egner W. Isoantibody formation in chronic pancreatic disease. Lancet 1959;1(7083):1128–9.
9. Sarles H., Sarles J.C., Muratore R., Guien C. Chronic inflammatory sclerosis of the pancreas – an autonomous pancreatic disease? Am J Dig Dis 1961;6:688–98.
10. Comings D.E., Skubi K.B., Ven Eyes J., Motulsky A.G. Familial multifocal fibrosclerosis. Ann Intern Med 1967;66:884–92.
11. Waldram R., Kopelman H., Tsantoulas D., Williams R. Chronic pancreatitis, sclerosing cholangitis, and sicca complex in two siblings. Lancet 1975;1(7906):550–2.
12. Børkje B., Odegaard S., Vetvik K., Schrumpf E. Chronic pancreatitis associated with ulcerative colitis and sclerosing cholangitis. A case report. Tidsskr Nor Laegeforen 1984;104(8):585–7.
13. Montefusco P.P., Geiss A.C., Bronzo R.L. et al. Sclerosing cholangitis, chronic pancreatitis and Sjorgren’s syndrome: a syndrome complex. Am J Surg 1984;147(6):822–6.
14. Versapuech J.M., Labayle D., Grange D. et al. Sclerosing cholangitis, chronic pancreatitis and Sjorgren’s syndrome. Ann Med Interne (Paris) 1986;137(2):147–51.
15. Sarles H. Classification and definition of pancreatitis. Marseilles-Rome 1988. Gastroenterol Clin Biol 1989;13(11):857–9.
16. Yoshida K., Toki F., Takeuchi T. et al. Chronic pancreatitis caused by an autoimmune abnormality. Proposal of the concept of autoimmune pancreatitis. Dig Dis Sci 1995;40:1561–8.
17. Mayev I.V., Kucheryavy Yu.A., Oganesyan T.S. Autoimmune pancreatitis. Textbook. Moscow, 2011. 80 p. (In Russ.).
18. Etemad B., Whitcomb D.C. Chronic pancreatitis: diagnosis, classification and new genetic developments. Gastroenterol 2001;120:682–707.
19. Hamano H., Kawa S., Horiuchi A. et al. High serum IgG4 concentrations in patients with sclerosing pancreatitis. N Engl J Med 2001;344(10):732–8.
20. Kamisawa T., Funata N., Hayashi Y. et al. A new clinicopathological entity of IgG4-related autoimmune disease. J Gastroenterol 2003;38(10):982–4.
21. Members of the Criteria Committee for Autoimmune Pancreatitis of the Japan Pancreas Society. Diagnostic criteria for autoimmune pancreatitis by the Japan Pancreas Society (2002). J Jpn Pancreas Soc (Suizou) 2002;17:585–7.
22. Okazaki K., Kawa S., Kamisawa T. et al. Clinical diagnostic criteria of autoimmune pancreatitis: revised proposal. J Gastroenterol 2006;41:626–31.
23. Chari S.T., Smyrk T.C., Levy M.J. et al. Diagnosis of autoimmune pancreatitis: the Mayo Clinic experience. Clin Gastroenterol Hepatol 2006;4:1010–6.
24. Kim K., Kim M., Kim J. et al. Diagnostic criteria for autoimmune chronic pancreatitis revisited. Word J Gastroenterol 2006;12(16):2487–96.
25. Otsuki M., Chung J.B., Okazaki K. et al. Research Committee of Intractable Pancreatic Diseases provided by the Ministry of Health, Labour and Welfare of Japan and the Korean Society of Pancreatobiliary Diseases. Asian diagnostic criteria for autoimmune pancreatitis: consensus of the JapanKorea Symposium on Autoimmune Pancreatitis. J Gastroenterol 2008;43(6):403–8.
26. Frulloni L., Scattolini C., Falconi M. et al. Autoimmune pancreatitis: differences between the focal and diffuse forms in 87 patients. Am J Gastroenterol 2009;104(9):2288–94.
