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.

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 Disease of The Larynx Mimicking Malignancy: A Case Report and Review of the Literature

2021 ◽  
Author(s):  
Sevda Akyol ◽  
Ozlem Saraydaroglu ◽  
Omer Afsin Ozmen
Keyword(s):  
Plasma Cells ◽  
Clinical Laboratory ◽  
Review Of The Literature ◽  
Immunoglobulin G4 ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Serum Igg4 ◽  
Storiform Fibrosis ◽  
Lymphoplasmacytic Infiltration ◽  
Igg4 Level

Abstract Objectives: Immunoglobulin G4–related disease is characterized by increased serum IgG4 level, enlargement in the relevant organs and histopathologically intense storiform fibrosis, lymphoplasmacytic infiltration rich in IgG4 positive plasma cells, and obliterative phlebitis.Methods and Results: In this report, a patient who underwent a laryngeal biopsy with a pre-diagnosis of malignancy, but had findings consistent with immunoglobulin G4–related disease in the biopsy sample, is described.Conclusion: Immunoglobulin G4–related disease can be seen in very rare localizations. It should be kept in mind in differential diagnosis when tissues especially containing inflammation rich in plasma cells are encountered. Clinical, laboratory and pathological correlation is extremely important in the diagnosis of an IgG4-related disease.

scholarly journals Diagnosis and management of IgG4-related disease

2018 ◽  
Vol 10 (3) ◽  
pp. 275-283 ◽  
Author(s):  
Vinod S Hegade ◽  
Maria B Sheridan ◽  
Matthew T Huggett
Keyword(s):  
Sclerosing Cholangitis ◽  
Current Knowledge ◽  
Evidence Base ◽  
Obliterative Phlebitis ◽  
Related Disease ◽  
Multiple Organs ◽  
Igg4 Related Disease ◽  
Storiform Fibrosis ◽  
Type 1 Autoimmune Pancreatitis ◽  
Lymphoplasmacytic Infiltration

IgG subclass 4-related disease (IgG4-RD) is a rare but increasingly recognised fibroinflammatory condition known to affect multiple organs. IgG4-RD is characterised by unique histological features of lymphoplasmacytic infiltration, storiform fibrosis and obliterative phlebitis. In this review we describe the pancreaticobiliary manifestations of IgG4-RD, with particular emphasis on type 1 autoimmune pancreatitis (AIP) and IgG4-related sclerosing cholangitis (IgG4-SC). AIP and IgG4-SC can pose diagnostic challenges to the clinician as they may mimic pancreatic cancer and primary sclerosing cholangitis, respectively. We discuss current knowledge, clinical diagnostic criteria and recent advances and summarise the evidence base for current therapeutic approaches for AIP and IgG4-SC.

scholarly journals Histopathology of salivary glands

Reumatismo ◽  
2018 ◽  
pp. 146-154 ◽  
Author(s):  
F. Carubbi ◽  
A. Alunno ◽  
R. Gerli ◽  
R. Giacomelli
Keyword(s):  
Subcutaneous Fat ◽  
Rectal Mucosa ◽  
Obliterative Phlebitis ◽  
Related Disease ◽  
The Past ◽  
Igg4 Related Disease ◽  
Rare Manifestation ◽  
Storiform Fibrosis ◽  
Consensus Guidance ◽  
Lymphoplasmacytic Infiltration

Salivary gland (SG) biopsy is a technique broadly applied for the diagnosis of primary Sjögren’s syndrome (pSS), lymphoma accompanying SS, sarcoidosis, amyloidosis, and IgG4-related disease The most peculiar feature of pSS on biopsy is focal lymphocytic sialadenitis. In the past, several histological scores have been reported in the literature to describe glandular involvement during pSS. However, the variability among centres in reporting glandular scores is one of the rationales behind the development of standardised consensus guidance. SGs as well as lacrimal glands are involved in up to 50% of patients with IgG4-related disease with 3 histopathological hallmarks such as dense lymphoplasmacytic infiltration, storiform fibrosis and obliterative phlebitis. SGs can be also affected by amyloidosis with MSG biopsy being more sensitive than that of rectal mucosa or subcutaneous fat. SG involvement is a rare manifestation during sarcoidosis, and the presence of non-caseating granulomas needs to be differentiated from granulomas of other etiology. This review article provides an overview of normal and pathological SGs in the context of rheumatic diseases, identifying key elements in the tissue as diagnostic and prognostic biomarkers, useful in the current clinical practice.

