The immunobiology and clinical characteristics of IgG4 related diseases

Takahashi, Hiroki; Yamamoto, Motohisa; Tabeya, Tetsuya; Suzuki, Chisako; Naishiro, Yasuyoshi; Shinomura, Yasuhisa; Imai, Kohzoh

doi:10.1016/j.jaut.2012.01.007

Cited by 49 publications

(33 citation statements)

References 19 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…However, serology may reveal elevated IgE (5, 6), polyclonal hypergammaglobulinemia (37), hypocomplementemia (21% of cases, particularly with renal involvement), presence of antinuclear antibodies (16-50%), and rheumatoid factor (20%) (49,50). Elevated serum IgG4…”

Section: Laboratory Testsmentioning

confidence: 99%

“…In contrast to SS, patients with IgG4-related sialoadenitis/dacryodenitis (or so-called MD) have male dominance, more often involvement of submandibular than parotid glands, and mild symptoms of dry mouth/dry eyes. They are usually negative for anti-SS-A/SS-B antibodies, and respond rapidly to corticosteroids (2,49). In addition, minor salivary gland biopsy reveals intact structure of salivary ducts despite lymphocytic infiltration and formation of lymphoid follicles, and immunostaining for IgG4-positive plasma cells helps further in differential diagnosis (2,27,66).…”

Section: Salivary and Lacrimal Glandsmentioning

confidence: 99%

See 1 more Smart Citation

IgG4-related disease: A relatively new concept for clinicians

Vasaitis

2016

European Journal of Internal Medicine

105

129

View full text Add to dashboard Cite

IgG4-related disease (IgG4-RD) is a recently recognized chronic fibrotic inflammation, which can affect almost every organ, and may come to clinical attention first due to visible organ swelling or organ dysfunction, or is identified incidentally by imaging and specific biopsy.The disorder has an allergic background and is immune-mediated. Up-regulated responses of T helper 2 and T regulatory cells and their cytokines play a major role in disease progression.About 30-50% of patients are atopic or have mild eosinophilia. IgG4-RD predominantly affects middle-aged male patients. The cornerstones of diagnosis of the disease are compatible clinical features and typical histopathology. Swelling of salivary and lacrimal glands, lymphadenopathy, and type 1 autoimmune pancreatitis (AIP) are the most common manifestations of the disease. However, other tissues and organs, such as retroperitoneum, lung, kidney, aorta, upper airways, thyroid gland, meninges, heart, mesenterium and skin may be involved. Typical histopathology is lymphoplasmacytic infiltration abundant in IgG4-positive plasma cells, storiform-type fibrosis, and obliterative phlebitis. Elevated serum IgG4 concentration supports the diagnosis. Characteristic imaging features such as a "capsule-like rim" surrounding the pancreatic lesions is highly specific to type 1 AIP. 18F-fluorodeoxyglucose positron emission tomography/computed tomography enables mapping the sites of inflammation, permits evaluation of the extent of the disease, helps in guiding biopsy decision, and may be used in monitoring response to treatment. Glucocorticoids alone or in combination with B-cell depletion with rituximab induces prompt clinical response to IgG4-RD.This article reviews the current understanding, different clinical manifestations, and approaches to diagnosis and treatment of IgG4-RD. 3Highlights Ø IgG4-related disease is a chronic fibrotic inflammation.Ø The disease affects multiple organs, is progressive, and impairs function.Ø The disorder has an allergic background and is immune-mediated.Ø Compatible clinical signs and typical pathology are the basis for the diagnosis.Ø Glucocorticoids and/or B-cell depletion induces prompt clinical response.Immunoglobulin G4-related disease (IgG4-RD) is a chronic fibrotic inflammation, characterized by tissue infiltration by lymphocytes, IgG4-positive plasma cells, simultaneous development of fibrosis, and often, but not always, elevated serum levels of IgG4 (1, 2). The disease can affect multiple organs. Early diagnosis of IgG4-RD is not easy because of the indolent course of the disease with vague, nonspecific symptoms. It may first come to clinical attention as visible organ swelling or detected organ dysfunction due to fibrosis, or is identified incidentally by imaging and specific biopsy when the patient undergoes investigation of suspected tumor disease. Most forms of organ involvement in IgG4-RD, even in the advanced fibrotic stages, respond well to glucocorticoids (3, 4). 4A comprehensive search for all articles in E...

show abstract

Section: Laboratory Testsmentioning

confidence: 99%

Section: Salivary and Lacrimal Glandsmentioning

confidence: 99%

IgG4-related disease: A relatively new concept for clinicians

Vasaitis

2016

European Journal of Internal Medicine

105

129

View full text Add to dashboard Cite

show abstract

“…The immunologic profile of IgG4-RD manifests characteristics of a predominantly type 2 helper cell immune response and infiltration by regulatory T cells, which produce interleukin-10. [13][14][15] This cytokine imbalance is thought to potentiate IgG4 production and promote IgG4-RD. There is also evidence of expanded plasmablast (stage of B lymphocyte development between activated B cell and plasma cell) population that is oligoclonal in nature.…”

Section: Immunologic Mechanismsmentioning

confidence: 99%

Immunoglobulin G4-Related Disease and the Lung

Ryu

2016

Clinics in Chest Medicine

View full text Add to dashboard Cite

“…31,[36][37][38] Low rate of autoantibodies, including rheumatoid factor, antinuclear antibodies, anti-Sjögren'ssyndrome-related antigen A, anti-Sjögren'ssyndrome-related antigen B, higher serum IgG4 and immunoglobulin E concentrations and better response to steroid therapy are other findings that can help differentiating IgG4-related disease from SS. 31,[36][37][38] These differences enabled us to rule out Kuttner tumor in our first patient and Miculicz disease in our second patient.…”

mentioning

confidence: 99%

“…48,49 Good response to glucocorticoids is so remarkable that it may further confirm the diagnosis and even help to discriminate it from other similar diseases such as SS. 15,31,[36][37][38] Other medications such as methotrexate, azathioprine or mycophenolate mofetil may be used to reduce adverse effects of long-term steroids usage. In addition, recent studies have shown significant efficacy of B cell depletion therapy with rituximab in refractory patients.…”

mentioning

confidence: 99%