No overlap between IgG4-related disease and microscopic polyangiitis and granulomatosis with polyangiitis despite elevated serum IgG4 at diagnosis: a retrospective monocentric study

Yoo, Juyoung; Ahn, Sung Soo; Jung, Seung Min; Song, Jason Jungsik; Park, Yong Bum; Lee, Sang Won

doi:10.1007/s10067-018-4402-x

Cited by 25 publications

(20 citation statements)

References 21 publications

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“…C reactive protein (CRP) is usually normal except in some clinical manifestations such as retroperitoneal and aortic involvement, in which a slight increase can be observed 61626364656667. Marked elevation of acute phase reactants should raise concern about infectious or inflammatory conditions that closely mimic IgG4-RD, such as ANCA (anti-neutrophil cytoplasmic antibody) associated vasculitis and multicentric Castleman disease 68697071727374. Peripheral blood eosinophilia and increased serum IgE concentrations occur in almost 30% of patients 5462636465.…”

Section: Diagnosis Of Igg4 Related Diseasementioning

confidence: 99%

“…In these patients, baseline urinalysis with evaluation of the urinary sediment is warranted because decreased serum C3 and C4 concentrations might indicate subclinical or overt renal involvement 8485. Disease specific autoantibodies such as ANCA, SSA/Ro or SSB/La, double stranded DNA, RNP, and Sm, are not observed in IgG4-RD and should orient diagnosis towards mimicking autoimmune conditions 68697071727374…”

Section: Diagnosis Of Igg4 Related Diseasementioning

confidence: 99%

“…Notably, the first three histopathological features are critical for diagnosis, whereas immunohistochemistry is secondary in importance because mimicker neoplastic and inflammatory disorders can also show increased tissue IgG4 counts. Neutrophils, leukocytoclastic vasculitis, granulomas, and tissue necrosis do not belong to the spectrum of pathological features of IgG4-RD and, even if accompanied by a prominent IgG4 positive plasma cell infiltrate, should prompt exclusion of other inflammatory conditions such as ANCA associated vasculitis and sarcoidosis 1669707172737475…”

Section: Guidelinesmentioning

confidence: 99%

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Advances in the diagnosis and management of IgG4 related disease

Lanzillotta

Mancuso

Della‐Torre

2020

BMJ

199

172

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IgG4 related disease was recognized as a unified disease entity only 15 years ago. Awareness of IgG4 related disease has increased worldwide since then, and specialists are now familiar with most of its clinical manifestations. Involvement of the pancreato-biliary tract, retroperitoneum/aorta, head and neck, and salivary glands are the most frequently observed disease phenotypes, differing in epidemiological features, serological findings, and prognostic outcomes. In view of this multifaceted presentation, IgG4 related disease represents a great mimicker of many neoplastic, inflammatory, and infectious conditions. Histopathology remains key to diagnosis because reliable biomarkers are lacking. Recently released classification criteria will be invaluable in improving early recognition of the disease. IgG4 related disease is highly treatable and responds promptly to glucocorticoids, but it can lead to end stage organ failure and even death if unrecognized. Prolonged courses of corticosteroids are often needed to maintain remission because the disease relapses in most patients. Rapid advancement in our understanding of the pathophysiology of IgG4 related disease is leading to the identification of novel therapeutic targets and possible personalized approaches to treatment.

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Section: Diagnosis Of Igg4 Related Diseasementioning

confidence: 99%

Section: Diagnosis Of Igg4 Related Diseasementioning

confidence: 99%

Section: Guidelinesmentioning

confidence: 99%

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Advances in the diagnosis and management of IgG4 related disease

Lanzillotta

Mancuso

Della‐Torre

2020

BMJ

199

172

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“…In a recently published retrospective analysis, Yoo and colleagues investigated whether elevated serum IgG4 at the time of diagnosis of microscopic polyangiitis (MPA) and GPA may be associated with concurrent IgG4-related disease (IgG4-RD) in immunosuppressive drug-naïve patients [19]. The mean serum IgG did not vary between the groups, and among the 46 patients, they could not find patients who could be classified as IgG4-RD.…”

