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Respirology (Carlton, Vic.) Feb 2023Immunoglobulin G4-related disease (IgG4-RD) is a recently described rare systemic fibroinflammatory disease with an estimated incidence of less than 1 in 100,000 persons... (Review)
Review
Immunoglobulin G4-related disease (IgG4-RD) is a recently described rare systemic fibroinflammatory disease with an estimated incidence of less than 1 in 100,000 persons per year. The disease can affect virtually any organ and is characterized by unifying histopathological findings. Recently, four subgroups of patients have been characterized: hepatobiliary, head and neck, Mikulicz syndrome and retroperitoneal fibrosis, who illustrate the mainly abdominal and ENT tropism of the disease. Yet, thoracic involvement is not uncommon. It can be detected in up to 30% of patients with systemic IgG4-RD and is the exclusive manifestation of the disease in about 10% of cases. Clinical symptoms are nonspecific and may include dyspnoea, cough or chest pain. Chest CT findings are heterogeneous and primarily include peribronchovascular thickening, nodules, ground-glass opacities and lymphadenopathy. There is no specific diagnostic test for IgG4-RD thoracic involvement, which may mimic malignancy or vasculitis. Therefore, a cautious approach is needed to make an accurate diagnosis: a search for extra-thoracic manifestations, elevated serum IgG4 levels, circulating levels of plasmablasts and pathologic evidence of disease is warranted. Although very suggestive, neither the presence of a polyclonal IgG4 lymphoplasmacytic infiltrate, storiform fibrosis or obliterative phlebitis are sufficient to confirm the histological diagnosis. Steroids are recommended as first-line therapy. Rituximab or disease-modifying antirheumatic drugs may be used in relapsed or rare cases of steroid-refractory disease. In this review, we summarize current knowledge regarding the pathophysiology, epidemiology, diagnostic modalities (clinical-biological-imaging-histopathology) and treatment of IgG4-RD thoracic involvement.
Topics: Humans; Immunoglobulin G4-Related Disease; Lymphadenopathy; Fibrosis; Plasma Cells; Immunoglobulin G
PubMed: 36437514
DOI: 10.1111/resp.14422 -
Annals of the Rheumatic Diseases Mar 2019IgG4-related disease (IgG4-RD) is a heterogeneous, multiorgan condition of unclear aetiology that can cause organ failure. Difficulty recognising IgG4-RD contributes to...
OBJECTIVE
IgG4-related disease (IgG4-RD) is a heterogeneous, multiorgan condition of unclear aetiology that can cause organ failure. Difficulty recognising IgG4-RD contributes to diagnostic delays. We sought to identify key IgG4-RD phenotypes.
METHODS
We used two cross-sectional studies assembled by an international, multispecialty network of IgG4-RD specialists who submitted 765 cases to derive and replicate phenotypic groups. Phenotype groups of disease manifestations and key covariate distributions across the identified groups were measured using latent class analysis.
RESULTS
In the derivation cohort (n=493), we identified four groups with distinct manifestations: Group 1 (31%), Pancreato-Hepato-Biliary disease; Group 2 (24%), Retroperitoneal Fibrosis and/or Aortitis; Group 3 (24%), Head and Neck-Limited disease and Group 4 (22%), classic Mikulicz syndrome with systemic involvement. We replicated the identification of four phenotype groups in the replication cohort. Compared with cases in Groups 1, 2 and 4, respectively, cases in Group 3 were more likely to be female (OR 11.60 (95% CI 5.39 to 24.98), 10.35 (95% CI 4.63 to 23.15) and 9.24 (95% CI 3.53 to 24.20)) and Asian (OR 6.68 (95% CI 2.82 to 15.79), 7.43 (95% CI 2.97 to 18.56) and 6.27 (95% CI 2.27 to 17.29)). Cases in Group 4 had a higher median serum IgG4 concentration (1170 mg/dL) compared with groups 1-3 (316, 178 and 445 mg/dL, respectively, p<0.001).
CONCLUSION
We identified four distinctive IgG4-RD phenotypes according to organ involvement. Being Asian or female may predispose individuals to head and neck-limited disease. These phenotypes serve as a framework for identifying IgG4-RD and studying its aetiology and optimal treatment.
Topics: Adult; Americas; Aortitis; Asia; Asian People; Cross-Sectional Studies; Digestive System Diseases; Europe; Female; Humans; Immunoglobulin G; Immunoglobulin G4-Related Disease; Male; Middle Aged; Mikulicz' Disease; Otorhinolaryngologic Diseases; Phenotype; Racial Groups; Retroperitoneal Fibrosis
PubMed: 30612117
DOI: 10.1136/annrheumdis-2018-214603 -
Taiwan Journal of Ophthalmology 2018Immunoglobulin G4-related disease (IgG4-RD) is a recently recognized inflammatory disease of unknown etiology. It characterized by distinctive histopathological... (Review)
Review
Immunoglobulin G4-related disease (IgG4-RD) is a recently recognized inflammatory disease of unknown etiology. It characterized by distinctive histopathological appearance of dense IgG4-positive lymphoplasmacytic infiltrates, storiform fibrosis, and obliterative phlebitis in one or more organs, simultaneously or subsequently. In cases of ocular adnexal involvement, unique clinicohistopathological features were delineated by recent studies, and IgG4-related ophthalmic disease (IgG4-ROD) is generally recognized as the disease name. A significant proportion of previous labeled idiopathic orbital inflammations and Mikulicz's disease are now consistent with a diagnosis of IgG4-ROD. Increasing studies have accumulated regarding its epidemiology, diagnosis, clinical features, treatment, and the association between lymphoma. In this review, we summarize our present understanding of IgG4-ROD.
