IgG4-related disease: Advances in the diagnosis and treatment

Brito-Zerón, Pilar; Bosch, Xavier; Ramos‐Casals, Manuel; Stone, John H.

doi:10.1016/j.berh.2016.07.003

Cited by 70 publications

(82 citation statements)

References 123 publications

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“…Moreover, 9% of the IgG4-RD cases did not have a biopsy, 37% lacked the classic histopathologic findings, and >40% did not meet previously defined cutoffs for IgG4+ plasma cell infiltrates (9). These criteria reflect the reality of clinical care and clinical investigation in IgG4-RD; clinicians consider a combination of factors when determining whether to classify a patient as having this disease (10). The 2019 IgG4-RD classification criteria are one of the first sets of classification criteria in rheumatology to include absolute exclusion criteria that are not based solely on having an alternative diagnosis, but rather focus on clinical, serologic, radiologic, and pathologic features.…”

Section: Discussionmentioning

confidence: 99%

“…Moreover, 9% of the IgG4‐RD cases did not have a biopsy, 37% lacked the classic histopathologic findings, and >40% did not meet previously defined cutoffs for IgG4+ plasma cell infiltrates . These criteria reflect the reality of clinical care and clinical investigation in IgG4‐RD; clinicians consider a combination of factors when determining whether to classify a patient as having this disease .…”

Section: Introductionmentioning

confidence: 99%

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The 2019 American College of Rheumatology/European League Against Rheumatism Classification Criteria for IgG4‐Related Disease

Wallace

Della‐Torre

Dicaire³

et al. 2019

Arthritis & Rheumatology

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384

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Objective IgG4‐related disease (IgG4‐RD) can cause fibroinflammatory lesions in nearly any organ. Correlation among clinical, serologic, radiologic, and pathologic data is required for diagnosis. This work was undertaken to develop and validate an international set of classification criteria for IgG4‐RD. Methods An international multispecialty group of 86 physicians was assembled by the American College of Rheumatology (ACR) and the European League Against Rheumatism (EULAR). Investigators used consensus exercises, existing literature, derivation and validation cohorts of 1,879 subjects (1,086 cases, 793 mimickers), and multicriterion decision analysis to identify, weight, and test potential classification criteria. Two independent validation cohorts were included. Results A 3‐step classification process was developed. First, it must be demonstrated that a potential IgG4‐RD case has involvement of at least 1 of 11 possible organs in a manner consistent with IgG4‐RD. Second, exclusion criteria consisting of a total of 32 clinical, serologic, radiologic, and pathologic items must be applied; the presence of any of these criteria eliminates the patient from IgG4‐RD classification. Third, 8 weighted inclusion criteria domains, addressing clinical findings, serologic results, radiology assessments, and pathology interpretations, are applied. In the first validation cohort, a threshold of 20 points had a specificity of 99.2% (95% confidence interval [95% CI] 97.2–99.8%) and a sensitivity of 85.5% (95% CI 81.9–88.5%). In the second, the specificity was 97.8% (95% CI 93.7–99.2%) and the sensitivity was 82.0% (95% CI 77.0–86.1%). The criteria were shown to have robust test characteristics over a wide range of thresholds. Conclusion ACR/EULAR classification criteria for IgG4‐RD have been developed and validated in a large cohort of patients. These criteria demonstrate excellent test performance and should contribute substantially to future clinical, epidemiologic, and basic science investigations.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

The 2019 American College of Rheumatology/European League Against Rheumatism Classification Criteria for IgG4‐Related Disease

Wallace

Della‐Torre

Dicaire³

et al. 2019

Arthritis & Rheumatology

Self Cite

384

207

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“…The pancreas is affected in 41% of the IgG4-related diseases. Eighty per cent of the type 1 AIP patients are males, the average age is 62 years, and typical symptoms are jaundice (75%), abdominal pain (48%) and weight loss [6]. Long-term complications include malabsorption and pancreoprivic diabetes due to endocrine and exocrine insufficiency.…”

Section: Manifestations In the Gastrointestinal Tractmentioning

confidence: 99%

“…Interestingly, men mostly are affected by IgG4-related pancreatitis, while women mostly present with inflammation of the salivary glands [5]. The average patient age is 61 years, but the youngest patient was only 14 years old [6]. The organs affected most frequently are the pancreas, the salivary glands, the kidneys, the lacrimal glands and the aorta.…”

Section: Introductionmentioning

confidence: 99%

IgG4-Related Diseases in the Gastrointestinal Tract: Clinical Presentation, Diagnosis and Treatment Challenges

et al. 2018

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Background: IgG4-related diseases are a rare but an important entity. Due to the variable clinical presentation, this multiorgan disease was attributed to single-organ systems for many years. Also, it often remains a challenge to differentiate between IgG4-related diseases and malignancies. The pathogenesis seems to be a mixture of Th1- and Th2- immune responses, whereas the role of the non-pathogenic IgG4 antibodies is still unclear. Histopathological characteristics are a lymphoplasmacellular infiltrate with IgG4+ plasma cells, a storiform fibrosis and an obliterative phlebitis. This can lead to the functional destruction of every organ affected. In most cases, glucocorticoid treatment leads to remission and is used as maintenance therapy as well. Immune modulatory therapies are employed in case of steroid resistance. However, a majority of patients achieve remission without any therapy. Summary: In this study, we review the current state-of-the-art regarding pathophysiology, diagnostics, organ manifestation and therapeutic approaches. Key Messages: While the diagnosis of IgG4-related diseases is still challenging, there have been significant improvements in diagnostic as well as in therapeutic approaches. This is partially due to a better understanding of the pathophysiology of the disease but also due to improved imaging modalities and novel, more targeted therapies.

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“…IgG4-related disease is a rare immune-mediated systemic disease of unknown origin with the capability of involving essentially any organ [18]. The most current investigational treatment approaches have focused on targeting cells of the Bcell lineage, including B-cell-depleting agents that can lead to attenuation of serological biomarkers of the disease.…”

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confidence: 99%