IgG4-Related disease: the Rheumatologist’s Point of View

Abstract

The IgG4-related disease is a rare disorder with an estimated yearly incidence of 1 case per 100 000 population in Japan, characterized by the infiltration of IgG4-positive plasma cells into one or more organs. C-reactive protein and Serum IgG4 levels are often, but not invariably elevated. The most common clinical manifestation is lymphadenopathy, followed by autoimmune pancreatitis and sclerosing cholangitis. Imaging procedures are helpful to identify the affected organs and to monitor the disease course. However, biopsy is often required to secure the diagnosis. Glucocorticoids are the mainstay of therapy, but immunosuppressive agents are useful to prevent recurrences of disease in patients with a chronic relapsing course.

Zusammenfassung

Die IgG4-assozierte Krankheit hat eine Inzidenz von 1/100 000 Personen pro Jahr in Japan. Das histologische Kennzeichen ist ein Infiltrat von IgG4+ Plasmazellen in einem oder mehreren Organen. Im Labor sind das C-reaktive Protein und der IgG4-Spiegel in der Mehrzahl der Fälle erhöht. Die häufigsten klinischen Erscheinungen sind die Lymphadenopathie, die autoimmune Pankreatitis und die sklerosierende Cholangitis. Bildgebende Verfahren werden zum Nachweise des Organbefalls sowie zur Verlaufsbeobachtung verwendet. Eine Biopsie ist jedoch häufig erforderlich, um die Diagnose zu sichern. Steroide sind als Mittel der Wahl bei der IgG4-assozierte Krankheit anzusehen. Bei Patienten die wiederholt Rückfälle bzw. Rezidive erleiden ist es sinnvoll, steroideinsparende Medikamente einzusetzen

Key words

IgG4 - IgG4-related disease - chronic periaortitis - retroperitoneal fibrosis

Schlüsselwörter

IgG4 - IgG4-assozierte Krankheit - chronische Periaortitis - retroperitoneale Fibrose