27. Yamashita H., Takahashi Y., Ishiura H. et al. Hypertrophic pachymeningitis and tracheobronchial stenosis in IgG4-related disease: case presentation and literature review. Intern Med 2012;51:935–41.
28. Winkel M., Lawton C.D., Sanusi O.R. et al. Neuro-surgical considerations for tereating IgG4-related disease with rare spinal epidural compression. Surg Neurol Int 2018;9:209.
29. Wong S., Lam W.Y., Wong W.K., Lee K.C. Hypophysitis presented as inflammatory pseudotumor in immunoglobulin G4-related systemic disease. Hum Pathol 2007;38:1720–3.
30. Okamoto T., Kamisawa A. Autoimmune pancreatitis: proposal of IgG4-related sclerosing disease. J Gastroenterol 2006;41:613–25.
31. Yamamoto M., Takahashi H., Ohara M. et al. A new conceptualization for Mikulicz’s disease as an IgG4-related plasmacytic disease. Mod Rheumatol 2006;16:335–40.
32. Masaki Y., Dong L., Kurose N. et al. Proposal for a new clinical entity, IgG4-positive multi-organ lymphoproliferative syndrome: analysis of 64 cases of IgG4-related disorders. Ann Rheum Dis 2009;68:1310–5.
33. Deshpande V., Zen Y., Chan J.K. et al. Consensus statement on the pathology of IgG4-related disease. Mod Pathol 2012;25(9):1181–92.
34. Stone J.H., Khosroshahi A., Deshpande V. et al. Recommendations for the nomenclature of IgG4-related disease and its individual organ system manifestations. Arthr Rheum 2012;64(10):3061–7.
35. Okazaki K., Kawa S., Kamisawa T. et al. Japanese clinical guidelines for autoimmune pancreatitis. Pancreas 2009;3:849–66.
36. Zen Y., Nakanuma Y. Pathogenesis of IgG4-related disease. Curr Opin Rheumatol 2011;23:114–8.
37. Aalberse R.C., Stapel S.O., Schuurmann J., Rispens T. Immunoglobulin G4: an odd antibody. Clin Exp Allergy 2009;396:469–77.
38. Okazaki K., Uchida K., Ohana M. et al. Autoimmune-related pancreatitis is associated with autoantibodies and a Th1/Th2-type cellular immune response. Gastroenterol 2000;118:573–81.
39. Pezzilli R., Corinaldesi R. IgG4 as a serological marker of autoimmune pancreatitis: the latest news. JOP 2004;5(6):531–3.
40. Mihai S., Chiriac M.T., HerreroGonzalez J.E. IgG4 autoantibodies induce dermal-epidermal separation. J Cell Mol Med 2007;11:1117–28.
41. Umehara H., Okazaki K., Masaki Y. et al. Comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD), 2011. Mod Rheumatol 2012;22(1):21–30.
42. Strehl J.D., Hartmann A., Agaimy A. Numerous IgG4-positive plasma cells are ubiquitous in diverse localised nonspecific chronic inflammatory conditions and need to be distinguished from IgG4-related systemic disorders. J Clin Pathol 2011;64(3):237–43.
43. Schneider A., Lцhr J.M. Autoimmune pancreatitis. Internist (Berl) 2009;50:318–30.
44. Chari S.T., Kloeppel G., Zhang L. et al. Histopathologic and clinical subtypes of autoimmune pancreatitis: the Honolulu consensus document. Pancreas 2010;39:549–54.
45. Shimosegawa T., Chari S.T., Frulloni L. et al. International consensus diagnostic criteria for autoimmune pancreatitis: Guidelines of the International Association of Pancreatology. Pancreas 2011;40(3):352–8.
46. The Japan Pancreas Society, the Ministry of Health and Welfare Investigation Research Team for Intractable Pancreatic Disease. Clinical Diagnostic for Autoimmune Pancreatitis 2011 (Proposal). J Jpn Pancreas (Suizo) 2012;27:17–25.
47. Буеверов А.О., Драпкина О.М. IgG4-ассоциированное системное заболевание. Российские медицинские вести 2014;19(3):4–15.
