60 results match your criteria igg4-related fibrosing

[Immunoglobulin G4( IgG4)-related Fibrosing Mediastinitis Localized in the Retrosternal Area:Report of a Case].

Kyobu Geka 2021 Apr;74(4):317-320

Department of Thoracic Surgery, Kanazawa Medical Center, Kanazawa, Japan.

An 84-year-old man was referred to our out-patient clinic with an elongated mass localized to the retrosternal area that was incidentally identified by computed tomography. On 18F-fluorodeoxyglucose-positron emission tomography, this lesion showed intense tracer uptake. Thus, a surgical biopsy under thoracoscopy was performed. Read More

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IgG4-related disease of the thyroid gland.

BMJ Case Rep 2021 Mar 1;14(3). Epub 2021 Mar 1.

Endocrinology, Diabetes, and Metabolism Department, Centro Hospitalar Universitário Lisboa Norte EPE, Lisboa, Portugal.

IgG4-thyroid-related disease (TRD) represents an uncommon spectrum of diseases, with four subcategories established so far, IgG4-related Hashimoto's thyroiditis, fibrosing variant of Hashimoto's thyroiditis, Riedel's thyroiditis and Graves disease with elevated IgG4 levels. We report the case of a 59-year-old woman presenting with painless cervical swelling and hypothyroidism. Thyroid gland was enlarged and distinctively very hard, with reduced mobility. Read More

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CD4CTLs in Fibrosing Mediastinitis Linked to .

J Immunol 2021 02 16;206(3):524-530. Epub 2020 Dec 16.

Ragon Institute of MGH, MIT and Harvard, Cambridge, MA 02139;

Although fibrotic disorders are frequently assumed to be linked to T cells, quantitative tissue interrogation studies have rarely been performed to establish this link and certainly many fibrotic diseases do not fall within the type 2/allergic disease spectrum. We have previously linked two human autoimmune fibrotic diseases, IgG4-related disease and systemic sclerosis, to the clonal expansion and lesional accumulation of CD4CTLs. In both these diseases T cell accumulation was found to be sparse. Read More

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February 2021

IgG4 immunohistochemistry in Riedel's thyroiditis and the recommended criteria for diagnosis: A case series and literature review.

Clin Endocrinol (Oxf) 2021 May 21;94(5):851-857. Epub 2020 Dec 21.

Department of Endocrinology, Peking University First Hospital, Beijing, China.

Objective: Riedel's thyroiditis (RT) is a very rare chronic fibrosing thyroiditis that is often associated with multifocal fibrosclerosis. Although the relationship of RT and IgG4-related disease (IgG4-RD) has been suggested, the expression of IgG and IgG4 in thyroid tissues of patients with RT has seldom been studied.

Design: A case series and literature review. Read More

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Imaging of IgG4-related fibrosing mediastinitis.

Radiologia (Engl Ed) 2020 Sep 21. Epub 2020 Sep 21.

Servicio de Radiodiagnóstico, Hospital Universitario Morales Meseguer, Murcia, España.

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September 2020

IgG4-related fibrosing mediastinitis: clinical presentation, treatment efficacy and comparison with IgG4-RD without fibrosing mediastinitis.

Clin Exp Rheumatol 2020 Nov-Dec;38(6):1206-1214. Epub 2020 Sep 4.

Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education & National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Beijing, China.

Objectives: This study aimed to investigate the clinical characteristics and treatment efficacy of immunoglobulin G4 (IgG4)-related fibrosing mediastinitis (IgG4-RFM) and to compare IgG4-RFM patients with IgG4-related disease (IgG4-RD) patients without fibrosing mediastinitis (FM).

Methods: Twenty IgG4-RFM patients and 60 randomly matched IgG4-RD patients without FM from a prospective cohort at the Peking Union Medical College Hospital (PUMCH) were enrolled from 2011 to 2019. Patient demographic data, clinical characteristics, laboratory parameters and treatment efficacy were analysed. Read More

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December 2020

IgG4-Related Disease Manifesting as Hypocomplementemic Tubulointerstitial Nephritis: A Rare Case Report and Literature Review.

