scholarly journals Paratesticular fibrous pseudotumour: case report of a rare manifestation of IgG4-related disease

2021 ◽  
Vol 27 (1) ◽  
Author(s):  
Jeena Raju Kudunthail ◽  
Nikhil Dhimole ◽  
Gurpreet Singh ◽  
Savitri Honakeri
Keyword(s):  
Frozen Section ◽  
Radical Resection ◽  
Tumour Markers ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Benign Pathology ◽  
Frozen Section Analysis ◽  
Serum Igg4 ◽  
Rare Manifestation ◽  
Serum Igg Levels

Abstract Background Paratesticular fibrous pseudotumour is a rare benign growth with unclear pathogenesis and clinical management. It has been linked to Serum IgG4-related disease. This mass can notoriously masquerade around as a malignancy; however, a conservative (testis sparing) approach is sufficient as surgical management. Case presentation We present the case of a 35-year-old gentleman who presented with a slow growing paratesticular mass, raising the suspicion of a malignancy. However, normal tumour markers and benign appearance on imaging modalities compelled us to give trial of local excision with intra-operative frozen section analysis. After confirmation of benign pathology of the mass, the incision was closed. Final histopathology confirmed the diagnosis of benign paratesticular fibrous pseudotumour, and serum IgG levels were noted to be elevated. Conclusion Thus, using tumour markers, radiological imaging and intraoperative frozen section, a potential radical resection can be avoided, testis sparing surgery, employed.

scholarly journals Inflammatory pseudotumor of Castleman disease and IgG4-related disease masquerading as kidney malignancy

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Bolong Liu ◽  
Yong Huang ◽  
Luying Tang ◽  
Jiexia Guan ◽  
Xiangfu Zhou ◽  
...  
Keyword(s):  
Renal Pelvis ◽  
Inflammatory Pseudotumor ◽  
Frozen Section ◽  
Imaging Techniques ◽  
Castleman Disease ◽  
Diagnostic Challenge ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Kidney Involvement ◽  
Serum Igg4

Abstract Background With widespread clinical application of imaging techniques, renal space-occupying lesions have been identified at an increasing frequency. Here, we report two rare cases, Castleman disease (CD) and IgG4-related disease (IgG4-RD), presenting primarily with the symptoms and imaging findings of kidney malignancy. Case presentation In case 1, an occupying lesion located in the right renal pelvis was detected using magnetic resonance imaging in a 32-year-old female who presented with hematuria and lumbago. First misdiagnosed as carcinoma of the renal pelvis, the patient underwent right radical nephroureterectomy. However, postoperative pathological and immunohistochemistry studies finally confirmed the diagnosis of CD. In case 2, a 45-year-old male presented with the chief complaint of anuria. Nephrostomy and renal biopsy indicated lymphoma, following which, antegrade urography and computed tomography urography were performed, which revealed bilateral hydronephrosis and mass lesions around the renal pelvis. Partial resection of the masses and frozen section examination indicated the diagnosis of CD. However, the results of postoperative histopathology and immunohistochemistry combined with serum IgG4 were consistent with IgG4-RD. Both the patients recovered well after drug treatment without recurrence of the diseases. Conclusions Inflammatory pseudotumor of CD and IgG4-RD with kidney involvement are primarily diagnosed by postoperative histopathology and can pose a preoperative diagnostic challenge because these lesions can masquerade as kidney malignancy. Therefore, we recommend core biopsy as a nonnegligible procedure to evaluate renal masses and potentially prevent unnecessary surgical treatment.

scholarly journals Inflammatory Pseudotumor of Castleman Disease and IgG4-related Disease Masquerading as Kidney Malignancy

2021 ◽  
Author(s):  
Bolong Liu ◽  
Yong Huang ◽  
Luying Tang ◽  
Jiexia Guan ◽  
Xiangfu Zhou ◽  
...  
Keyword(s):  
Renal Pelvis ◽  
Inflammatory Pseudotumor ◽  
Frozen Section ◽  
Imaging Techniques ◽  
Castleman Disease ◽  
Diagnostic Challenge ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Kidney Involvement ◽  
Serum Igg4

