Lifetime Allergy Symptoms in <scp>IgG4‐Related</scp> Disease: A Case–Control Study

Sanders, Samantha; Fu, Xiaoqing; Zhang, Yuqing; Perugino, Cory A.; Wallwork, Rachel; Della‐Torre, Emanuel; Harvey, Liam; Harkness, Tyler; Long, Aidan A.; Choi, Hyon K.; Stone, John H.; Wallace, Zachary S

doi:10.1002/acr.24545

Cited by 15 publications

(30 citation statements)

References 30 publications

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“…Originally, an association between IgG4-RD and allergic diseases was suggested 1,13,14 . However, some more recent studies have indicated that allergic symptoms are not more frequent in IgG4-RD as compared to several control populations despite the common findings of serum IgE elevation and peripheral blood eosinophilia in the former [15][16][17] . Accordingly, any association of allergic predisposition with IgG4-RD pathogenesis or disease deterioration is yet unproven and remains to be clarified through further investigations.…”

Section: Discussionmentioning

confidence: 89%

Serum IgG4 levels at diagnosis can predict unfavorable outcomes of untreated patients with IgG4-related disease

Mizushima

Konishi

Sanada

et al. 2021

Sci Rep

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The outcomes of patients with immunoglobulin G4 (IgG4)-related disease (IgG4-RD) who are not treated are unclear. This study aimed to clarify these outcomes and identify the factors related to them. We retrospectively evaluated various clinical features including laboratory data and involved organs at diagnosis in 107 patients with IgG4-RD, who were followed up for more than 6 months, at a single center in Japan. We compared the clinical features of the 27 untreated patients with those of the 80 patients treated with glucocorticoid. The patient outcomes were investigated, and logistic regression analysis was performed to identify factors related to them. The patients comprised 73 men and 34 women (median age 67 years). The untreated patients had significantly lower IgG4-RD responder index (9 vs. 12) and fewer affected organs (1 vs. 3) than did those treated with glucocorticoid. Of these 27 patients, 8 experienced deterioration of IgG4-RD after the diagnosis. In the age- and sex-adjusted logistic regression analysis, serum IgG4 elevation (per 100 mg/dL, odds ratio 1.194, 95% confidence interval 1.017–1.402) was the only significant factor related to disease deterioration in untreated patients with IgG4-RD, whereas not serum IgG4 levels (per 100 mg/dL, odds ratio 0.995, 95% confidence interval 0.921–1.075) but history of allergy (OR 3.134, 95% confidence interval 1.094–8.977, P = 0.033) related to deterioration in patients who underwent treatment. Serum IgG4 levels may be a useful predictor of unfavorable outcomes in untreated patients with IgG4-RD, who tend to have fewer affected organs and lower IgG4-RD responder index.

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Section: Discussionmentioning

confidence: 89%

Serum IgG4 levels at diagnosis can predict unfavorable outcomes of untreated patients with IgG4-related disease

Mizushima

Konishi

Sanada

et al. 2021

Sci Rep

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“…To assess sensitivity of algorithms, we identified patients from the MGH Center for IgG4-RD registry who were enrolled in Medicare Fee-for-Service with Part A, B, and D coverage during the study period after the initial date of their IgG4-RD diagnosis. The MGH Center for IgG4-RD maintains a registry of all patients evaluated who have IgG4-RD confirmed to be definite, probable, or atypical according to the American College of Rheumatology (ACR) and European League Against Rheumatism (EULAR) classification criteria for IgG4-RD and previously described methods (7)(8)(9). This study was approved by the MGB Institutional Review Board.…”

Section: Methodsmentioning

confidence: 99%

“…Two authors (ZSW and JHS) reviewed cases identified by each algorithm and determined whether they were IgG4-RD on the basis of the classification criteria approved by the EULAR and ACR (7). We included patients who fell into one of three categories: 1) definite IgG4-RD, 2) probable IgG4-RD, and 3) atypical IgG4-RD (8,9). Patients in the definite category fulfilled the published classification criteria.…”

Section: Significance and Innovationmentioning

confidence: 99%

Derivation and Validation of Algorithms to Identify Patients With Immunoglobulin‐G4‐Related Disease Using Administrative Claims Data

