Journal ArticleDOI
The 2019 American College of Rheumatology/European League Against Rheumatism classification criteria for IgG4-related disease
Zachary S. Wallace,Ray Naden,Suresh T. Chari,Hyon K. Choi,Emanuel Della-Torre,Jean Francois Dicaire,Phillip A. Hart,Dai Inoue,Mitsuhiro Kawano,Arezou Khosroshahi,Marco Lanzillotta,Kazuichi Okazaki,Cory A. Perugino,Amita Sharma,Takako Saeki,Nicolas Schleinitz,Naoki Takahashi,Hisanori Umehara,Yoh Zen,John H. Stone +19 more
TLDR
In this article, an international multispecialty group of 86 physicians was assembled by the American College of Rheumatology (ACR) and the European League Against Rheumatism (EULAR).Abstract:
IgG4-related disease (IgG4-RD) can cause fibroinflammatory lesions in nearly any organ. Correlation among clinical, serological, radiological and pathological data is required for diagnosis. This work was undertaken to develop and validate an international set of classification criteria for IgG4-RD. 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 1879 subjects (1086 cases, 793 mimickers); and multicriterion decision analysis to identify, weight and test potential classification criteria. Two independent validation cohorts were included. A three-step classification process was developed. First, it must be demonstrated that a potential IgG4-RD case has involvement of at least one of 11 possible organs in a manner consistent with IgG4-RD. Second, exclusion criteria consisting of a total of 32 clinical, serological, radiological and pathological items must be applied; the presence of any of these criteria eliminates the patient from IgG4-RD classification. Third, eight weighted inclusion criteria domains, addressing clinical findings, serological results, radiological assessments and pathological interpretations, are applied. In the first validation cohort, a threshold of 20 points had a specificity of 99.2% (95% CI 97.2% to 99.8%) and a sensitivity of 85.5% (95% CI 81.9% to 88.5%). In the second, the specificity was 97.8% (95% CI 93.7% to 99.2%) and the sensitivity was 82.0% (95% CI 77.0% to 86.1%). The criteria were shown to have robust test characteristics over a wide range of thresholds. 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, epidemiological and basic science investigations.read more
Citations
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Journal ArticleDOI
IgG4-related disease: an update on pathophysiology and implications for clinical care.
TL;DR: The central role of B cells in the disease has been demonstrated by both the robust clinical responsiveness of IgG4-RD to B cell depletion and by the identification of multiple self-antigens that promote B cell expansion.
Journal ArticleDOI
The 2020 revised comprehensive diagnostic (RCD) criteria for IgG4-RD.
Hisanori Umehara,Kazuichi Okazaki,Shigeyuki Kawa,Hiroki Takahashi,Hiroshi Goto,Shoko Matsui,Nobukazu Ishizaka,Takashi Akamizu,Yasuharu Sato,Mitsuhiro Kawano +9 more
TL;DR: The Japanese IgG4 team has updated the 2011 comprehensive diagnostic criteria for IgG 4-RD and proposed the 2020 revised comprehensive diagnostic (RCD) criteria for igG4-RD, which consists of 3 domains; 1) Clinical and radiological features, 2) Serological diagnosis and 3) Pathological diagnosis.
Journal ArticleDOI
Advances in the diagnosis and management of IgG4 related disease
TL;DR: IgG4 related disease was recognized as a unified disease entity only 15 years ago as mentioned in this paper, and specialists are now familiar with most of its clinical manifestations, including the involvement of the pancreato-biliary tract, retroperitoneum/aorta, head and neck, and salivary glands.
Journal ArticleDOI
Fibroblast Activation Protein-Targeted PET/CT with 68Ga-FAPI for Imaging IgG4-Related Disease: Comparison to 18F-FDG PET/CT.
TL;DR: 68Ga-FAPI might be a promising imaging agent for the assessment of IgG4-RD and demonstrated significantly higher uptake than 18F-FDG in the matched disease in the pancreas, bile duct/liver, and salivary gland.
Journal ArticleDOI
European Guideline on IgG4-related digestive disease – UEG and SGF evidence-based recommendations:
J-Matthias Löhr,Ulrich Beuers,Miroslav Vujasinovic,Domenico Alvaro,Jens Brøndum Frøkjær,Frank Buttgereit,Gabriele Capurso,Emma L. Culver,Enrique de-Madaria,Emanuel Della-Torre,Sönke Detlefsen,Enrique Dominguez-Munoz,Piotr Czubkowski,Nils Ewald,Luca Frulloni,Natalya Gubergrits,Deniz Güney Duman,Thilo Hackert,Julio Iglesias-Garcia,Nikolaos Kartalis,Andrea Laghi,Frank Lammert,Fredrik Lindgren,Alexey Okhlobystin,Grzegorz Oracz,Andrea Párniczky,Raffaella Pozzi Mucelli,Vinciane Rebours,Jonas Rosendahl,Nicolas Schleinitz,Alexander Schneider,Eric F.H. van Bommel,Caroline S. Verbeke,Marie Pierre Vullierme,Heiko Witt +34 more
TL;DR: Treatment with glucocorticoids should be weight-based and initiated at a dose of 0.6–0.8 mg/kg body weight/day orally for 1 month to induce remission and then be tapered within two additional months.
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TL;DR: The ACR/EULAR classification criteria for SSc performed better than the 1980 ACR criteria and should allow for more patients to be classified correctly as having the disease.
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