J Investig Med High Impact Case Rep 2020 Jan-Dec;8:2324709620952213

James H. Quillen Veterans Affairs Medical Center, Johnson City, TN, USA.

Immunoglobulin G4-related disease (IgG4-RD) is a chronic fibrosing inflammatory systemic disorder that has been recognized relatively recently in the medical literature. Little is known about the exact disease pathogenesis and epidemiology. IgG4-RD may be asymptomatic or may have minimal symptoms or involve multiple organs with overt symptoms. Read More

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A case of IgG4-related anterior mediastinal sclerosing disease coexisting with autoimmune pancreatitis.

Surg Case Rep 2020 Jul 23;6(1):180. Epub 2020 Jul 23.

Department of Thoracic Surgery, Kansai Medical University Hospital, Osaka, Japan.

Background: IgG4-related disease (IgG4-RD) is a systemic fibro-inflammatory condition that predominantly involves exocrine organs. Concerning its thoracic presentation, it often manifests as interstitial lung disease or fibrosing mediastinitis. It is very rare for IgG4-RD to form a well-defined mass in the anterior mediastinum, mimicking an encapsulated thymoma. Read More

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IgG4 sclerosing disease of the esophagus: a case-based review.

Rheumatol Int 2020 10 2;40(10):1733-1737. Epub 2020 May 2.

Internal Medicine Residency, Hennepin Healthcare, Minneapolis, Minnesota, USA.

IgG4-related disease (IgG4-RD) is an inflammatory and fibrosing disease which causes tumor-like swelling of organs and commonly mimics symptoms of malignancy. It has been increasing in prevalence in the last decade, but esophageal involvement remains rare. IgG4-RD was first known to involve certain organs, such as the pancreas. Read More

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October 2020

Does a Subset of Localized Chronic Fibrosing Vasculitis Represent Cutaneous Manifestation of IgG4-Related Disease/a Histologic Pattern of IgG4-Related Skin Disease? A Reappraisal of an Enigmatic Pathologic Entity.

Tien Anh Tran

Am J Dermatopathol 2020 Sep;42(9):683-688

Department of Pathology, Advent Health Orlando, Orlando, FL.

Localized chronic fibrosing vasculitis (LCFV) is a rare cutaneous fibroinflammatory and vasculitic process of poorly defined etiology. Furthermore, controversy remains as to whether LCFV represents a primary pathologic process or a histologic pattern. The current case documents a 52-year-old male patient with a scrotal mass and clinical history of a retroperitoneal mass as well as a previously resected tumor of the right submandibular salivary gland displaying morphologic features of eosinophilic angiocentric fibrosis. Read More

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September 2020

Interstitial pneumonia with autoimmune features that met the proposed diagnostic criteria for IgG4-related respiratory disease.

Respirol Case Rep 2020 Mar 17;8(2):e00512. Epub 2019 Dec 17.

Department of Chest Surgery International University of Health and Welfare Hospital Nasushiobara Japan.

We held a multidisciplinary discussion (MDD) about a 61-year-old woman who had an interstitial lung disease (ILD) without extrathoracic lesions that met the classification criteria for interstitial pneumonia with autoimmune features (IPAF) and the proposed diagnostic criteria for immunoglobulin G4 (IgG4)-related respiratory disease (IgG4-RRD). Clinically, the marked progression of lung-limited diffuse lesions was consistent with IPAF. Serum IgG4 and rheumatoid factor levels simultaneously increased and did not contribute to a diagnosis. Read More

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[IgG4-related disease : Microscopic diagnosis and differential diagnosis].

S Detlefsen

Pathologe 2019 Nov;40(6):619-626

Abteilung für Pathologie, Odense Universitätshospital, J. B. Winsløws Vej 15, 5000, Odense, Dänemark.

IgG4-related disease (IgG4-RD) is a potentially systemic inflammatory and fibrosing disease. Independent of the affected organ, the main histological features are a dense lymphoplasmacytic infiltrate, storiform fibrosis, and obliterative phlebitis. These features are weighted slightly different, dependent on the respective organ. Read More

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November 2019

Tracheal Stenosis Due to IgG4-Related Fibrosing Mediastinitis.