48. Winkel M., Lawton C.D., Sanusi O.R. et al. Neuro-surgical considerations for treating IgG4-related disease with rare spinal epidural compression. Surg Neurol Int 2018;9:1–5.
49. Uccella S., Amaglio C., Brouland J.-P. et al. Disease heterogeneity in IgG4- related hypophysitis: report of two histopathologically proven cases and review of the literature. Virchows Arch 2019 March 25:1–9.
50. Rotondo F., Quaddoura A., Syro L.V. et al. Immunoglobulin G4 (IgG4)-related hypophysitis. Endocr Pathol 2017 January 13:1–7.
51. Leporati P., Landek-Salgado M.A., Lupi I. et al. IgG4-related hypophysitis: a new addition to the hypophysitis spectrum. J Clin Endocrinol Metab 2011;96:1971–80.
52. Varrassi M., Gianneramo C., Arrigoni F. et al. Neurological involvement of IgG4-related disease: description of a case and review of the literature. Neuroradiol J 2017;1:1–7.
53. Goulam-Houssein S., Grenville J.L., Mastrocostas K. et al. IgG4-related intracranial disease. Neuroradiol J 2019;32(1):1–7.
54. Mehta S.H., Switzer J.A., Biddinger P., Rojiani A.M. IgG4-related leptomeningitis: a reversible cause of rapidly progressive cognitive decline. Neurology 2014;82:540–2.
55. Parney I.F., Johnson E.S., Allen P.B. “Idiopathic” cranial hypertrophic pachymeningitis responsive to antituberculous therapy: case report. Neurosurgery 1997;141:965–71.
56. Hamano H., Kawa S., Horiuchi A. et al. High serum IgG4 concentrations in patients with sclerosing pancreatitis. N Engl J Med 2001;344:732–38.
57. Joshi D., Jager R., Hurel S. et al. Cerebral involvement in IgG4-related disease. Clin Med (Lond) 2015;15:130–4.
58. Vauchot F., Bourdon A., Hay B. et al. Therapeutic response to Rituximab in IgG4-related hypophysitis evidenced on 18F-FDG PET and MRI. Clin Nucl Med 2019;44(5):e362–3.
59. Fernandez-Codina A., HernandezGonzalez M., Solans-Laque R. et al. Alteration of IgG4 levels in cerebrospinal fluid in IgG4-related disease. Int J of Rheum Dis 2017;20:1865–7.
60. Stone J.H., Zen Y., Deshpande V. IgG4-relted disease. N Engl J Med 2012;366:539–51.
61. Lindstrom K.M., Cousar J.B., Lopes M.B. IgG4-related meningeal disease: clinicopathological and proposal for diagnostic criteria. Acta Neuropathol 2010;120:765–76.
62. Lu Z., Tongxi L., Jie L. et al. IgG4-related spinal pachymeningitis. Clin Rheumatol 2016;35:1549–53.
63. Kosakai A., Ito D., Yamada S. et al. A case of definite IgG4-related pachymeningitis. Neurology 2010;75:1390–2.
64. Tajima Y., Mito Y. Cranial neuropathy because of IgG4-related pachymeningitis; intracranial and spinal mass lesions. BMJ Case Rep 2012;2012:bcr2012006471.
65. Radotra B.D., Aggarwal A., Kapoor A. et al. An orphan disease: IgG4-related spinal pachymeningitis: reports of 2 cases. J Neurosurg Spine 2016;25:790–4.
66. Ferreira N.R., Vaz R., Carmona S. et al. IgG4-related disease presenting with an epidural inflammatory pseudotumor: a case report. J Med Case Rep 2016;10(61):e1–6.
Review
For citations:
Smirnov V.A., Grin A.A. Central nervous system damage in IgG4-related disease: review. Russian journal of neurosurgery. 2020;22(3):84-101. (In Russ.) https://doi.org/10.17650/1683-3295-2020-22-3-84-101
Views:
10103
Email this article 