IgG4-Related Disease

Stone, Julie M.; Zen, You; Deshpande,

doi:10.1056/nejmra1104650

Cited by 2,257 publications

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References 88 publications

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“…Isolated ACTH deficiency was defined as secondary adrenal insufficiency with low or absent cortisol production and normal secretion of pituitary hormones other than ACTH (24). Among the remaining patients with hypophysitis or without known causes of hypopituitarism and/or central DI, we tried to identify criteria suggesting IgG4-related hypophysitis: i) serum IgG4 concentrations R135 mg/dl; ii) involvement of other organs observed in IgG4-RD; and/or iii) infiltration of IgG4-positive plasma cells in the involved tissues (1,2,5,25,26) (Fig. 1).…”

Section: Subjectsmentioning

confidence: 99%

The prevalence of IgG4-related hypophysitis in 170 consecutive patients with hypopituitarism and/or central diabetes insipidus and review of the literature

Bando

Iguchi

Fukuoka

et al. 2014

European Journal of Endocrinology

113

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Objective: The prevalence and clinical characteristics of IgG4-related hypophysitis remain unclear due to the limited number of case reports. Therefore, in this study, we screened consecutive outpatients with hypopituitarism and/or diabetes insipidus (DI) to estimate its prevalence. Methods: A total of 170 consecutive outpatients with hypopituitarism and/or central DI were screened at Kobe University Hospital for detecting IgG4-related hypophysitis by pituitary magnetic resonance imaging, measuring serum IgG4 concentrations, assessing the involvement of other organs, and carrying out an immunohistochemical analysis to detect IgG4-positive cell infiltration. Results: Among the screened cases, 116 cases were excluded due to diagnosis of other causes such as tumors and congenital abnormalities. Additionally, 22 cases with isolated ACTH deficiency were analyzed and were found not to meet the criteria of IgG4-related hypophysitis. The remaining 32 cases were screened and seven were diagnosed with IgG4-related hypophysitis, of which three cases were diagnosed by analyzing pituitary specimens. IgG4-related hypophysitis was detected in 30% (seven of 23 patients) of hypophysitis cases and 4% of all hypopituitarism/DI cases. The mean age at the onset of IgG4-related hypophysitis was 61.8G8.8 years, and the serum IgG4 concentration was 191.1G78.3 mg/dl (normal values 5-105 mg/dl and values in IgG4-related disease (RD) R135 mg/dl). Pituitary gland and/or stalk swelling was observed in six patients, and an empty sella was observed in one patient. Multiple co-existing organ involvement was observed in four of the seven patients prior to the onset of IgG4-related hypophysitis. Conclusion: These data suggest that the prevalence of IgG4-related hypophysitis has been underestimated. We should also consider the possibility of the development of hypopituitarism/DI caused by IgG4-related hypophysitis during the clinical course of other IgG4-RDs.

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Section: Subjectsmentioning

confidence: 99%

The prevalence of IgG4-related hypophysitis in 170 consecutive patients with hypopituitarism and/or central diabetes insipidus and review of the literature

Bando

Iguchi

Fukuoka

et al. 2014

European Journal of Endocrinology

113

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“…IgG4‐associated cholangitis (IAC) and autoimmune pancreatitis are the predominant manifestations of IgG4‐related diseases (IgG4‐RD); their pathogenesis is enigmatic 1, 2. Serum IgG4 concentrations are generally elevated in IgG4‐RD but the biological implications thereof have not been explained 3.…”

Section: Tablementioning

confidence: 99%

Exposure to occupational antigens might predispose to IgG4‐related disease

2014

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“…Immunohistochemistry stains showed a dense lymphoplasmacytic infiltrate rich in IgG4 + plasma cells. IgG4RD was first documented in an autoimmune pancreatitis (AIP) IgG4-positive plasma cells may involve not only the pancreas, but also in almost every organ system, such as the bile duct, gallbladder, salivary gland, thyroid gland, lungs, stomach, colon, liver, retroperitoneum, kidney, prostate and lymph nodes [1] . Nevertheless, IgG4-related mass lesions in the stomach have been rarely reported.…”

Section: Introductionmentioning

confidence: 99%

Gastric ulcer caused by immunoglobulin G4-related diseases: a case report

Zheng

Xiao

et al. 2015

CRIM

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Objective: To enhance the diagnosis and treatment of Immunoglobulin G4-related gastric disease. Method:One case of IgG4-related gastric disease in our hospital was presented and analyzed.Result: Patients with IgG4-related gastric disease usually showed nonspecific clinical symptoms such as abdominal discomfort, dull pain, anorexia, nausea and vomiting, anemia, weight loss, etc. By gastroscopy, we could divide IgG4-related gastro intestinal disease into two categories: organ enlargement and ulceration, whose histopathological features consisting of abundant infiltration of IgG4 positive lymphocytes, plasma cells, formation of lymph follicle. The lesion could spreading to every layer of the stomach, nerve, minute vessels and regional lymph nodes, which only existed in stomach that were not associated with other IgG4-related diseases. The diagnosis of the disease may refer to Japanese IgG4-related disease diagnostic criteria. We should identify it to exclude gastric cancer clinically. Treatment with steroids was given priority to, and surgery could be a complementary method. Conclusion:The incidence of IgG4 related-gastric disease is rare, and it is not difficult to diagnose and treat by analysis of the limited cases. It is of utmost importance for us to raise vigilance, consider IgG4-related disease and especially to accumulate more cases, explore its pathogenesis, clinical characteristics if possible.

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IgG4-Related Disease

Cited by 2,257 publications

References 88 publications

The prevalence of IgG4-related hypophysitis in 170 consecutive patients with hypopituitarism and/or central diabetes insipidus and review of the literature

The prevalence of IgG4-related hypophysitis in 170 consecutive patients with hypopituitarism and/or central diabetes insipidus and review of the literature

Exposure to occupational antigens might predispose to IgG4‐related disease

Gastric ulcer caused by immunoglobulin G4-related diseases: a case report

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