An International Multispecialty Validation Study of the IgG4‐Related Disease Responder Index

Wallace, Zachary S; Khosroshahi, Arezou; Carruthers, Mollie D.; Perugino, Cory A.; Choi, Hyon K.; Campochiaro, Corrado; Culver, Emma L.; Cortazar, Frank B.; Della‐Torre, Emanuel; Ebbo, Mikaël; Fernandes, Ana D.; Frulloni, Luca; Hart, Phil A.; Karadağ, Ömer; Kawa, Shigeyuki; Kawano, Mitsuhiro; Kim, Myung Hwan; Lanzillotta, Marco; Matsui, Shoko; Okazaki, Kazuichi; Ryu, Jay H.; Saeki, Takako; Schleinitz, N.; Tanasa, Paula; Umehara, Hisanori; Webster, George; Zhang, Wen; Stone, John H.

doi:10.1002/acr.23543

Cited by 127 publications

(102 citation statements)

References 13 publications

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“…The demographic characteristics of patients with IgG4-RD are summarized in Table 2. The IgG4-RD responder index (IgG4-RD RI) score was obtained as described 32 . Rhinosinusitis, bronchial asthma, and drug-allergy were defined as allergic symptoms 13 .…”

Section: Methodsmentioning

confidence: 99%

Thymus and Activation-regulated Chemokine as a Biomarker for IgG4-related Disease

Umeda

Origuchi

Kawashiri

et al. 2020

Sci Rep

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High serum concentrations of thymus and activation-regulated chemokine (TARC) are observed in allergic diseases such as atopic dermatitis and bronchial asthma. Frequent allergic symptoms have been reported in patients with IgG4-related disease (IgG4-RD). We investigated the pathogenic role of TARC as a biomarker in IgG4-RD patients. We evaluated the serum concentrations of TARC from 29 IgG4-RD patients, 28 primary Sjögren syndrome (pSS) patients, and 23 healthy controls (HCs) by enzyme-linked immunosorbent assay (ELISA). We analyzed the correlations between the TARC concentrations and the subjects' clinical parameters. To investigate the biological effect of TARC on the pathogenesis of IgG4-RD, we evaluated the in vitro induction of plasmablasts from IgG4-RD patients by TARC. The serum concentrations of TARC in the IgG4-RD patients were significantly higher than those of the pSS patients and HCs. The serum TARC concentration of the IgG4-RD group was positively correlated with the IgG4-RD responder index (IgG4-RD RI) score and with the number of organs involved, but it was not correlated with the serum IgG4 level or eosinophil number in the IgG4-RD patients' peripheral blood. The patients who had lung involvement had higher serum TARC concentrations. In vitro, TARC clearly induced the formation of plasmablasts from the IgG4-RD patients' peripheral blood mononuclear cells. Collectively, our data suggest that a systemic increment of TARC may contribute to the development of IgG4-RD through an aberrant induction of plasmablasts.

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Section: Methodsmentioning

confidence: 99%

Thymus and Activation-regulated Chemokine as a Biomarker for IgG4-related Disease

Umeda

Origuchi

Kawashiri

et al. 2020

Sci Rep

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“…12 , 13 , 14 The IgG4-RD Responder Index, designed to serve as a clinical trials outcome measure, is now undergoing a worldwide validation study. 15, 16 …”

Section: Introduction and Clinical Overviewmentioning

confidence: 99%

Emerging Treatment Models in Rheumatology: IgG4‐Related Disease: Insights Into Human Immunology and Targeted Therapies

Perugino

Mattoo

Mahajan

et al. 2017

Arthritis & Rheumatology

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“…Immunoglobulin subclass G4‐RD activity, organ damage, and response to treatment were assessed using the IgG4 Responder Index (IgG4‐RI) . This assessment tool has recently been validated in an international multi‐speciality study of IgG4‐RD for assessment of disease activity and longitudinally for treatment response . Scoring for disease activity (present in the preceding 28 days) and organ damage is given for 24 standard organ/sites, incorporating higher weights for disease manifestations that urgently require treatment or that worsen despite treatment.…”