• References

• 1 Umehara H, Okazaki K, Masaki Y et al. A novel clinical entity, IgG4-related disease (IgG4RD): general concept and details. Mod Rheumatol 2012; 22: 1-14
• 2 Carruthers MN, Stone JH, Khosroshahi A. The latest on IgG4-RD: a rapidly emerging disease. Curr Opin Rheumatol 2012; 24: 60-69
• 3 Ebbo M, Daniel L, Pavic M et al. IgG4-related systemic disease: features and treatment response in a French cohort: results of a multicenter registry. Medicine (Baltimore) 2012; 91: 49-56
• 4 Khosroshahi A, Stone JH. A clinical overview of IgG4-related systemic disease. Curr Opin Rheumatol 2011; 23: 57-66
• 5 Zen Y, Nakanuma Y. IgG4-related disease: a cross-sectional study of 114 cases. Am J Surg Pathol 2010; 34: 1812-1819
• 6 Zen Y, Nakanuma Y. Pathogenesis of IgG4-related disease. Curr Opin Rheumatol 2011; 23: 114-118
• 7 Okazaki K, Uchida K, Fukui T. Recent advances in autoimmune pancreatitis: concept, diagnosis, and pathogenesis. J Gastroenterol 2008; 43: 409-418
• 8 Stone JH, Zen Y, Deshpande V. IgG4-related disease. N Engl J Med 2012; 366: 539-551
• 9 Zen Y, Fujii T, Harada K et al. Th2 and regulatory immune reactions are increased in immunoglobin G4-related sclerosing pancreatitis and cholangitis. Hepatology 2007; 45: 1538-1546
• 10 Tanaka A, Moriyama M, Nakashima H et al. Th2 and regulatory immune reactions contribute to IgG4 production and the initiation of Mikulicz disease. Arthritis Rheum 2012; 64: 254-263
• 11 Okazaki K, Uchida K, Ohana M et al. Autoimmune-related pancreatitis is associated with autoantibodies and a Th1/Th2-type cellular immune response. Gastroenterology 2000; 118: 573-581
• 12 Guarneri F, Guarneri C, Benvenga S. Helicobacter pylori and autoimmune pancreatitis: role of carbonic anhydrase via molecular mimicry?. J Cell Mol Med 2005; 9: 741-744
• 13 Umehara H, Okazaki K, Masaki Y et al. Comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD), 2011. Mod Rheumatol. 2012 22. 21-30
• 14 Divatia M, Kim SA, Ro JY. IgG4-related sclerosing disease, an emerging entity: a review of a multi-system disease. Yonsei Med J 2012; 53: 15-34
• 15 Okazaki K, Uchida K, Koyabu M et al. Recent advances in the concept and diagnosis of autoimmune pancreatitis and IgG4-related disease. J Gastroenterol 2011; 46: 277-288
• 16 Okazaki K, Uchida K, Miyoshi H et al. Recent concepts of autoimmune pancreatitis and IgG4-related disease. Clin Rev Allergy Immunol 2011; 41: 126-138
• 17 Okazaki K, Kawa S, Kamisawa T et al. Clinical diagnostic criteria of autoimmune pancreatitis: revised proposal. J Gastroenterol 2006; 41: 626-631
• 18 Masaki Y, Sugai S, Umehara H. IgG4-related diseases including Mikulicz’s disease and sclerosing pancreatitis: diagnostic insights. J Rheumatol 2010; 37: 1380-1385
• 19 Kawano M, Saeki T, Nakashima H et al. Proposal for diagnostic criteria for IgG4-related kidney disease. Clin Exp Nephrol 2011; 15: 615-626
• 20 Grimm KE, Barry TS, Chizhevsky V et al. Histopathological findings in 29 lymph node biopsies with increased IgG4 plasma cells. Mod Pathol 2012; 25: 480-491
• 21 Geyer JT, Deshpande V. IgG4-associated sialadenitis. Curr Opin Rheumatol 2011; 23: 95-101
• 22 Yamamoto M, Harada S, Ohara M et al. Clinical and pathological differences between Mikulicz’s disease and Sjogren’s syndrome. Rheumatology (Oxford) 2005; 44: 227-234
• 23 Ryu JH, Sekiguchi H, Yi ES. Pulmonary manifestations of immunoglobulin G4-related sclerosing disease. Eur Respir J 2012; 39: 180-186
• 24 Matsui S, Taki H, Shinoda K et al. Respiratory involvement in IgG4-related Mikulicz’s disease. Mod Rheumatol 2012; 22: 31-39
• 25 Mizushima I, Yamada K, Fujii H et al. Clinical and histological changes associated with corticosteroid therapy in IgG4-related tubulointerstitial nephritis. Mod Rheumatol 2012;
• 26 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: 1310-1315
• 27 Shinoda K, Matsui S, Taki H et al. Deforming arthropathy in a patient with IgG4-related systemic disease: comment on the article by Stone et al. Arthritis Care Res (Hoboken ) 2011; 63: 172
• 28 Umekita K, Kaneko Y, Yorita K et al. Arthropathy with infiltrate IgG4-positive plasma cells in synovium. Rheumatology (Oxford) 2012; 51: 580-582
• 29 Palmisano A, Vaglio A. Chronic periaortitis: a fibro-inflammatory disorder. Best Pract Res Clin Rheumatol 2009; 23: 339-353
• 30 Salvarani C, Pipitone N, Versari A et al. Positron emission tomography (PET): evaluation of chronic periaortitis. Arthritis Rheum 2005; 53: 298-303
