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Diagnosis and management of IgG4-related disease.

ABSTRACT: IgG subclass 4-related disease (IgG4-RD) is a rare but increasingly recognised fibroinflammatory condition known to affect multiple organs. IgG4-RD is characterised by unique histological features of lymphoplasmacytic infiltration, storiform fibrosis and obliterative phlebitis. In this review we describe the pancreaticobiliary manifestations of IgG4-RD, with particular emphasis on type 1 autoimmune pancreatitis (AIP) and IgG4-related sclerosing cholangitis (IgG4-SC). AIP and IgG4-SC can pose diagnostic challenges to the clinician as they may mimic pancreatic cancer and primary sclerosing cholangitis, respectively. We discuss current knowledge, clinical diagnostic criteria and recent advances and summarise the evidence base for current therapeutic approaches for AIP and IgG4-SC.

SUBMITTER: Hegade VS

PROVIDER: S-EPMC6583577 | biostudies-literature | 2019 Jul

REPOSITORIES: biostudies-literature

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Diagnosis and management of IgG4-related disease.

Hegade Vinod S VS Sheridan Maria B MB Huggett Matthew T MT

Frontline gastroenterology 20181031 3

IgG subclass 4-related disease (IgG4-RD) is a rare but increasingly recognised fibroinflammatory condition known to affect multiple organs. IgG4-RD is characterised by unique histological features of lymphoplasmacytic infiltration, storiform fibrosis and obliterative phlebitis. In this review we describe the pancreaticobiliary manifestations of IgG4-RD, with particular emphasis on type 1 autoimmune pancreatitis (AIP) and IgG4-related sclerosing cholangitis (IgG4-SC). AIP and IgG4-SC can pose dia ...[more]

PMID: 31288262

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IgG4 autoantibodies and autoantigens in the context of IgG4-autoimmune disease and IgG4-related disease.

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| S-EPMC8718705 | biostudies-literature

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| S-EPMC7197178 | biostudies-literature

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A Challenging Diagnosis of IgG4-Related Disease When Understanding Limitations of Laboratory Testing Was Pivotal.

Project description:A 76-year-old man was incidentally found on a CT scan to have lymphadenopathy and bilateral kidney enlargement suggestive of infiltrative renal disease. He was largely asymptomatic but had bilateral salivary and lacrimal gland enlargement. A grossly elevated serum IgG (>70 g/L) with concomitant suppression of other immunoglobulins, a small IgG restriction, and a parotid biopsy revealing lymphoplasmacytic infiltrate with slight kappa light chain excess all suggested a lymphoproliferative disorder (LPD). The diagnostic workup was further confounded by a normal serum IgG4 concentration. Moreover, bone marrow and renal biopsies did not reveal evidence of LPD. Discussion with the laboratory not only clarified that the markedly increased total IgG could not be accounted for by the small IgG restriction, but also identified a discrepancy in the IgG4 measurement. Repeat analysis of a follow-up sample revealed an elevated IgG4 of 5.94 (reference interval: 0.039-0.864) g/L, which prompted a repeat parotid biopsy that showed predominant IgG4+ lymphocytic infiltrates. Despite the deluding presentations, a final diagnosis of IgG4-related disease (IgG4-RD) was made based on elevated serum IgG4 concentrations and histopathological findings. This case highlights the importance of recognizing limitations of laboratory testing and the benefit of close communications among clinical subspecialties and the laboratory.

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Neurological Manifestations of IgG4-Related Disease.

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Clinical Significance of Serum IgG4 in the Diagnosis and Treatment Response of IgG4-Related Disease in Adults of Southwest China: A Retrospective Study.

Project description:BackgroundThere is no standard cut-off value of serum IgG4 concentration and serum IgG4/total IgG ratio for the diagnosis of IgG4-related disease (IgG4-RD) or as a marker of treatment responses. We aimed to explore this issue through a retrospective cohort analysis of adults in southwest China.MethodsThe diagnostic performance of serum IgG4 concentration and IgG4/IgG ratio for IgG4-RD was evaluated in a retrospective analysis of 177 adults newly diagnosed as having IgG4-RD and 877 adults without IgG4-RD. Dynamic analysis was performed to evaluate the significance of serum IgG4 concentration on IgG4-RD treatment responses.ResultsThe serum IgG4 concentration differed according to sex. The optimal cut-off values of serum IgG4 concentration and IgG4/IgG ratio for IgG4-RD diagnosis were 1.92 g/L and 0.12 in males and 1.83 g/L and 0.11 in females, respectively. For patients with serum IgG4 concentration >2.01 g/L, the cut-off values in the total population were >3.00 g/L and 0.19, respectively. The median serum IgG4 concentration decreased over time, and the decrease rate increased over time. The serum IgG4 concentration significantly decreased at >1 week post-treatment (P=0.004), and the median decrease rate was close to 50% at >4 weeks post-treatment.ConclusionsSerum IgG4 can be a good indicator for IgG4-RD diagnosis; however, different diagnostic cut-off values should be determined according to sex. The decreasing rate is more conducive than the serum IgG4 concentration to monitor treatment efficacy. The IgG4/IgG ratio did not improve the diagnostic efficacy for IgG4-RD.

| S-EPMC10151269 | biostudies-literature

Project description:Cervical lymph node enlargement as the first and sole manifestation of IgG4-related disease (IgG4-RD) is rare and is often difficult to distinguish from lymphoma. Here, we report a case of a 63-year-old man initially presenting with bilateral posterior neck masses. Ultrasonography revealed multiple matted, ovoid, homogenous, hypoechoic, and enlarged lymph nodes below the right parotid gland. In addition, there was heterogeneous echotexture with small and indistinct hypoechoic nodules over bilateral parotid and submandibular glands which suggested sclerosing sialadenitis. Pathology of the tissues obtained by core needle biopsy revealed reactive hyperplasia, but a diagnosis of lymphoma could not be excluded. Subsequently, excisional biopsy and serological tests were done. The diagnosis of IgG4-RD was confirmed due to marked elevation of serum IgG4 levels and pathological evidence of IgG+ and IgG4+ plasma cell infiltration in the lymph node specimen. The patient's neck masses subsided gradually after 1 week of oral steroid therapy. The differential diagnosis of IgG4-RD should always be considered when sclerosing sialadenitis is presented with cervical lymphadenopathy.

| S-EPMC6445037 | biostudies-literature

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