scholarly journals Does the Intensity of IGG4 Immunostaining Have a Correlation with the Clinical Presentation of Riedel’s Thyroiditis?

2018 ◽  
Vol 2018 ◽  
pp. 1-4
Author(s):  
C. A. Simões ◽  
M. R. Tavares ◽  
N. M. M. Andrade ◽  
T. M. Uehara ◽  
R. A. Dedivitis ◽  
...  
Keyword(s):  
Plasma Cells ◽  
Clinical Presentation ◽  
Airway Protection ◽  
High Power Field ◽  
Anterior Neck ◽  
Obliterative Phlebitis ◽  
Riedel’S Thyroiditis ◽  
Storiform Fibrosis ◽  
Riedel's Thyroiditis ◽  
Progressive Enlargement

Riedel’s thyroiditis (RT) represents one type of IgG4-related thyroid disease (IgG4RTD) and the diagnosis involves quantitative immunohistochemistry showing dense lymphoplasmacellular inflammatory infiltrate consisting of IgG4-positive plasma cells with storiform fibrosis and obliterative phlebitis. We report a case of RT with progressive enlargement of the anterior neck, severe dysphagia, odynophagia, and dyspnea. The patient underwent surgical decompression of the airway, protection tracheotomy, and gastrostomy for nutritional intake 6 months after first symptoms. Complete resolution occurred after surgical treatment combined with prednisolone. Immunostaining revealed IgG4-positive plasma cells 12/HPF (high-power field) and the IgG4/IgG ratio 25%, values that were disproportionate to the intensity of the patient’s symptoms. As to this case and the few cases described and analyzed in the literature, our impression is that there is no relation between the intensity of symptoms in RT with the total number of IgG4-positive plasma cells and the IgG4/IgG ratio, but more studies are needed.

scholarly journals Needle biopsy compared with surgical biopsy: pitfalls of small biopsy in histologial diagnosis of IgG4-related disease

2021 ◽  
Vol 23 (1) ◽  
Author(s):  
Yanying Liu ◽  
Fei Yang ◽  
Xiying Chi ◽  
Yuxin Zhang ◽  
Jiangnan Fu ◽  
...  
Keyword(s):  
Needle Biopsy ◽  
Plasma Cells ◽  
Classification Criteria ◽  
Histological Diagnosis ◽  
Obliterative Phlebitis ◽  
Surgical Biopsy ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Significant Difference ◽  
Storiform Fibrosis

Abstract Objective The growing utilization of needle biopsy has challenged the current pathology consensus of IgG4-related disease (IgG4-RD). The aims of this study were to identify the histological characteristics of needle biopsy and surgical specimens and evaluate the ability of needle biopsy in histological diagnosis of IgG4-RD. Methods Biopsies from patients who were referred to as IgG4-RD by the 2019 ACR/EULAR IgG4-RD classification criteria in Peking University People’s Hospital from 2012 to 2019 were re-evaluated. Typical histological features and diagnostic categories were compared between needle biopsy and surgical biopsy. Results In total, 69 patients met the 2019 ACR/EULAR classification criteria and 72 biopsies of them were re-evaluated. All cases showed lymphoplasmacytic infiltrate, while storiform fibrosis and obliterative phlebitis were only present in 35 (48.6%) and 23 (31.9%) specimens, respectively. Storiform fibrosis was more likely to be seen in retroperitoneum lesion (P = 0.033). Surgical biopsy showed significantly higher IgG4+ plasma cells/high-power field (IgG4/HPF) count (P < 0.01) and higher proportion of IgG4/HPF > 10 (P < 0.01). No significant difference was observed with regard to the ratio of IgG4+ plasma cells/IgG+ plasma cells (IgG4/IgG) (P = 0.399), storiform fibrosis (P = 0.739), and obliterative phletibis (P = 0.153). According to the 2011 comprehensive diagnostic criteria, patients who performed a needle biopsy were less likely to be probable IgG4-RD (P = 0.045). Based on the 2011 pathology consensus, needle biopsy was less likely to be diagnosed as IgG4-RD (P < 0.01), especially to be highly suggestive IgG4-RD (P < 0.01). Only 1/18 (5.6%) needle salivary specimens fulfilled the cutoff of IgG4/HPF > 100, which was significantly less than 15/23 (65.2%) of surgical ones (P < 0.01). Conclusions Needle biopsy shows an inferiority in detecting IgG4/HPF count but not in IgG4/IgG ratio, storiform fibrosis, and obliterative phlebitis. Compared with surgical samples, needle biopsy is less likely to obtain a histological diagnosis of IgG4-RD. A different IgG4/HPF threshold for needle biopsy of the salivary glands may be considered.