Abstract Background: With widespread clinical application of imaging techniques, renal space-occupying lesions have been identified at an increasing frequency. Here, we report two rare cases, Castleman disease (CD) and IgG4-related disease (IgG4-RD), presenting primarily with the symptoms and imaging findings of kidney malignancy.Case presentation: In case 1, an occupying lesion located in the right renal pelvis was detected using magnetic resonance imaging in a 32-year-old female who presented with hematuria and lumbago. First misdiagnosed as carcinoma of the renal pelvis, the patient underwent right radical nephroureterectomy. However, postoperative pathological and immunohistochemistry studies finally confirmed the diagnosis of CD. In case 2, a 45-year-old male presented with the chief complaint of anuria. Nephrostomy and renal biopsy indicated lymphoma, following which, antegrade urography and computed tomography urography were performed, which revealed bilateral hydronephrosis and mass lesions around the renal pelvis. Partial resection of the masses and frozen section examination indicated the diagnosis of CD. However, the results of postoperative histopathology and immunohistochemistry combined with serum IgG4 were consistent with IgG4-RD. Both the patients recovered well after drug treatment without recurrence of the diseases.Conclusions: Inflammatory pseudotumor of CD and IgG4-RD with kidney involvement are primarily diagnosed by postoperative histopathology and can pose a preoperative diagnostic challenge because these lesions can masquerade as kidney malignancy. Although rare, we recommend that clinicians consider CD and IgG4-RD when diagnosing patients with renal masses to avoid unnecessary surgical treatment.

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 Evaluate the Differences in CT Features and Serum IgG4 Levels between Lymphoma and Immunoglobulin G4-Related Disease of the Orbit

2020 ◽  
Vol 9 (8) ◽  
pp. 2425
Author(s):  
Wei-Hsin Yuan ◽  
Anna Fen-Yau Li ◽  
Shu-Yi Yu ◽  
Ying-Yuan Chen ◽  
Chia-Hung Wu ◽  
...  
Keyword(s):  
Elevated Serum ◽  
Hounsfield Unit ◽  
Immunoglobulin G4 ◽  
Related Disease ◽  
Serum Igg4 ◽  
Orbital Lymphoma ◽  
Serum Igg Levels ◽  
Igg Level ◽  
Ct Features ◽  
Higher Sensitivity

Background: Benign immunoglobulin G4 (IgG4)-related orbital disease (IgG4-ROD)—characterized as tumors mimicking malignant orbital lymphoma (OL)—responds well to steroids, instead of chemotherapy, radiotherapy and/or surgery of OL. The objective of this study was to report the differences in computed tomography (CT) features and- serum IgG4 levels of IgG4-ROD and OL. Methods: This study retrieved records for patients with OL and IgG4-ROD from a pathology database during an eight-year-and-five-month period. We assessed the differences between 16 OL patients with 27 lesions and nine IgG4-ROD patients with 20 lesions according to prebiopsy CT features of lesions and prebiopsy serum IgG4 levels and immunoglobulin G (IgG) levels This study also established the receiver-operating curves (ROC) of precontrast and postcontrast CT Hounsfield unit scales (CTHU), serum IgG4 levels, serum IgG levels and their ratios. Results: Significantly related to IgG4-ROD (all p < 0.05) were the presence of lesions with regular borders, presence of multiple lesions—involving both lacrimal glands on CT scans—higher median values of postcontrast CTHU, postcontrast CTHU/precontrast CTHU ratios, serum IgG4 levels and serum IgG4/IgG level ratios. Compared to postcontrast CTHU, serum IgG4 levels had a larger area under the ROC curve (0.847 [95% confidence interval (CI): 0.674–1.000, p = 0.005] vs. 0.766 [95% CI: 0.615–0.917, p = 0.002]), higher sensitivity (0.889 [95% CI: 0.518–0.997] vs. 0.75 [95% CI: 0.509–0.913]), higher specificity (0.813 [95% CI: 0.544–0.960] vs. 0.778 [95% CI: 0.578–0.914]) and a higher cutoff value (≥132.5 mg/dL [milligrams per deciliter] vs. ≥89.5). Conclusions: IgG4-ROD showed distinct CT features and elevated serum IgG4 (≥132.5 mg/dL), which could help distinguish IgG4-ROD from OL.

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.

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