Section: Discussionmentioning

confidence: 99%

Cardiovascular and Lung Involvement in Patients with Autoimmune Pancreatitis

Nikolic

Brehmer

Panić

et al. 2020

JCM

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Introduction: Immunoglobulin G4-related disease (IgG4-RD) is a systemic immune-mediated disease characterised pathologically by the infiltration of IgG4-bearing plasma cells into the involved organs. Autoimmune pancreatitis (AIP) is a form of chronic pancreatitis with a heavy lymphocytic infiltration and two distinct histopathological subtypes, namely: lymphoplasmacytic sclerosing pancreatitis (AIP type 1) and idiopathic duct-centric pancreatitis (AIP type 2). Lung involvement and aortic involvement have been reported in 12% and 9% of patients with systemic IgG4-RD, respectively. In series including patients with AIP, both lung and aortic involvement were described in 2% of the patients. Most of the epidemiological data come from Japan, and there is a lack of information from Europe, especially the Scandinavian countries. Patients and methods: We performed a single-centre retrospective study on a prospectively collected cohort of patients diagnosed with AIP at the Department for Digestive Diseases at Karolinska University Hospital in Stockholm, Sweden, from 2004 to 2019. Demographic and clinical data were collected from the medical charts. Results: One hundred and thirty-three patients with AIP were analysed. Six patients were excluded because they lacked some of the clinical data relevant to the study. Demographic and clinical features of 127 patients were presented. There were 98 patients with AIP type 1-35 (35.7%) female and 63 (64.3%) male, with a mean age of 55.4 ± 18.2. Among them, 15 (15.3%) patients had lung and/or cardiovascular involvement-11 (11.2%) patients had lung involvement, 10 (10.2%) patients had cardiovascular involvement (six patients had both). Most of them (67.0%) had never smoked. The mean follow-up time of the patients with AIP type 1 was 49 months. Conclusions: Lung and/or cardiovascular involvement were diagnosed in 15 (15.3%) patients in our historical cohort of patients with AIP type 1. Most of the lung involvement was presented in the form of nodular lesions in the lungs, non-specific infiltrates, “ground-glass” appearance with pleura thickening, and effusion. Aortic involvement was a major form of vascular involvement in patients with AIP, as in previous published studies on patients with IgG4-RD.

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“…Serum IgG4 levels and oligoclonal plasmablasts are the two main biomarkers for diagnosis and/or prognosis accepted to date [9,10]. However, both tools had their limitations: IgG4 levels may be normal in up to a third of patients with IgG4-RD [11,12] and could be elevated in other conditions that are typically part of the differential diagnosis with IgG4-RD such as pancreatic malignancies, Erdheim-Chester disease and systemic vasculitides [13,14,15,16]; whereas, oligoclonal plasmablasts had a better speci city and positive predictive value, but their determination by ow cytometry is not easily accessible. Due to these shortcomings, different biomarkers have been proposed for IgG4-RD such as serum soluble IL-2 receptor, cc-chemokine ligand 18 and B cell-activating factor of the tumor necrosis factor family [17,18,19].…”

Section: Introductionmentioning

confidence: 99%

Serum immunoglobulin free light chains and their association with clinical phenotypes, serology and activity in patients with IgG4-related disease: a cross-sectional study.

Martín‐Nares

Saavedra‐González

Fagundo-Sierra

et al. 2020

Preprint

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Introduction: The clinical utility of serum immunoglobulin free light chains (sFLC) in IgG4-related disease (IgG4-RD) is unknown. Herein we evaluated their association with clinical phenotypes, serology and activity in patients with IgG4-RD.Methods: Cross-sectional study that included 45 patients with IgG4-RD, and as controls 25 with Sjögren’s syndrome (SS) and 15 with sarcoidosis. IgG4-RD patients were classified in clinical phenotypes: pancreato-hepato-biliary, retroperitoneum/aorta, head/neck-limited and Mikulicz/systemic; as well as proliferative vs. fibrotic phenotypes. We assessed the IgG4-RD Responder Index (IgG4-RD RI) at recruitment and measured IgG1, IgG4, κ and λ sFLC serum levels by turbidometry.Results: sFLC levels were similar among IgG4-RD, SS and sarcoidosis groups. Regarding the IgG4-RD patients, the mean age was 49 years, 24 (53.3%) were men and 55.5% had activity. Eight (17.7%) belonged to pancreato-hepato-biliary, 6 (13.3%) to retroperitoneum/aorta, 14 (31.1%) to head/neck-limited, 16 (35.5%) to Mikulicz/systemic phenotypes, whereas 36 (80%) to proliferative and 9 (20%) to fibrotic phenotypes. High κ sFLC, λ sFLC and κ/λ ratio were present in 29 (64.4%), 13 (28.9%) and 13 (28.9%) patients, respectively. There were no differences in sFLC among phenotypes. κ sFLC and κ/λ ratio correlated positively with the number of involved organs and IgG4-RD RI. Patients with renal involvement had higher k sFLC and λ sFLC. The AUC for k sFLC and λ sFLC, for renal involvement was 0.78 and 0.72, respectively. Active IgG4-RD had higher levels of κ sFLC and more frequently a high κ/λ ratio. The AUC for k sFLC and k/λ ratio for predicting active IgG4-RD was 0.67 and 0.70, respectively. sFLC correlated positively with IgG1 and IgG4 levels.Conclusions: sFLC may be useful as a biomarker of disease activity as well as multiorgan and renal involvement. In particular, a high κ/λ ratio may identify patients with active disease.

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No overlap between IgG4-related disease and microscopic polyangiitis and granulomatosis with polyangiitis despite elevated serum IgG4 at diagnosis: a retrospective monocentric study

Cited by 25 publications

References 21 publications

Advances in the diagnosis and management of IgG4 related disease

Advances in the diagnosis and management of IgG4 related disease

Cardiovascular and Lung Involvement in Patients with Autoimmune Pancreatitis

Serum immunoglobulin free light chains and their association with clinical phenotypes, serology and activity in patients with IgG4-related disease: a cross-sectional study.

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