scholarly journals IgG4-Related Sclerosing Cholangitis: Rarely Diagnosed, but not a Rare Disease

2021 ◽  
Vol 2021 ◽  
pp. 1-16
Author(s):  
Sylvia Drazilova ◽  
Eduard Veseliny ◽  
Patricia Denisa Lenartova ◽  
Dagmar Drazilova ◽  
Jakub Gazda ◽  
...  
Keyword(s):  
Diagnostic Criteria ◽  
Sclerosing Cholangitis ◽  
Plasma Cells ◽  
Histological Finding ◽  
Good Prognosis ◽  
High Serum ◽  
Obliterative Phlebitis ◽  
Igg4 Related Disease ◽  
Serum Igg4 ◽  
Storiform Fibrosis

IgG4-related sclerosing cholangitis, a biliary manifestation of an IgG4-related disease, belongs to the spectrum of sclerosing cholangiopathies which result in biliary stenosis. It presents with signs of cholestasis and during differential diagnosis it should be distinguished from cholangiocarcinoma or from other forms of sclerosing cholangitis (primary and secondary sclerosing cholangitis). Despite increasing information and recently established diagnostic criteria, IgG4-related sclerosing cholangitis remains underdiagnosed in routine clinical practice. The diagnosis is based on a combination of the clinical picture, laboratory parameters, histological findings, and a cholangiogram. Increased serum IgG4 levels are nonspecific but are indeed a part of the diagnostic criteria proposed by the Japan Biliary Association and the HISORt criteria for IgG4-SC. High serum IgG4 retains clinical utility depending on the magnitude of elevation. Approximately 90% of patients have concomitant autoimmune pancreatitis, while 10% present with isolated biliary involvement only. About 26% of patients have other organ involvement, such as IgG4-related dacryoadenitis/sialadenitis, IgG4-related retroperitoneal fibrosis, or IgG4-related renal lesions. A full-blown histological finding characterized by IgG4-enriched lymphoplasmacytic infiltrates, obliterative phlebitis, and storiform fibrosis is difficult to capture in practice because of its subepithelial localization. However, the histological yield is increased by immunohistochemistry, with evidence of IgG4-positive plasma cells. Based on a cholangiogram, IgG-4 related sclerosing cholangitis is classified into four subtypes according to the localization of stenoses. The first-line treatment is corticosteroids. The aim of the initial treatment is to induce clinical and laboratory remission and cholangiogram normalization. Even though 30% of patients have a recurrent course, in the literature data, there is no consensus on chronic immunosuppressive maintenance therapy. The disease has a good prognosis when diagnosed early.

scholarly journals Needle Biopsy Compared With Surgical Biopsy: Pitfalls of Small Biopsy in Histologial Diagnosis of IgG4-related Disease

2020 ◽  
Author(s):  
Yanying Liu ◽  
Fei Yang ◽  
Xiying Chi ◽  
Yuxin Zhang ◽  
Jiangnan Fu ◽  
...  
Keyword(s):  
Needle Biopsy ◽  
Classification Criteria ◽  
Histological Diagnosis ◽  
High Power Field ◽  
Obliterative Phlebitis ◽  
Surgical Biopsy ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Significant Difference ◽  
Storiform Fibrosis