• 31 Zen Y, Onodera M, Inoue D et al. Retroperitoneal fibrosis: a clinicopathologic study with respect to immunoglobulin G4. Am J Surg Pathol 2009; 33: 1833-1839
• 32 Kasashima S, Zen Y.. IgG4-related inflammatory abdominal aortic aneurysm. Curr Opin Rheumatol 2011; 23: 18-23
• 33 Laco J, Steiner I, Holubec T et al. Isolated thoracic aortitis: clinicopathological and immunohistochemical study of 11 cases. Cardiovasc Pathol 2011; 20: 352-360
• 34 Yamamoto M, Takahashi H, Tabeya T et al. Risk of malignancies in IgG4-related disease. Mod Rheumatol 2011;
• 35 Smyrk TC. Pathological features of IgG4-related sclerosing disease. Curr Opin Rheumatol 2011; 23: 74-79
• 36 Palmisano A, Corradi D, Carnevali ML et al. Chronic periaortitis associated with membranous nephropathy: clues to common pathogenetic mechanisms. Clin Nephrol 2010; 74: 485-490
• 37 Nirula A, Glaser SM, Kalled SL et al. What is IgG4? A review of the biology of a unique immunoglobulin subtype. Curr Opin Rheumatol 2011; 23: 119-124
• 38 Yamamoto M, Tabeya T, Naishiro Y et al. Value of serum IgG4 in the diagnosis of IgG4-related disease and in differentiation from rheumatic diseases and other diseases. Mod Rheumatol 2011;
• 39 Vaglio A, Strehl JD, Manger B et al. IgG4 immune response in Churg-Strauss syndrome. Ann Rheum Dis 2012; 71: 390-393
• 40 Inoue D, Zen Y, Abo H et al. Immunoglobulin G4-related periaortitis and periarteritis: CT findings in 17 patients. Radiology. 2011. 261. 625-633
• 41 Kottra JJ, Dunnick NR. Retroperitoneal fibrosis. Radiol Clin North Am 1996; 34: 1259-1275
• 42 Katsura M, Mori H, Kunimatsu A et al. Radiological features of IgG4-related disease in the head, neck, and brain. Neuroradiology 2012;
• 43 Pipitone N, Versari A, Salvarani C. Role of imaging studies in the diagnosis and follow-up of large-vessel vasculitis: an update. Rheumatology (Oxford) 2008; 47: 403-408
• 44 Saegusa H, Momose M, Kawa S et al. Hilar and pancreatic gallium-67 accumulation is characteristic feature of autoimmune pancreatitis. Pancreas 2003; 27: 20-25
• 45 Nakatani K, Nakamoto Y, Togashi K. Utility of FDG PET/CT in IgG4-related systemic disease. Clin Radiol 2012; 67: 297-305
• 46 Vaglio A, Greco P, Versari A et al. Post-treatment residual tissue in idiopathic retroperitoneal fibrosis: active residual disease or silent “scar”? A study using 18 F-fluorodeoxyglucose positron emission tomography. Clin Exp Rheumatol 2005; 23: 231-234
• 47 Kamisawa T, Okazaki K, Kawa S et al. Japanese consensus guidelines for management of autoimmune pancreatitis: III. Treatment and prognosis of AIP. J Gastroenterol 2010; 45: 471-477
• 48 Yamamoto M, Harada S, Ohara M et al. Beneficial effects of steroid therapy for Mikulicz’s disease. Rheumatology (Oxford) 2005; 44: 1322-1323
• 49 Tomiyama T, Uchida K, Matsushita M et al. Comparison of steroid pulse therapy and conventional oral steroid therapy as initial treatment for autoimmune pancreatitis. J Gastroenterol 2011; 46: 696-704
• 50 Khosroshahi A, Carruthers MN, Deshpande V et al. Rituximab for the treatment of IgG4-related disease: lessons from 10 consecutive patients. Medicine (Baltimore) 2012; 91: 57-66
• 51 Vaglio A, Salvarani C, Buzio C. Retroperitoneal fibrosis. Lancet 2006; 367: 241-251
• 52 Vaglio A, Palmisano A, Corradi D et al. Retroperitoneal fibrosis: evolving concepts. Rheum Dis Clin North Am 2007; 33: 803-817
• 53 Binder M, Uhl M, Wiech T et al. Cyclophosphamide is a highly effective and safe induction therapy in chronic periaortitis: a long-term follow-up of 35 patients with chronic periaortitis. Ann Rheum Dis 2012; 71: 311-312
• 54 Vaglio A, Palmisano A, Alberici F et al. Prednisone versus tamoxifen in patients with idiopathic retroperitoneal fibrosis: an open-label randomised controlled trial. Lancet 2011; 378: 338-346
• 55 Khosroshahi A, Bloch DB, Deshpande V et al. Rituximab therapy leads to rapid decline of serum IgG4 levels and prompt clinical improvement in IgG4-related systemic disease. Arthritis Rheum 2010; 62: 1755-1762
• 56 Tabata T, Kamisawa T, Takuma K et al. Serial changes of elevated serum IgG4 levels in IgG4-related systemic disease. Intern Med 2011; 50: 69-75
• 57 Nishimori I, Kohsaki T, Onishi S et al. IgG4-related autoimmune prostatitis: two cases with or without autoimmune pancreatitis. Intern Med 2007; 46: 1983-1939
• 58 Walter MA, Melzer RA, Schindler C et al. The value of [18 F]FDG-PET in the diagnosis of large-vessel vasculitis and the assessment of activity and extent of disease. Eur J Nucl Med Mol Imaging 2005; 32: 674-681