IgG4-Related Disease

Mahajan, Vinay S.; Mattoo, Hamid; Deshpande,; Ss, Pillai; Stone, Julie M.

doi:10.1146/annurev-pathol-012513-104708

Cited by 324 publications

(280 citation statements)

References 151 publications

Supporting

Mentioning

266

Contrasting

Unclassified

Order By: Relevance

“…A continuous or recurrent painless uni-or bilateral swelling of the parotid and/or submandibular glands (sialadenitis) and/or upper eyelids (dacryoadenitis) with changes in facial appearance is particularly characteristic of IgG4- RD (27,71). 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.…”

Section: Salivary and Lacrimal Glandsmentioning

confidence: 99%

“…The lymph nodes are non-tender and 1-3 cm in diameter (71,72). Fibrosis is uncommon feature and is only seen in pseudotumor-like lymph nodes (34).…”

Section: Lymph Nodesmentioning

confidence: 99%

“…The radiologic manifestations are often extensive even in the setting of mild clinical symptomatology (71). CT scan findings could be categorized into four major features: 15 1) solid nodular densities; 2) round-shaped ground-glass opacities; 3) alveolar interstitial type with bronchiectasis resembling interstitial lung disease and pulmonary fibrosis with honeycombing; and 4) thickening of the bronchovascular bundles and interlobular septa (59).…”

Section: Lungs Pleura and Mediastinummentioning

confidence: 99%

“…Other radiological features could also be seen in IgG4-related lung disease: mass density in mediastinum, nodular or diffuse pleural thickening, pleural effusion, and enlargement of regional lymph nodes (71). The diagnosis of IgG4-related lung disease can be difficult to differentiate from malignant tumors, sarcoidosis, multicentric Castleman's disease, GPA, or other inflammatory diseases.…”

Section: Lungs Pleura and Mediastinummentioning

confidence: 99%

“…Among the three layers comprising the wall of aorta, the adventitia is most susceptible to IgG4-related inflammatory process, which can disrupt the lamellar elastic fibers in the media leading to aneurysmal transformation (71,91). Involvement of other organs is seen in 70% of cases and overlap between IgG4-related periaortitis and retroperitoneal fibrosis can occur (60).…”

Section: Igg4-related Periaortitis And/or Periarteritismentioning

confidence: 99%

See 4 more Smart Citations

IgG4-related disease: A relatively new concept for clinicians

Vasaitis

2016

European Journal of Internal Medicine

100

119

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: Salivary and Lacrimal Glandsmentioning

confidence: 99%

“…The lymph nodes are non-tender and 1-3 cm in diameter (71,72). Fibrosis is uncommon feature and is only seen in pseudotumor-like lymph nodes (34).…”

Section: Lymph Nodesmentioning

confidence: 99%