Abstract ObjectiveThe growing utilization of needle biopsy has challenged the current pathology consensus of IgG4-related disease (IgG4-RD). The aims of this study were to identify the histological characteristics of needle biopsy and surgical specimens, and evaluate the ability of needle biopsy in histological diagnosis of IgG4-RD.MethodsBiopsies from patients who were referred to as IgG4-RD by the 2019 ACR/EULAR IgG4-RD classification criteria in Peking University People’s Hospital from 2012 to 2019 were re-evaluated. Typical histological features and diagnostic categories were compared between needle biopsy and surgical biopsy.ResultsIn total, 69 patients met the 2019 ACR/EULAR classification criteria and 72 biopsies of them were re-evaluated. All cases showed lymphoplasmacytic infiltrate, while storiform fibrosis and obliterative phlebitis were only present in 35 (48.6%) and 23 (31.9%) specimens, respectively. Storiform fibrosis was more likely to be seen in retroperitoneum lesion (P=0.033). Surgical biopsy showed significantly higher IgG4+ plasma cells/high power field (IgG4/HPF) count (P<0.01) and higher proportion of IgG4/HPF>10 (P<0.01). No significant difference was observed with regard to the ratio of IgG4+/IgG+ cells (IgG4/IgG) (P=0.399), storiform fibrosis (P=0.739), and obliterative phletibis (P=0.153). According to the 2011 comprehensive diagnostic criteria, patients who performed a needle biopsy were less likely to be probable IgG4-RD (P=0.045). Based on the 2011 pathology consensus, needle biopsy was tougher to be diagnosed as IgG4-RD (P<0.01), especially to be highly suggestive IgG4-RD (P<0.01). Only 1/18 (5.6%) needle salivary specimens fulfilled the cutoff of IgG4/HPF>100, which was significantly less than 15/23 (65.2%) of surgical ones (P<0.01).ConclusionsNeedle biopsy shows an inferiority in detecting IgG4/HPF count but not in IgG4/IgG ratio, storiform fibrosis and obliterative phlebitis. Compared with surgical samples, it is tougher for needle biopsy to obtain a histological diagnosis of IgG4-RD. A different IgG4/HPF threshold for needle biopsy of salivary glands may be considered.

IgG4-related disease: an analysis of the clinicopathological spectrum: UK centre experience

2021 ◽  
pp. jclinpath-2021-207748
Author(s):  
Wajira Dassanayaka ◽  
Kanchana Sanjeewani Liyanaarachchi ◽  
Aftab Ala ◽  
Izhar N Bagwan
Keyword(s):  
Plasma Cells ◽  
High Serum ◽  
High Power Field ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Serum Igg4 ◽  
Storiform Fibrosis ◽  
Common Clinical Presentation ◽  
Characteristic Features

AimTo retrospectively evaluate the characteristic clinicopathological spectrum in patients with suspicion of IgG4-related disease (IgG4RD).MethodsWinpath histology database from January 2011 to April 2018 identified all suspected IgG4RD cases wherein IgG4 immunohistochemistry was performed. The histology slides were reviewed to categorise cases into Boston criteria groups—highly suggestive of IgG4RD, probable IgG4RD and insufficient evidence. Information regarding clinical data, treatment received, follow-up and serum IgG4 levels was obtained from medical records and AllScripts Patient Administration System (APAS) clinical database.ResultsThe study included 204 patients and the most common sites of biopsy/resection were pancreas and duodenum. The most common clinical presentation was fibroinflammatory lesion or mass/lump. On histology, 54/204 (26.47%) cases showed typical storiform fibrosis, 65/204 (32.64%) had >10 IgG4+ plasma cells per high power field and only one case showed thrombophlebitis (0.49%). There were 14/204 (6.78%) cases categorised as highly suggestive of IgG4RD; 8 of these showed high serum IgG4 levels and were managed clinically as true IgG4RD.ConclusionHistological diagnosis of IgG4RD remains challenging, as not all characteristic features are always present especially in small biopsies. Due to the novelty of its experience, fear of over diagnosis in the context of malignancy and features overlapping with diseases of similar clinical scenario, diagnosis of IgG4RD has become more puzzling. Further multicentre clinical trials/studies are advisable.

scholarly journals Clinical and Image Characteristics of IgG4-Related Sclerosing Cholecystitis

