IgG4-related systemic disease

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Mugilan Poongkunran M.B.B.S [2]

## Overview

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IgG4-related systemic disease (IgG4-RSD), also known as hyper-IgG4 disease and IgG4-related disease is a disease in which inflammatory cells cause fibrosis, the deposition of connective tissue, in one or more organs. The disease is so named because the antibody subtype IgG4 can be detected on tissue samples and often at elevated levels in the bloodstream. The association with IgG4 is a relatively recent finding, and the condition has been described under numerous other names in the past. Diseases such as autoimmune pancreatitis, retroperitoneal fibrosis, mediastinal fibrosis, Riedel's thyroiditis, Mikulicz's syndrome, Kuttner's tumor and inflammatory pseudotumor are now regarded as forms of IgG4-RSD.[1] [2]

## Historical Perspective

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## Classification

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## Pathophysiology

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## Causes

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## Differentiating IgG4-related Systemic Diseases from other Diseases

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## Epidemiology and Demographics

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### Age

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### Gender

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### Race

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## Risk Factors

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## Natural History, Complications and Prognosis

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## Diagnosis

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### Diagnostic Criteria

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### Symptoms

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### Physical Examination

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### Laboratory Findings

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### Imaging Findings

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### Other Diagnostic Studies

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## Treatment

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### Medical Therapy

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### Surgery

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### Prevention

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## References

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1. ↑ Khosroshahi A, Stone JH (2011). "A clinical overview of IgG4-related systemic disease". Curr Opin Rheumatol. 23 (1): 57–66. doi:10.1097/BOR.0b013e3283418057. PMID 21124086. Unknown parameter `|month=` ignored (help)
2. ↑ Stone JH (2012). "IgG4-Related Disease". New England Journal of Medicine. 336 (6): 539–51. doi:10.1056/NEJMra1104650. PMID 22316447. Unknown parameter `|month=` ignored (help)

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