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Review ArticleReview

IgG4-related Diseases Including Mikulicz’s Disease and Sclerosing Pancreatitis: Diagnostic Insights

YASUFUMI MASAKI, SUSUMU SUGAI and HISANORI UMEHARA
The Journal of Rheumatology July 2010, 37 (7) 1380-1385; DOI: https://doi.org/10.3899/jrheum.091153
YASUFUMI MASAKI
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SUSUMU SUGAI
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HISANORI UMEHARA
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Abstract

Since the first report of serum IgG4 elevation in sclerosing pancreatitis in 2001, various systemic disorders have been reported to elevate IgG4, and many names have been proposed from the perspective of the systemic condition. Despite similarities in the organs damaged in IgG4-related Mikulicz’s disease and Sjögren’s syndrome, there are marked clinical and pathological differences between the 2 entities. The majority of cases diagnosed with autoimmune pancreatitis in Japan are IgG4-related sclerosing pancreatitis, and it should be recognized that this is distinct from the Western type. Diagnosis of IgG4-related disease is defined by both elevated serum IgG4 (> 1.35 g/l) and histopathological features, including lymphocyte and IgG4+ plasma cell infiltration (IgG4+ plasma cells/IgG+ plasma cells > 50% on a highly magnified slide checked at 5 points). Differential diagnosis from other distinct disorders is necessary: these include sarcoidosis, Castleman’s disease, Wegener’s granulomatosis, lymphoma, cancer, and other existing conditions. The Japanese IgG4 research group has begun multicenter prospective studies to improve diagnostic criteria and treatment strategies.

  • MIKULICZ’S DISEASE
  • SJÖGREN’S SYNDROME
  • AUTOIMMUNE PANCREATITIS
  • GLUCOCORTICOID
  • IgG4-RELATED DISEASES

Footnotes

  • Supported by grants from Intractable Diseases, the Health and Labor Sciences Research Grants from the Ministry of Health, Labor, and Welfare, and the Japanese Ministry of Education, Culture, Sports, Science, and Technology (13557160, 15024236, 15390313, and 13877075 to H. Umehara and 17591060 to Y. Masaki), Umehara Memorial Foundation (to H. Umehara), The Vehicle Racing Commemorative Foundation, and the Kanazawa Medical University Research Foundation (C2009-4 to H. Umehara and S2004-16 and S2007-5 to Y. Masaki).

  • Accepted for publication February 8, 2010.
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The Journal of Rheumatology: 37 (7)
The Journal of Rheumatology
Vol. 37, Issue 7
1 Jul 2010
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IgG4-related Diseases Including Mikulicz’s Disease and Sclerosing Pancreatitis: Diagnostic Insights
YASUFUMI MASAKI, SUSUMU SUGAI, HISANORI UMEHARA
The Journal of Rheumatology Jul 2010, 37 (7) 1380-1385; DOI: 10.3899/jrheum.091153

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IgG4-related Diseases Including Mikulicz’s Disease and Sclerosing Pancreatitis: Diagnostic Insights
YASUFUMI MASAKI, SUSUMU SUGAI, HISANORI UMEHARA
The Journal of Rheumatology Jul 2010, 37 (7) 1380-1385; DOI: 10.3899/jrheum.091153
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