Arch Bronconeumol (Engl Ed) 2019 Oct 20;55(10):533. Epub 2019 Feb 20.

Servicio de Neumología, Hospital Universitario Puerta del Mar, Cádiz, España.

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October 2019

Chronic Periaortitis: an Update.

Curr Rheumatol Rep 2018 11 5;20(12):80. Epub 2018 Nov 5.

Nephrology Unit, Parma University Hospital, Parma, Italy.

Purpose Of Review: We aim to review traditional concepts and recent developments on the nosology, pathophysiology, clinical phenotypes and treatment of chronic periaortitis (CP).

Recent Findings: CP is a rare disorder hallmarked by a periaortic fibro-inflammatory tissue. It can present as an isolated disease, but it can also be associated with other autoimmune and fibro-inflammatory lesions (e. Read More

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November 2018

IgG4-Related Disease of the Thyroid Gland Requiring Emergent Total Thyroidectomy: A Case Report.

Head Neck Pathol 2019 Sep 31;13(3):523-527. Epub 2018 May 31.

Division of Pathology, Department of Anatomical Pathology, Singapore General Hospital, Singapore, Singapore.

IgG4-related disease of the thyroid gland is a recently recognized subtype of thyroiditis, often with rapid progression requiring surgical treatment. It is considered as a spectrum of disease varying from early IgG4-related Hashimoto's thyroiditis (HT) pattern to late fibrosing HT or Riedel's thyroiditis patterns. Here, we report a 47-year-old Malay woman presenting with progressively painless neck swelling over 3 years and subclinical hypothyroidism. Read More

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September 2019

IgG4-related fibrosing mediastinitis diagnosed with computed tomography-guided percutaneous needle biopsy: Two case reports and a review of the literature.

Medicine (Baltimore) 2018 Jun;97(22):e10935

Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, Tokyo, Japan.

Rationale: Immunoglobulin G4-related disease (IgG4-RD) is a fibroinflammatory disease characterized by elevated serum IgG4 levels with infiltration of IgG4+ plasma cells and severe fibrosis in affected tissues. Recently, idiopathic fibrosing mediastinitis (FM), an extremely rare fibroinflammatory disorder, has been recognized as a form of IgG4-RD. As IgG4-RD can be treated by glucocorticoids, identification of the etiology of FM by surgical biopsy is essential; however, mediastinal biopsy is often difficult. Read More

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Unrecognised IgG4 association in progressively transformed germinal centers of lymph nodes with subsequent full-blown IgG4-related chronic fibrosing pancreatitis: A case report.

Malays J Pathol 2018 Apr;40(1):73-78

Eulji University, School of Medicine, Department of Pathology, Daejeon, Republic of Korea.

Progressively transformed germinal centers (PTGC) is a benign process characterised by a morphological variant of reactive follicular hyperplasia in lymph nodes. It was recently shown that some cases of PTGC are associated with IgG4-related disease (IgG4-RD) or increased IgG4 plasma cells. Five years ago, a 57-year-old woman presented with enlargement of multiple lymph nodes in the left parotid, submandibular, and neck areas, pathologically diagnosed as PTGC after excisional biopsy. Read More

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Imaging appearance of fibrosing diseases of the retroperitoneum: can a definitive diagnosis be made?

Abdom Radiol (NY) 2018 05;43(5):1204-1214

Department of Nephrology, University of Michigan Hospital, Ann Arbor, MI, USA.

Purpose: To assess the frequency with which previously reported characteristic findings of retroperitoneal fibrosis (RPF) (a circumferential or almost circumferential peri-aortic mass centered at L4, which does not displace the abdominal aorta or proximal common iliac arteries) are present in patients with RPF, in patients with other fibrosing diseases, and in cancer patients referred to a subspecialty clinic with a suspected diagnosis of RPF, in order to determine whether diagnostic percutaneous biopsy can be avoided in some patients.