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.

scholarly journals P117 Rituximab for IgG4-related disease: the Newcastle experience

Rheumatology ◽  
2020 ◽  
Vol 59 (Supplement_2) ◽  
Author(s):  
Fiona Rayner ◽  
Bridget Griffiths ◽  
Manu Nayar ◽  
Kofi Oppong ◽  
Josephine Vila
Keyword(s):  
Head And Neck ◽  
High Dose ◽  
Obliterative Phlebitis ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Neck Disease ◽  
Abdominal Disease ◽  
Storiform Fibrosis ◽  
Life Threatening ◽  
Lymphoplasmacytic Infiltration

Abstract Background IgG4-related disease (IgG4-RD) is a multisystem immune mediated fibro-inflammatory condition characterised by the three histopathologic features of IgG4-RD lymphoplasmacytic infiltration, storiform fibrosis and obliterative phlebitis. The condition can be indolent with few symptoms or present with organ or life-threatening disease. First-line treatment with high dose corticosteroids is often effective, however when tapering steroids, the disease can relapse, and second line agents such as methotrexate (MTX), azathioprine (AZA) or mycophenolate (MMF) are not always effective or tolerated. In 2016, following evidence from observational studies, NHS England approved the use of rituximab (RTX) in refractory IgG4-RD according to strict criteria. In our unit we have used rituximab, with or without cyclophosphamide induction, in eight patients with IgG4-RD. Our aim was to assess effectiveness of rituximab treatment and adherence to NHS England guidelines. Methods Using our connective tissue disease database, patients with IgG4-RD were identified and their electronic notes were reviewed. Outcome after rituximab treatment was assessed by the evaluation of clinical and radiological responses. Results Between August 2017 and September 2019, 15 patients with IgG4-RD were seen in the rheumatology service. 8 patients went on to receive rituximab therapy, 4 with IV cyclophosphamide (CYP). 5/8 patients had head and neck disease and 3/8 had abdominal disease (pancreas, retroperitoneal, renal). By comparison, those patients that did not receive rituximab had a preponderance of abdominal disease (4/7 had abdominal disease, 2/7 head and neck, 1/7 breast). 8/8 patients receiving rituximab were discussed in a designated MDT and met NHS diagnostic guidelines. MDT treatment decisions were made based on ‘refractory’ or ‘organ critical’ disease criteria. In all patients, with available post treatment imaging, radiological response was demonstrated. In one case, imaging showed improvement in some areas and progression in other areas and response was described as ‘partial’. Conclusion In the patients treated to date in our unit, rituximab has been shown to be an effective treatment for IgG4-RD. Cyclophosphamide induction has been used in a subset of patients. Patients with head and neck disease were more likely to require escalation to rituximab therapy, compared with those with abdominal disease. Disclosures F. Rayner None. B. Griffiths None. M. Nayar None. K. Oppong None. J. Vila None.

IgG4-related disease: a clinical perspective

Rheumatology ◽  
2020 ◽  
Vol 59 (Supplement_3) ◽  
pp. iii123-iii131 ◽  
Author(s):  
Federica Maritati ◽  
Francesco Peyronel ◽  
Augusto Vaglio
Keyword(s):  
Plasma Cells ◽  
Organ Damage ◽  
Inflammatory Disorder ◽  
Obliterative Phlebitis ◽  
Steroid Resistant ◽  
Related Disease ◽  
Resistant Disease ◽  
Igg4 Related Disease ◽  
Lymphoplasmacytic Infiltrate ◽  
Storiform Fibrosis

Abstract IgG4-related disease (IgG4-RD) is a recently recognized fibro-inflammatory disorder that can affect almost any organ. Common presentations include major salivary and lacrimal gland enlargement, orbital disease, autoimmune pancreatitis, retroperitoneal fibrosis and tubulointerstitial nephritis. The main histopathological features are a dense, polyclonal, lymphoplasmacytic infiltrate rich in IgG4+ plasma cells, storiform fibrosis and obliterative phlebitis. The precise pathogenic mechanisms of IgG4-RD are still unclear. CD4+ T and B cells, including IgG4-expressing plasmablasts, constitute the major inflammatory cell populations and are believed to cause organ damage and tissue fibrosis. The diagnosis of the disease may be challenging and should be based on specific histopathological findings, typical laboratory and radiological aspects and an appropriate clinical context. The first-line treatment of IgG4-RD is based on glucocorticoids, which are usually efficacious. However, B cell depletion induced by rituximab has also been found to induce remission in steroid-resistant disease or has been used as steroid-sparing agent for relapsing disease. This review provides an update on clinical and therapeutic aspects of IgG4-RD.