Wallace

Cook

et al. 2022

ACR Open Rheumatology

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Objective Immunoglobulin‐G4‐related disease (IgG4‐RD) is a systemic autoimmune disease that can affect nearly any organ, but its epidemiology remains poorly understood. Validated algorithms to identify cases in claims data will enable studies to describe IgG4‐RD epidemiology in the general population. Methods Potential claims‐based algorithms were developed by IgG4‐RD experts using a combination of International Classification of Diseases, Ninth Revision (ICD‐9) and International Classification of Diseases, 10th Revision (ICD‐10) codes, dispensed medications, and procedure codes for immunoglobulin G (IgG) subclass testing. Algorithms were tested using Medicare Parts A, B, and D linked to medical records (2007‐2017). Classification of cases as IgG4‐RD was determined using the American College of Rheumatology (ACR) and European League Against Rheumatism (EULAR) classification criteria for IgG4‐RD. We estimated the positive predictive value (PPV) of each algorithm; sensitivity was determined using a cohort of patients with IgG4‐RD also enrolled in Medicare Parts A, B, and D during the study period. Results We identified seven algorithms that used a combination of ICD‐9 and ICD‐10 codes, medication prescriptions, and/or IgG subclass tests to identify patients with IgG4‐RD. The PPV of algorithms in the derivation cohort ranged from 57% to 100%, and sensitivity ranged from 0% to 58%. The best performing algorithm in the validation cohort had a PPV of 81% and a sensitivity of 58%. Typical IgG4‐RD manifestations were observed in the cohort (n = 36) assembled by this algorithm, including 50% with sialadenitis, 64% with pancreatic disease, 31% with renal disease, and 59% with an elevated IgG4 concentration. Conclusion We derived and validated a well‐performing algorithm to identify IgG4‐RD cases with typical manifestations of the disease. The claims‐based algorithm can be used in research studies of IgG4‐RD.

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“…this phenotype compared with others. 4,[6][7][8]34 Juvenile-onset IgG4-RD patients present more frequently with this phenotype. 35,36 Clinical, serological, radiological, and histological correlation is of paramount importance to diagnose this phenotype due to the myriad of differential diagnoses (Table ).…”

Section: Head and Neck Limited Phenotypementioning

confidence: 99%

IgG4-Related Disease

et al. 2021

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Background:The tendency of IgG4-related disease (IgG4-RD) to form pseudotumors, as well as its multisystemic nature, makes it the perfect mimicker of many conditions. Moreover, some clinical, serological, radiological, or histological features of the disease might be shared with some mimickers.Recently, 4 clinical phenotypes have been identified, and patients grouped in each phenotype have distinctive demographic, clinical, and serological features and outcomes, and, as expected, for each phenotype, a set of differential diagnoses should be considered. Summary of the Literature:The main differential diagnoses for the pancreato-hepato-biliary phenotype are pancreatic adenocarcinoma and cholangiocarcinoma. Other differential diagnoses include type 2 autoimmune pancreatitis and primary sclerosing cholangitis. In patients with retroperitoneal/aortic phenotype, inflammatory conditions such as idiopathic retroperitoneal fibrosis and large vessel vasculitides should be ruled out, and most of the time, a biopsy will be needed to exclude malignancies. In head and neck limited phenotype, autoimmune conditions (eg, granulomatosis with polyangiitis, Graves orbitopathy, sarcoidosis), malignancies, and histiocytosis should be ruled out, whereas the main differential diagnoses of the Mikulicz/ systemic phenotype are Sjögren syndrome, granulomatosis with polyangiitis, and multicentric Castleman disease.Conclusions: Approaching a patient with probable IgG4-RD through a clinical phenotype framework will ease the diagnostic algorithm and facilitate the prompt recognition of the disease. There are certain clinical, serological, radiological, and histological features in each clinical phenotype that, if present, increase the likelihood that a patient may have IgG4-RD instead of the mimicker condition. Those clues that point toward IgG4-RD diagnosis should be actively sought in the workup of patients.

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Lifetime Allergy Symptoms in IgG4‐Related Disease: A Case–Control Study

Cited by 15 publications

References 30 publications

Serum IgG4 levels at diagnosis can predict unfavorable outcomes of untreated patients with IgG4-related disease

Serum IgG4 levels at diagnosis can predict unfavorable outcomes of untreated patients with IgG4-related disease

Derivation and Validation of Algorithms to Identify Patients With Immunoglobulin‐G4‐Related Disease Using Administrative Claims Data

IgG4-Related Disease

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