Diagnostics ◽  
2021 ◽  
Vol 11 (8) ◽  
pp. 1358
Author(s):  
Masaki Kuwatani ◽  
Naoya Sakamoto
Keyword(s):  
Gallbladder Cancer ◽  
Plasma Cells ◽  
Image Features ◽  
Wall Thickening ◽  
Disease Entity ◽  
Obliterative Phlebitis ◽  
Image Characteristics ◽  
Morphological Finding ◽  
Mucosal Layer ◽  
Storiform Fibrosis

Since autoimmune pancreatitis (AIP) was established as a new disease entity, sclerosing change with abundant immunoglobulin-4 (IgG4)-positive plasma cells, storiform fibrosis, and obliterative phlebitis are main pathological features in IgG4-related diseases. Regarding IgG4-related sclerosing cholecystitis (IgG4-CC), which is occasionally associated with AIP cases and is rarely isolated, there are no diagnostic criteria and insufficient perceptions of the image findings. Although there have been some reports on IgG4-CC, differentiation between IgG4-CC and gallbladder cancer is very difficult in some cases with a localized lesion. In this review, we especially focused on image findings of IgG4-CC and summarized its image features for diagnostic assistance. The ultrasonography and CT findings of IgG4-CC could be classified into diffuse and localized types. Based on these findings, the presence of wall thickening with an intact or smooth mucosal layer, followed by a homogenously thickened outer layer, would be a helpful morphological finding to distinguish IgG4-CC from gallbladder cancer.

IgG4-Related Sclerosing Disease of the Breast in a Male Patient

2017 ◽  
Vol 25 (8) ◽  
pp. 711-715 ◽  
Author(s):  
Taisia Vitkovski ◽  
Galina S. Marder ◽  
Dominic A. Filardi ◽  
Ekta Gupta ◽  
Frank Breuer
Keyword(s):  
Male Patient ◽  
Plasma Cells ◽  
Excisional Biopsy ◽  
Breast Mass ◽  
High Power Field ◽  
Obliterative Phlebitis ◽  
Igg4 Related Disease ◽  
First Case ◽  
History Of ◽  
Skin Retraction

IgG4-related sclerosing disease of the breast is a rare entity with 10 reports in the literature. We report the first case in a male patient. A 48-year-old male presented with 4-week history of palpable right upper outer quadrant breast mass associated with skin puckering. He reported a family history of breast cancer in his aunt. Ultrasound and mammography showed a spiculated 2.5-cm mass associated with skin retraction with extension to the pectoralis muscle. Ultrasound-guided core biopsy was performed. The findings were interpreted as acute and chronic inflammatory process. The patient subsequently denied improvement of the mass, and an excisional biopsy was performed. Histologic examination showed fibrosis and dense lymphoplasmacytic inflammation in the breast mass and muscle biopsy. Focal obliterative phlebitis was noted. IgG4-positive plasma cells were increased with counts of over 50 per high-power field. The diagnosis was confirmed as sclerosing IgG4-related disease of breast.

scholarly journals Riedel’s thyroiditis: clinical presentation, treatment and outcomes

Endocrine ◽  
2018 ◽  
Vol 60 (1) ◽  
pp. 185-192 ◽  
Author(s):  
Henrik Falhammar ◽  
Carl Christofer Juhlin ◽  
Caroline Barner ◽  
Sergiu-Bogdan Catrina ◽  
Christos Karefylakis ◽  
...  
Keyword(s):  
Clinical Presentation ◽  
Riedel’S Thyroiditis ◽  
Riedel's Thyroiditis

IgG4-related disease: an atypical presentation of steroid-responsive renal mass

2021 ◽  
Vol 14 (5) ◽  
pp. e240611
Author(s):  
Shu Hui Ng ◽  
Jing Shin Tay ◽  
Ee Ling Lai
Keyword(s):  
Renal Mass ◽  
Plasma Cells ◽  
Normal Serum ◽  
Obliterative Phlebitis ◽  
Related Disease ◽  
First Line Therapy ◽  
Igg4 Related Disease ◽  
Serum Igg ◽  
Storiform Fibrosis ◽  
Lymphoplasmacytic Infiltration

