scholarly journals IgG4-related disease: can non-classical histopathological features or the examination of clinically uninvolved tissues be helpful in the diagnosis?

2012 ◽  
Vol 65 (11) ◽  
pp. 963-969 ◽  
Author(s):  
Emma L Culver ◽  
Adrian C Bateman
Keyword(s):  
Cell Count ◽  
Plasma Cell ◽  
Supporting Evidence ◽  
Diagnostic Features ◽  
Histopathological Features ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Multisystem Involvement ◽  
Immune Mediated ◽  
Serum Igg4

IgG4-related disease (IgG4-RD) is an increasingly recognised inflammatory and fibrosing condition that commonly shows multisystem involvement. The disease may mimic malignancy and other inflammatory or immune-mediated disorders, but usually has a good response to corticosteroid therapy, underlining the requirement for recognition of the condition. Accurate diagnosis requires careful interpretation of varying combinations of serum IgG4 levels, radiological features and characteristic histopathological appearances within an appropriate clinical setting. The presence of ‘classical’ histopathological features together with an elevated tissue IgG4+ plasma cell count and IgG4 to IgG ratio is often diagnostic and at the very least can strongly support a clinicopathological diagnosis of IgG4-RD. The authors describe the most recent diagnostic criteria for IgG4-RD, especially the histopathological features. The authors then discuss the merits of examining tissues that may be more easily obtainable than those that commonly show the ‘classical’ histopathological features, but within which not all of these ‘diagnostic’ features may be present. The authors conclude that while a combination of ‘classical’ histopathological features and an elevated tissue IgG4+ plasma cell count is the gold standard for diagnosis, examination of tissues that show some but not all of these features can, in the appropriate context, provide useful supporting evidence for a clinicopathological diagnosis of IgG4-RD.

scholarly journals IgG4-Related Disease without Overexpression of IgG4: Pathogenesis Implications

2012 ◽  
Vol 2012 ◽  
pp. 1-4 ◽  
Author(s):  
Naoshi Nishina ◽  
Yuko Kaneko ◽  
Masataka Kuwana ◽  
Hironari Hanaoka ◽  
Hideto Kameda ◽  
...  
Keyword(s):  
Plasma Cell ◽  
Histopathological Examination ◽  
High Serum ◽  
Cell Infiltration ◽  
Pituitary Mass ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Plasma Cell Infiltration ◽  
Serum Igg4 ◽  
Systemic Lymphadenopathy

IgG4-related disease is a new disease group that affects multiple organs. It is characterized by high serum IgG4 and abundant infiltration of IgG4-bearing plasma cells in the affected organ. Here, we describe an intriguing case that suggested that IgG4-related disease might present without IgG4 overexpression or infiltration, at least during a relapse. A 47-year-old man had been diagnosed with systemic lupus erythematosus 15 years. He was admitted due to a pituitary mass, systemic lymphadenopathy, and multiple nodules in the lungs and kidneys. The serum IgG4 level was normal and histopathological examination of the pituitary mass showed abundant lymphocyte and plasma cell infiltration with very few IgG4-positive cells. When we examined specimens preserved from 15 years ago, we found high serum IgG4 levels and IgG4-bearing plasma cell infiltration. This resulted in a diagnosis of IgG4-related disease, and we considered the current episode to be a relapse without IgG4 overexpression. This case indicated that, to clarify the pathogenesis of IgG4-related disease, current cases should repeat specimen evaluations over the course of IgG4-related disease to define diagnostic markers.

scholarly journals A condition closely mimicking IgG4-related disease despite the absence of serum IgG4 elevation and IgG4-positive plasma cell infiltration

2016 ◽  
Vol 26 (5) ◽  
pp. 784-789 ◽  
Author(s):  
Satoshi Hara ◽  
Mitsuhiro Kawano ◽  
Ichiro Mizushima ◽  
Kazunori Yamada ◽  
Kentaro Fujita ◽  
...  
Keyword(s):  
Plasma Cell ◽  
Cell Infiltration ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Plasma Cell Infiltration ◽  
Serum Igg4

scholarly journals IgG4-Related Disease, the Malignancy Mimicker: Case Series from Bahrain

2018 ◽  
Vol 2018 ◽  
pp. 1-9
Author(s):  
Majeed Haider ◽  
Fatima Haji ◽  
Osama Alalwan ◽  
Eman Aljufairi ◽  
Tejal S. Shah
Keyword(s):  
Plasma Cells ◽  
Elevated Serum ◽  
Case Series ◽  
Variable Degree ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Immune Mediated ◽  
Serum Igg4 ◽  
Igg4 Level ◽  
Single Organ

IgG4-related disease is an evolving immune-mediated condition. The hallmark of this condition is IgG4(+) plasma cells infiltration of the affected organs accompanied by a variable degree of fibrosis and occasionally elevated serum IgG4 level. It links many conditions that were once recognized as isolated unrelated idiopathic single organ disorders (e.g., autoimmune pancreatitis, Mikulicz syndrome, and retroperitoneal fibrosis) under one umbrella. It usually presents clinically as tumor-like swelling of the involved organs that can be misdiagnosed as neoplasia. In this case series, we present four cases that were considered as neoplasia but turned out to be IgG4-related disease, we demonstrate the protean manifestations of this condition and variable organs involvement, and we share our experience in using rituximab as the steroid sparing immunosuppressant agent to control this disease.