Section: Methodsmentioning

confidence: 99%

“…The diagnosis of IgG4-RD was made using the HISORt criteria (2006/7) for IgG4-related pancreatic and biliary disease 14 and the Japanese Comprehensive Diagnostic Criteria (2011) for systemic disease. 15 Organ involvement included the bile ducts (22), pancreas (18), salivary glands (6), lacrimal glands (2), lymph nodes (3), lung (5), kidney (2), mesentery (3), retroperitoneum (1), and aorta (1).…”

Section: Methodsmentioning

confidence: 99%

“…17 This assessment tool has recently been validated in an international multi-speciality study of IgG4-RD for assessment of disease activity and longitudinally for treatment response. 18 Scoring for disease activity (present in the preceding 28 days) and organ damage is given for 24 standard organ/sites, incorporating higher weights for disease manifestations that urgently require treatment or that worsen despite treatment. Disease activity reflects a patient's symptoms attributable to active IgG4-RD as well as significant findings from the physical examination, imaging studies, and laboratory evaluations.…”

Section: Methodsmentioning

confidence: 99%

See 1 more Smart Citation

Unique patterns of glycosylation in immunoglobulin subclass G4‐related disease and primary sclerosing cholangitis

Culver

Bovenkamp

Derksen

et al. 2018

J of Gastro and Hepatol

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Background and AimImmunoglobulin subclass G4‐related disease (IgG4‐RD) is characterized by an abundance of IgG4 antibodies in the serum and tissue. Glycosylation status of antibodies can impact on immune effector functions and disease pathophysiology. We sought to establish glycosylation patterns in a prospective cohort of patients with IgG4‐RD and the relationship with disease activity and response to treatment.MethodsWe assessed IgG Fc‐tail and Fab‐arm glycosylation status in patients with IgG4‐RD (n = 22), disease controls with primary sclerosing cholangitis (PSC) (n = 22), and healthy controls (n = 22). Serum IgG and subclasses were quantified using ELISA. Fc and Fab glycosylation were analyzed by mass spectrometry and lectin affinity chromatography, respectively. Disease activity, organ damage, and response to treatment were assessed using the IgG4 Responder Index.ResultsImmunoglobulin G Fab sialylation was increased in IgG4‐RD compared with PSC and healthy control (P = 0.01), with a preferential increase in IgG4‐specific Fab sialylation, which was independent of IgG4 Fab‐arm exchange. There was a reduction in IgG1‐specific Fc bisection and hybrid structures in IgG4‐RD (P < 0.01), which recovered upon steroid treatment and correlated with disease activity. Overall, IgG Fc galactosylation was reduced in both IgG4‐RD and PSC (P < 0.01), with a preferential reduction in IgG1‐specific sialylation and enhancement of IgG4‐specific bisection in PSC. IgG4 fucosylation and IgG1/2/3 hybrid structures negatively correlated with complement C3 and C4 levels in IgG4‐RD (P < 0.01), but not PSC.ConclusionWe report the first study showing unique antibody glycosylation status in a prospective cohort of IgG4‐RD and PSC patients, which may determine modulation of the immune system and contribute to disease pathophysiology.

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An International Multispecialty Validation Study of the IgG4‐Related Disease Responder Index

Abstract: In this international, multispecialty study, we observed that the RI is a valid and reliable disease activity assessment tool that can be used to measure response to therapy.

Cited by 127 publications

References 13 publications

Thymus and Activation-regulated Chemokine as a Biomarker for IgG4-related Disease

Thymus and Activation-regulated Chemokine as a Biomarker for IgG4-related Disease

Emerging Treatment Models in Rheumatology: IgG4‐Related Disease: Insights Into Human Immunology and Targeted Therapies

Unique patterns of glycosylation in immunoglobulin subclass G4‐related disease and primary sclerosing cholangitis

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