Methods: This HIPAA-compliant Institutional Review Board-approved retrospective study assessed clinical and CT and MR imaging abnormalities on imaging studies in 92 patients referred to a subspecialty clinic with suspected RPF over a 14-year period. Two reviewers, in consensus, determined the frequency of different CT and MRI findings in three groups of patients (Group 1: those with an eventual diagnosis of RPF, Group 2: those with a fibrosing disease associated with vascular or urologic abnormalities, and Group 3: those with cancer). Read More

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Unexpected Fibrosing Mediastinitis Shown on FDG PET/CT in a Patient With IgG4-Related Disease.

Clin Nucl Med 2017 Oct;42(10):818-819

From the Department of Nuclear Medicine, Beijing Friendship Hospital of Capital Medical University, Beijing, China.

A 66-year-old man presented to our hospital because of abdominal pain for 5 days. A contrast abdominal CT raised the possibility of pancreatic carcinoma. FDG PET/CT showed increased FDG accumulation not only in the pancreas and the retroperitoneum, but also in the posterior mediastinum, which was not typical of pancreatic carcinoma. Read More

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October 2017


O Fain A Mekinian

Rev Med Interne 2017 Sep 14;38(9):585-591. Epub 2017 Apr 14.

Service de médecine interne, hôpital Saint-Antoine, AP-HP, DHUi2B, université Paris 6, 184, rue du Faubourg-Saint-Antoine, 75012 Paris, France.

Pachymeningitis is a fibrosing and inflammatory process, which involves the dura mater. Some pachymeningitis are cranial and induce headaches and cranial nerve palsies. Others are spinal and responsible for nerve roots or spinal cord compression. Read More

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September 2017

Immunoglobulin G4-Related Thyroid Diseases.

Eur Thyroid J 2016 Dec 3;5(4):231-239. Epub 2016 Dec 3.

Department of Endocrinology and Diabetes, The Alfred Hospital, Monash University, Melbourne, VIC, Australia; Department of Medicine, Monash University, Melbourne, VIC, Australia.

Immunoglobulin G4-related disease (IgG4-RD) is a new disease category involving many organ systems, including the endocrine system in general and the thyroid in particular. Since an initial association was made between hypothyroidism and autoimmune (IgG4-related) pancreatitis, more forms of IgG4-related thyroid disease (IgG4-RTD) have been recognized. Four subcategories of IgG4-RTD have so far been identified: Riedel thyroiditis (RT), fibrosing variant of Hashimoto thyroiditis (FVHT), IgG4-related Hashimoto thyroiditis, and Graves disease with elevated IgG4 levels. Read More

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December 2016

Clinical Update in Aspects of the Management of Autoimmune Thyroid Diseases.

Duncan J Topliss

Endocrinol Metab (Seoul) 2016 Dec;31(4):493-499

Department of Endocrinology and Diabetes, The Alfred, Melbourne, Australia.

Aspects of autoimmune thyroid disease updated in this review include: immunoglobulin G4 (IgG4)-related thyroid disease (Riedel's thyroiditis, fibrosing variant of Hashimoto's thyroiditis, IgG4-related Hashimoto's thyroiditis, and Graves' disease with elevated IgG4 levels); recent epidemiological studies from China and Denmark indicating that excess iodine increases the incidence of Hashimoto's thyroiditis and hypothyroidism; immunomodulatory agents (ipilimumab, pembrolizumab, nivolumab) activate immune response by inhibiting T-cell surface receptors which down-regulate immune response, i.e., cytotoxic T-lymphocyte antigen 4 and programmed cell death protein 1 pathways; alemtuzumab is a humanised monoclonal antibody to CD52 which causes immune depletion and thyroid autoimmune disease especially Graves' hyperthyroidism; small molecule ligand (SML) agonists which activate receptors, SML neutral antagonists, which inhibit receptor activation by agonists, and SML inverse agonists which inhibit receptor activation by agonists and inhibit constitutive agonist independent signaling have been identified. Read More

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December 2016

IgG4-related disease: Evidence from six recent cohorts.

Autoimmun Rev 2017 Feb 15;16(2):168-172. Epub 2016 Dec 15.