Serum IgG4 levels outperform IgG4/IgG RNA ratio in differential diagnosis of IgG4-related disease

2020 ◽  
Author(s):  
L Schulte ◽  
F Arnold ◽  
F Siegel ◽  
J Backhus ◽  
L Perkhofer ◽  
...  
Keyword(s):  
Differential Diagnosis ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Serum Igg4

scholarly journals SAT0532 POSITIVE DISEASE-SPECIFIC AUTOANTIBODIES LOWER DIAGNOSTIC SENSITIVITY BUT HAVE LITTLE CLINICAL SIGNIFICANCE IN DIAGNOSING IgG4-RELATED DISEASE USING THE 2019 ACR/EULAR CLASSIFICATION CRITERIA IN DAILY CLINICAL PRACTICE

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1224.1-1224
Author(s):  
I. Mizushima ◽  
T. Yamano ◽  
H. Kawahara ◽  
S. Hibino ◽  
R. Nishioka ◽  
...  
Keyword(s):  
Clinical Practice ◽  
False Negative ◽  
Classification Criteria ◽  
Daily Clinical Practice ◽  
Exclusion Criterion ◽  
Inclusion Criteria ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Serum Igg4 ◽  
Disease Specific

Background:Recently, the 2019 ACR/EULAR classification criteria for IgG4-related disease (IgG4-RD) were published mainly to identify more homogeneous subjects for inclusion in clinical trials and observational studies [1]. However, although their high specificity is presumed to be useful to differentiate IgG4-RD from various mimickers, their value in daily clinical practice needs to be evaluated.Objectives:This study aimed to clarify the usefulness of the 2019 ACR/EULAR classification criteria for IgG4-RD and characteristics of false-negative patients in daily clinical practice.Methods:We retrospectively reviewed the medical records of 162 patients with IgG4-RD and 130 consecutive non-IgG4-RD patients (mimickers) diagnosed by experts whose serum IgG4 levels were measured at a single center in Japan. Using the collected data, we calculated sensitivity, specificity, and fulfillment rates for the entry criteria, exclusion criteria, and threshold of inclusion criteria points. In addition, to clarify the characteristics of false-negative cases in IgG4-RD, we performed an intergroup comparison of their clinical features including disease-specific autoantibodies.Results:Both the patients with IgG4-RD and mimickers were relatively old (66 and 65 years) with male predominance (67% and 60%). The final diagnoses of mimickers mainly consisted of cancer, lymphoma, vasculitis, sarcoidosis, multicentric Castleman’s disease, and atherosclerotic or infectious aortic aneurysm. The classification criteria had a sensitivity of 72.8% and a specificity of 100%. Of the 44 false-negative cases, one did not fulfill the entry criteria, 20 fulfilled one exclusion criterion, and 27 did not achieve sufficient inclusion criteria points. Compared with the true-positive cases, the false-negative cases had significantly fewer affected organs, lower serum IgG4 levels, higher serum CH50 levels, and lower prevalence of salivary/lacrimal gland and renal parenchymal lesions. They were also less likely to have had biopsies (61% vs 97%). Of note, positivity of disease-specific autoantibodies including SSA/Ro antibody, ANCA, ds-DNA antibody, and ACPA was the most common exclusion criterion fulfilled in 18 patients, only 2 of whom were diagnosed with a specific autoimmune disease (rheumatoid arthritis) complicated by IgG4-RD. The remaining 16 patients had no specific clinical symptoms related to such autoantibodies. In addition, compared with IgG4-RD patients without disease-specific autoantibodies, the 18 patients with them had almost equal serum IgG4 and complement levels, number of affected organs, and histopathology and immunostaining scores despite higher serum IgG and CRP levels.Conclusion:The present study suggests that the 2019 ACR/EULAR classification criteria for IgG4-RD has excellent diagnostic specificity and moderate sensitivity in daily clinical practice. Positive disease-specific autoantibodies alone, which lowered the sensitivity in this study, may have little clinical significance concerning the diagnosis of IgG4-RD.References:[1]Wallace ZS et al. The 2019 American College of Rheumatology/European League Against Rheumatism classification criteria for IgG4-related disease. Ann Rheum Dis. 2020 Jan;79(1):77-87.Disclosure of Interests:None declared

Establishment of a serum IgG4 cut-off value for the differential diagnosis of IgG4-related disease in Chinese population

