The changing faces of IgG4-related disease: Clinical manifestations and pathogenesis

Islam, Arshia Duza; Selmi, Carlo; Datta-Mitra, Ananya; Sonu, Rebecca; Chen, Mingyi; Gershwin, M. Eric; Raychaudhuri, S. P.

doi:10.1016/j.autrev.2015.06.003

Cited by 42 publications

(35 citation statements)

References 171 publications

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“…The most common condition out of this group is AIP, often associated with multiple organ involvement, such as biliary cholangitis, Mikulicz syndrome, IgG4-positive pneumonitis, retroperitoneal fibrosis, lymphadenopathy, and IgG4-positive mastitis. IgG4 elevation is found in many allergic conditions, IgG4-positive thyroiditis, pericarditis, inflammatory pseudotumors, mediastinal fibrosis, as well as other conditions [13,14].…”

Section: Discussionmentioning

confidence: 99%

Pancreatic Solid Focal Lesions: Differential Diagnosis between Autoimmune Pancreatitis and Pancreatic Cancer

Dítě¹,

Novotný²,

Jana³

et al. 2019

Dig Dis

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Background: Diagnosis of pancreatic cancer (PC) in early stages is still challenging for gastroenterologists. The early detection of cancer is one of the utmost importance for the successful therapy of this malignancy. An accurate differential diagnosis of focal pancreatic lesions plays also an important role, whether it is differential diagnosis of chronic pancreatitis from PC or autoimmune pancreatitis (AIP) from PC. Raised serum immunoglobulin G4 (IgG4) levels to twice the normal value are considered one of significant diagnostic features of type 1 AIP. However, IgG4 can be increased also in patients with PC, but levels usually do not exceed twice the normal value. Methods: In years 2012–2017, IgG4 serum levels were examined in 115 patients with histologically confirmed PC. Patients with PC and elevated IgG4 level (above 135 mg/dL) had tested their histological resection specimens or bioptic specimens from pancreatic lesion, with targeted detection of the presence of IgG4 and plasmocytes in the pancreatic tissue and changes characteristic for type 1 AIP. Results: A plasmatic IgG4 level in 115 patients with diagnosed PC was higher than 135 mg/dL in 14 patients (12.2%). Out of them, 2 patients (1.7%) revealed a serum IgG4 level higher than double the normal value, that is, higher than 270.0 mg/dL (suggestive of AIP). One patient met histological criteria for diagnosis of AIP in the simultaneous presence of PC. Conclusion: Diagnosis of early cancer stages, particularly differentiating AIP from PC can be sometimes problematic. IgG4 levels can be slightly elevated also in case of PC. A targeted biopsy of the pancreas is the method of choice in cases suspected from a focal form of AIP and we recommend to prefer it over other modalities, such as, for example, response to steroid therapy.

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Section: Discussionmentioning

confidence: 99%

Pancreatic Solid Focal Lesions: Differential Diagnosis between Autoimmune Pancreatitis and Pancreatic Cancer

Dítě¹,

Novotný²,

Jana³

et al. 2019

Dig Dis

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“…Other possible predisposing conditions or contributors to IgG4-RD pathogenesis include specific human leukocyte antigen (HLA) alleles and single-nucleotide polymorphisms within some non-HLA genes. 21,22 Also puzzling is the precise role of IgG4 in the disease. IgG4 is the least common IgG subtype, comprising less than 5% of total IgG, 23 and is usually viewed as an antiinflammatory molecule.…”

Section: Pathophysiologymentioning

confidence: 99%

IgG4-Related Disease: A Reminder for Practicing Pathologists

Weindorf

Frederiksen

2017

Archives of Pathology &Amp; Laboratory Medicine

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IgG4-related disease (IgG4-RD) is a systemic autoimmune fibroinflammatory disease that produces sclerotic, tumefactive masses containing dense lymphoplasmacytic infiltrates rich in immunoglobulin (Ig) G4+ plasma cells. Initially characterized as a form of autoimmune pancreatitis, the distinctive histopathology of IgG4-RD has now been described in almost every organ system. However, because the clinical manifestations of IgG4-RD are diverse and nonspecific, the disease may go unsuspected until a biopsy or resection specimen is obtained to diagnose a presumed malignancy. Pathologists thus play a key role in the diagnosis of IgG4-RD, and familiarity with its histopathologic features is essential to preventing the irreversible comorbidities associated with this treatable disease. This brief review outlines the epidemiology, clinical manifestations, and histopathology of IgG4-RD, with the aim of furthering pathologists' awareness of and ability to diagnose this disorder.

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“…Importantly, these IgG4 anti-Dsg1 and anti-Dsg3 autoantibodies in PF/FS and PV are pathogenic by passive transfer into mice, recapitulating the human skin disease in the animals [37, 39, 40]. In addition, IgG4 antibodies are also present in numerous autoimmune diseases, including autoimmune pancreatitis [79], Mikulicz’s disease (primary Sjogren’s syndrome) [80], as well as the newly defined IgG4-related diseases [81-83]. Besides the link between IgE and IgG4 antibodies in allergy and autoimmunity, the regulation of the immune system may also connect allergy and autoimmunity.…”

Section: Introductionmentioning

confidence: 99%

“…Among them, two IgG4 monoclonal antibodies originally isolated by hybridoma method were also identified [92, 107]. This method may prove to be a very useful tool for the study of IgG4 antibody repertoires and the mechanism by which IgG4 antibodies develop in patients with the newly categorized IgG4-related diseases [81-83]. …”

Section: Introductionmentioning

confidence: 99%

Non-infectious environmental antigens as a trigger for the initiation of an autoimmune skin disease

Qian

Culton

Jeong

et al. 2016

Autoimmunity Reviews

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Pemphigus represents a group of organ specific autoimmune blistering disorders of the skin mediated by pathogenic autoantibodies with well-defined antigenic targets. While most of these diseases are sporadic, endemic forms of disease do exist. The endemic form of pemphigus foliaceus (also known as fogo selvagem, FS) exhibits epidemiological features that suggest exposure to hematophagous insect bites are a possible precipitating factor of this autoimmune disease, and provides a unique opportunity to study how environmental factors contribute to autoimmune disease development. FS patients and healthy individuals from endemic regions show an autoreactive IgM response that starts in early childhood and becomes restricted to IgG4 autoantibodies in FS patients. In searching for triggering environmental antigens, we have found that IgG4 and IgE autoantibodies from FS patients cross-react with a salivary antigen from sand flies. The presence of these cross-reactive antibodies and antibody genetic analysis confirming that these antibodies evolve from the same naïve B cells provides compelling evidence that this non-infectious environmental antigen could be the initial target of the autoantibody response in FS. Consequently, FS serves as an ideal model to study the impact of environmental antigens in the development of autoimmune disease.

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The changing faces of IgG4-related disease: Clinical manifestations and pathogenesis

Cited by 42 publications

References 171 publications

Pancreatic Solid Focal Lesions: Differential Diagnosis between Autoimmune Pancreatitis and Pancreatic Cancer

Pancreatic Solid Focal Lesions: Differential Diagnosis between Autoimmune Pancreatitis and Pancreatic Cancer

IgG4-Related Disease: A Reminder for Practicing Pathologists

Non-infectious environmental antigens as a trigger for the initiation of an autoimmune skin disease

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