IgG4-related disease (IgG4-RD) is a systemic fibroinflammatory disease characterised by dense lymphoplasmacytic infiltration rich in IgG4-positive plasma cells, storiform fibrosis and obliterative phlebitis. Serum IgG4 levels are typically elevated but half of the patients had normal serum IgG4 levels. IgG4-RD represents a spectrum of diseases that involve various organs such as the pancreas, liver, kidneys, and salivary glands often manifesting as diffuse organ enlargement or a mass-like lesion mimicking cancer. An increased incidence of malignancy among patients with IgG4-RD has been reported. Thus, differentiating malignancy from IgG4-RD manifestation is important as the treatment differs. Glucocorticoids are considered first-line therapy and should be started early to prevent fibrosis. Patients usually have an excellent clinical response to steroids, and poor steroid response is indicative of an alternative diagnoses such as malignancy. This case report describes a case of IgG4-RD with renal mass in a young man that resolved with glucocorticoid therapy alone.

Riedel’s thyroiditis with increased IgG4 plasma cells: evidence for an underlying IgG4 related sclerosing disease?

Thyroid ◽  
2012 ◽  
pp. 120521080945005 ◽  
Author(s):  
Marc Pusztaszeri ◽  
Frederic Triponez ◽  
Jean-Claude Pache ◽  
Massimo Bongiovanni
Keyword(s):  
Plasma Cells ◽  
Riedel’S Thyroiditis ◽  
Riedel's Thyroiditis

IgG4-related Disease in Thymus. A Very Rare Case of Chronic Fibrosis Mimicking Sarcoidosis

2017 ◽  
Vol 103 (1_suppl) ◽  
pp. S19-S24 ◽  
Author(s):  
Sara Simonetti ◽  
Noelia Pérez Muñoz ◽  
Josefa López Vivancos ◽  
Lluís Sanchez Sitjes ◽  
Javier Cesar Herranz Pérez ◽  
...  
Keyword(s):  
Plasma Cells ◽  
English Literature ◽  
Histopathological Analysis ◽  
Obliterative Phlebitis ◽  
Thymic Tissue ◽  
Positive Ratio ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Storiform Fibrosis ◽  
Serological Data

IgG4-related disease (IgG4-RD) is a multi-organ immune-mediated chronic fibroinflammatory condition, with unclear certain etiology. It is morphologically characterized by storiform fibrosis, dense IgG4-positive lympho-plasmacytic infiltrate, and obliterative phlebitis. It was recognized as a systemic condition as recently as 2003. IgG4-RD has been described in virtually every organ, forming sclerosing masses, and often mimicking tumors. Clinically, patients present unspecific symptoms and this condition is often recognized incidentally. The epidemiology remains poorly studied, but it has been noted that in the majority of recorded instances, patients are middle-aged men. IgG4-RD could mimic conditions other than tumors, such as infection, inflammation, or other systemic disorders. To ensure accuracy of diagnosis, an exhaustive histopathological analysis is required, together with clinical, radiological, and serological data. Thymic fibrosis in the absence of other primary thymic lesions is a very rare occurrence; in English literature only 1 case has been reported with scattered IgG4 plasma cells infiltrate and focal obliterative phlebitis. We will describe, for the first time, the case of a 49-year-old man displaying an anterior mediastinic, hilar, and intramyocardial mass simulating a sarcoidosis, with a definitive diagnosis of IgG4-related thymic fibrosis extending to the mediastinum and the heart. At the histological examination, we found many features of IgG4-RD in the thymic tissue, such as diffused storiform fibrosis, dense lymphoplasmacytic infiltrate with abundant plasma cells IgG4 positive (ratio IgG/IgG4: 40%), obliterative phlebitis, eosinophilic infiltrate, and Castleman-like lymphoid follicles. We discussed the differential diagnosis and reviewed the literature and the other cases of IgG4-related diseases that had been diagnosed in our department.

Sign in / Sign up

Export Citation Format

Share Document