2015 ◽  
Vol 26 (4) ◽  
pp. 583-587 ◽  
Author(s):  
Ping Li ◽  
Hua Chen ◽  
Chuiwen Deng ◽  
Ziyan Wu ◽  
Wei Lin ◽  
...  
Keyword(s):  
Differential Diagnosis ◽  
Chinese Population ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Serum Igg4

MO144OVERLAP SYNDROME OF ANTINEUTROPHIL CYTOPLASMIC ANTIBODY-ASSOCIATED VASCULITIS AND IGG4-RELATED DISEASE: DISTINCT CLINICOPATHOLOGIC CLUES FOR PRECISE DIAGNOSIS

2021 ◽  
Vol 36 (Supplement_1) ◽  
Author(s):  
Peifen Liang ◽  
Qianqian Han ◽  
Bo Liu ◽  
Qiongqiong Yang
Keyword(s):  
Remission Rate ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Overlap Syndrome ◽  
Clinicopathologic Characteristics ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Pubmed Database ◽  
Serum Igg4 ◽  
Precise Diagnosis ◽  
Atypical Manifestations

Abstract Background and Aims Both antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and IgG4-related disease (IgG4-RD) are multi-system inflammatory disorders. The coexistent of both diseases present the possibility of a new overlap syndrome which leads to different treatment and outcome. In this study, we aim to investigate the possibility and clinicopathologic clues to the diagnosis of this overlap syndrome. Method A case of a 67-year-old man in our hospital who exhibited the clinicopathologic characteristics of both AAV and IgG4-RD was presented. The serial alterations in serum parameters and treatment response of the case were followed up for the next 15 months. Then, a systematic literature review of the overlap syndrome was performed on PUBMED database from 1976 until January 2020. Results Forty-two patients fully met both AAV and IgG4-RD criteria in the literature. The median age was 60 years ranged from 31 to 73 years at diagnosis. Twenty-eight (66.7%) patients were men. Serum IgG4 concentration increased in 41 (97.6%) patients (median:395 mg/dl; range:177-876mg/dl). Forty-one patients (97.6%) tested positive for ANCA with 37 (90.2%) patients showing a specificity for MPO. Kidney histology of 23 (69.7%) patients presented pauci-immune necrotizing or crescentic glomerulonephritis and IgG4-relative tubulointerstitial nephritis. Glucocorticoids combined with cyclophosphamide therapy was commonly prescribed with a high remission rate within 3 months. Four common clinicopathologic features of the overlap syndrome were identified from the case and literature. Conclusion AAV may overlap with IgG4-RD while presenting atypical manifestations. Four common clinicopathologic characteristics could be used as specific clues to the diagnosis of overlap syndrome.

scholarly journals Usefulness of measuring serum IgG4 level as diagnostic and treatment marker in IgG4-related disease

2012 ◽  
Vol 35 (1) ◽  
pp. 30-37 ◽  
Author(s):  
Motohisa YAMAMOTO ◽  
Norihiro NISHIMOTO ◽  
Tetsuya TABEYA ◽  
Yasuyoshi NAISHIRO ◽  
Keisuke ISHIGAMI ◽  
...  
Keyword(s):  
Serum Igg4 Level ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Serum Igg4 ◽  
Igg4 Level

scholarly journals Immunoglobulin G4-related disease - diagnostic dilemma and importance of clinical judgement: a case report

2020 ◽  
Vol 8 (11) ◽  
pp. 4144
Author(s):  
Jagadeesh Chandrasekaran ◽  
Phani Krishna Machiraju
Keyword(s):  
Early Recognition ◽  
Elevated Serum ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Inflammatory Disorder ◽  
Imaging Features ◽  
Immunoglobulin G4 ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Serum Igg4 ◽  
The Right

Immunoglobulin G4 (IgG4)-related disease is a multi-organ, immune-mediated, fibro-inflammatory disorder characterized by tumefactive masses in the affected organs. Incidence and prevalence of IgG4-related disease (RD) are not clearly known and have slight male preponderance. It often involves multiple organs at the time of presentation or over the course of disease mimicking malignancy, Sjogren's syndrome, antineutrophil cytoplasmic antibodies associated vasculitis, infections. A thorough workup is needed to rule out these mimickers. A 33-year-old gentleman presented to us with history of progressive swelling in the right peri-orbital region for four years. On evaluation, abdominal imaging was notable for the sausage-shaped pancreas and hypoenchancing nodules in bilateral kidneys. Histological examination of right lacrimal gland revealed lymphoplasmacytic infiltrate and storiform fibrosis. Serum IgG4 levels were normal, and immunostaining was negative. A diagnosis of IgG4-RD was suggested because of multi-organ involvement, classical radiological and histopathological features. Awareness about IgG4-RD, an under-recognized entity is essential, as it is treatable, and early recognition may help in a favourable outcome. Appropriate use of clinicopathological, serological and imaging features in the right clinical context may help in accurate diagnosis. Elevated serum IgG4 levels and biopsy are not mandatory for the diagnosis.

Sign in / Sign up

Export Citation Format

Share Document