PubMed: 29675343
DOI: 10.4103/tjo.tjo_12_17 -
JMA Journal Mar 2019Immunoglobulin G4-related disease (IgG4-RD) is a recently established systemic disease that is characteristically associated with elevated serum immunoglobulin G4 (IgG4)... (Review)
Review
Immunoglobulin G4-related disease (IgG4-RD) is a recently established systemic disease that is characteristically associated with elevated serum immunoglobulin G4 (IgG4) levels and believed to be caused by autoimmune mechanisms. The clinical features of IgG4-RD include (i) systemic distribution, (ii) imaging findings of swelling, nodules, and/or wall thickening, (iii) high serum IgG4 levels, (iv) abundant IgG4-bearing plasma cell infiltration and fibrosis in affected organs, (v) a favorable response to corticosteroid therapy, and (vi) coexistence with other IgG4-RD manifestations simultaneously or in a metachronous fashion. The concept of IgG4-RD was established based on the culmination of specific discoveries. Specifically, a close association between autoimmune pancreatitis (AIP) and high serum IgG4 levels, massive IgG4-bearing plasma cell infiltration in pancreatic tissues affected by AIP, and systemic other organ involvements in AIP with similar IgG4-bearing plasma cell features opened the gateway from AIP to IgG4-RD. The systemic distribution of IgG4-RD seems to be capable of affecting every organ, causing well-established members including AIP, lacrimal and salivary gland lesions such as Mikulicz's disease, respiratory diseases, sclerosing cholangitis, kidney diseases, and retroperitoneal fibrosis. IgG4-RD has been diagnosed worldwide, and international collaboration efforts on the disease have led to consensus publications on its nomenclature, pathology findings, and management approach. The algorithms developed for the comprehensive diagnostic criteria for IgG4-RD have remarkably increased detection sensitivity. Oral glucocorticoids are the first-line agents for remission induction, and certain patients with high disease activity may benefit from maintenance therapy afterwards. Originally, IgG4-RD had been considered reversible and to have a good prognosis; however, long-term afflictions sometimes result in transition to advanced-stage conditions with dysfunction and/or complicating malignancy. The immunological abnormalities in IgG4-RD have been reported in both innate and adaptive immune systems; however, it remains unclear whether IgG4 has a pathogenic role or a protective one in disease onset and progression.
PubMed: 33681509
DOI: 10.31662/jmaj.2018-0017 -
Reumatologia 2020According to a new concept for the classification and division of autoimmune diseases, Mikulicz's disease and Küttner's tumor belong to immunoglobulin G4-related... (Review)
Review
According to a new concept for the classification and division of autoimmune diseases, Mikulicz's disease and Küttner's tumor belong to immunoglobulin G4-related diseases (IgG4-RD) and fulfil their diagnostic criteria. The aim of this study was to summarize the new classification concepts of IgG4-RD in the head and neck area and to review their clinical, histopathological and serologic criteria and the methods used in the diagnostic workup with respect to their advantages, limitations and differentiative value. The PubMed, Web of Science, Google Scholar, and Scopus databases were searched for articles published between 2009 and 2019 using the following key words: IgG4-related diseases, Mikulicz's disease, Küttner's tumor, salivary glands, xerostomia. Results of the review of the literature revealed that Mikulicz's disease and Küttner's tumor fulfil the same diagnostic criteria but may manifest different clinical symptoms which determine the choice of the different diagnostic tools.
PubMed: 32921832
DOI: 10.5114/reum.2020.98437 -
Clinics in Colon and Rectal Surgery Nov 2007Strictureplasty in patients with Crohn's disease is an option in the colorectal surgeon's armamentarium for fibrostenotic obstructive disease. Common types include the...
Strictureplasty in patients with Crohn's disease is an option in the colorectal surgeon's armamentarium for fibrostenotic obstructive disease. Common types include the Heineke-Mikulicz strictureplasty, Finney strictureplasty, and the side-to-side isoperistaltic strictureplasty. The procedure has potential for significant morbidity; therefore, it should be chosen for the patient carefully. Strictureplasty complements bowel resection in Crohn's disease; it is an excellent procedure to reduce the risk of developing short-bowel syndrome and its associated complications.
PubMed: 20011425
DOI: 10.1055/s-2007-991028 -
Internal Medicine (Tokyo, Japan) 2012
Topics: Granuloma, Plasma Cell; Humans; Immunoglobulin G; Kidney Diseases; Lymphoma, Large B-Cell, Diffuse; Male; Mikulicz' Disease
PubMed: 22333367
DOI: 10.2169/internalmedicine.51.6782 -
Proceedings of the Royal Society of... 1916
PubMed: 19979432
DOI: No ID Found -
Proceedings of the Royal Society of... 1912
PubMed: 19975642
DOI: No ID Found -
Proceedings of the Royal Society of... 1915
PubMed: 19978972
DOI: No ID Found