ReviewThe clinical spectrum of IgG4-related disease
Section snippets
Historical overview and definition
IgG4-related disease (IgG4-RD) is a systemic immune-mediated disease described in Japan in the first years of the 21st century [1]. The disease is characterized pathologically by the infiltration of IgG4-bearing plasma cells into involved organs [1]. IgG4-RD is not a new disease because many previously-recognized single- and even multi-organ conditions, once regarded as entirely separate disorders, are now known to comprise parts of the IgG4-RD spectrum. Such conditions include “Mikulicz
Geoepidemiology of IgG4-RD
IgG4-RD is generally regarded as an uncommon disorder but it is likely that substantial under-recognition of the disease exists because many physicians remain unfamiliar with this diagnosis. A recent study estimated that approximately 8000 patients had IgG4-RD in Japan in 2009 [8], representing an estimated prevalence in the Japanese population of 6 cases per 100,000 people. Fig. 1 summarizes the geographic origin of 3482 reported cases (MEDLINE search of articles published through March 24,
Pancreas
The pancreas was the first organ reported in the context of IgG4-RD [2], [3] and perhaps as a result this organ has been reported most frequently in the literature. Type I autoimmune pancreatitis was described in 345/840 (41%) systemic cases reported (Table 1). Eight studies including 1245 patients have focused on IgG4-related pancreatitis [10], [12], [13], [14], [15], [16], [17], [18]. Four studies [10], [12], [15], [18] have detailed the main presenting symptoms, which included jaundice in
Conclusions
IgG4-RD is an increasingly-recognized condition in adults, with a heterogeneous clinical presentation affecting a wide range of organ systems. This review shows that clinical studies have a low level of evidence, especially with respect to the lack of a homogeneous diagnostic approach. The body of evidence relies predominantly on series including a range of 10–50 patients in 80% of selected studies. Although a long list of features involving the majority of organs and systems has been reported
Take-home messages
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IgG4-related disease is a systemic immune-mediated disease described in the 21st century
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The disease is characterized by the infiltration of IgG4-bearing plasma cells and raised IgG4 levels
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The cardinal clinical feature of IgG4-RD is single or multiple organ swelling
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Nearly 75% of cases have been reported in Japan
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More than 40 different organs have been reported to be involved in IgG4-RD
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The organs more frequently involved are the pancreas, salivary/lacrimal glands, biliary tree, kidneys, thyroid
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Supported by the Josep Font Research Fellow Award, Hospital Clinic, Barcelona, Spain.