Thoracic Involvement in IgG4-Related Disease

2020 ◽  
Vol 41 (02) ◽  
pp. 202-213 ◽  
Author(s):  
Marta Casal Moura ◽  
Ria Gripaldo ◽  
Misbah Baqir ◽  
Jay H. Ryu
Keyword(s):  
Clinical Laboratory ◽  
Wide Spectrum ◽  
The Body ◽  
Obliterative Phlebitis ◽  
Immunoglobulin G4 ◽  
Related Disease ◽  
First Line Therapy ◽  
Igg4 Related Disease ◽  
Serum Igg4 ◽  
Histopathologic Features

AbstractImmunoglobulin G4-related disease (IgG4-RD) is a systemic fibroinflammatory disorder that has been recognized to involve virtually any organ in the body and typically manifests mass-like lesions (tumefactive). Although initial reports of this disease (autoimmune pancreatitis [AIP]) were described in the Japanese population, it has since been reported worldwide. It is most commonly seen in adults of middle age or older, more often men than women. The pathogenesis of IgG4-RD is largely unknown, but genetic factors, microorganisms, and autoimmunity are thought to play important roles. Serum IgG4 concentration is elevated in the majority of patients with IgG4-RD but is a nonspecific finding. Characteristic histopathologic features include dense lymphoplasmacytic infiltrate, fibrosis (often in storiform pattern), and obliterative phlebitis. Lung involvement in IgG4-RD was first reported in 2004 in two patients with AIP and coexisting interstitial lung disease. Since then, a wide spectrum of intrathoracic involvement has been reported and includes not only parenchymal lung diseases but also pleural, airway, vascular, and mediastinal lesions. Thoracic involvement in IgG4-RD is often found incidentally during the workup of extrathoracic lesions but can sometimes be the presenting abnormality. The diagnosis of IgG4-RD requires correlation of clinical, laboratory, imaging, and histopathologic features. Glucocorticoids are the first-line therapy but other options including B cell depletion are being investigated. IgG4-RD is generally associated with an indolent clinical course and most patients improve with glucocorticoid therapy.

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 Intrathoracic Manifestations of IgG4-Related Disease

2016 ◽  
Vol 4 (16) ◽  
pp. 33
Author(s):  
Sian Yik Lim ◽  
Deepa Panikkath
Keyword(s):  
Lung Parenchyma ◽  
Accurate Diagnosis ◽  
Clinical Context ◽  
Obliterative Phlebitis ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Lymphoplasmacytic Infiltrate ◽  
Pathologic Findings ◽  
Storiform Fibrosis ◽  
Glass Type

Intrathoracic involvement with IgG4-related disease (IgG4-RD) is frequently overlooked in IgG4-related disease patients. In this article we review the intrathoracic findings of IgG4-RD which are variable and protean. IgG4-related disease has been reported to affect the lung parenchyma, pleura, mediastinal/hilar lymph nodes, vasculature, and pericardium within the thorax. Mediastinal and hilar lymphadenopathy is the most common intrathoracic manifestation of IgG4-RD. Four main patterns of pulmonary disease have been described, including the solid nodular type, the bronchovascular type, the alveolar interstitial type, and the round shaped ground glass type. When feasible, a biopsy should be obtained to confirm the diagnosis. Most lesions show characteristic pathologic findings of IgG4-RD: dense lymphoplasmacytic infiltrate, storiform fibrosis, and obliterative phlebitis. While this helps establish the diagnosis, the interpretation of pathology findings in the clinical context is key in making an accurate diagnosis. Mimickers of IgG4-RD should be ruled out, before making a diagnosis. The intrathoracic findings of IgG4-RD can be treated effectively with prednisone, but may require additional immunosuppressive therapies, including rituximab.

Sign in / Sign up

Export Citation Format

Share Document