Systemic Autoimmune Diseases Unit, Internal Medicine Department, Hospital Universitari Vall d'Hebron, Vall d'Hebron Institute of Research, Universitat Autònoma de Barcelona, Barcelona, Spain.

IgG4-related disease (IgG4-RD) is a rare autoimmune fibrosing disorder. In this review we aim to describe and compare the characteristics of the six largest IgG4-RD cohorts, since the new 2012 consensus diagnostic criteria were released. These observational studies were published between 2012 and 2015. Read More

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February 2017

Immunoglobulin G4 related thyroid disorders: Diagnostic challenges and clinical outcomes.

Endokrynol Pol 2016 ;67(5):520-524

Department of Endocrinology, Postgraduate Institute of Medical Education & Research (PGIMER) & Dr. Ram Manohar Lohia (RML) Hospital, New Delhi, India.

Ig-G4 related disease (IgG4RD) is a heterogeneous disorder with multi-organ involvement recognised as a separate entity at the start of this century only. It is currently one of the hottest areas of clinical and translational research across specialties. Thyroid involvement in IgG4RD is rare, believed to occur in less than 4% of cases, may be isolated or may be associated with other organ involvement. Read More

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IgG4-related sclerosing mesenteritis causing bowel obstruction: a case report.

Surg Case Rep 2016 Dec 30;2(1):120. Epub 2016 Oct 30.

Department of Surgery and Oncology, Graduate School of Medical Sciences, Kyushu University, Maidashi 3-1-1, Higashi-ku, Fukuoka, 812-8582, Japan.

Sclerosing mesenteritis (SM) is a rare inflammatory and fibrosing disease primarily involving the small-bowel mesentery. Recently, SM was reported to be closely related to IgG4-related disease (IgG4-RD). This report describes a patient with SM associated with IgG4-RD. Read More

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December 2016

Idiopathic Mediastinal Fibrosis: a Systemic Immune-Mediated Disorder. A Case Series and a Review of the Literature.

Clin Rev Allergy Immunol 2017 Jun;52(3):446-459

Nephrology Unit, Parma University Hospital, Via Gramsci 14, 43126, Parma, Italy.

Mediastinal fibrosis is a rare disease characterised by fibrous proliferation in the mediastinum. It can be idiopathic or secondary to several conditions such as infections and malignancies. Anecdotal reports have described idiopathic mediastinal fibrosis (IMF) in association with other fibro-inflammatory or autoimmune diseases. Read More

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[Histopathology of IgG4-related disease].

Z Rheumatol 2016 Sep;75(7):666-74

Institut für Pathologie, Konsultationszentrum für Pankreas und Endokrine Tumore, Technische Universität München, Ismaningerstr. 22, 81675, München, Deutschland.

At an international consensus conference in 2011, multifocal chronic fibrosing inflammatory processes, which are associated with elevated IgG4 serum levels and/or tissue infiltration with IgG4 positive plasma cells, were recognized as a distinct disease entity called IgG4-related disease (IgG4-RD). As IgG4-RD responds well to steroid treatment but imitates a tumor in many organs, particularly in the pancreas, a biopsy for confirmation of the diagnosis is often warranted. The histological criteria for IgG4-RD as defined in 2011 are based on the following main features: 1) dense lymphoplasmacytic infiltrate, 2) storiform fibrosis and 3) obliterative phlebitis. Read More

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September 2016

A small subgroup of Hashimoto's thyroiditis is associated with IgG4-related disease.

Virchows Arch 2016 Mar 15;468(3):321-7. Epub 2015 Dec 15.

Institute of Pathology, Staedtisches Klinikum Munich, Sanatoriumsplatz 2, 81545, Munich, Germany.

IgG4-related disease is a newly identified syndrome characterized by high serum IgG4 levels and increased IgG4-positive plasma cells in involved organs. The incidence of IgG4-related thyroiditis in the Caucasian population of Europe is unknown. We investigated formalin-fixed thyroid gland samples of 216 patients (191 Hashimoto's thyroiditis, 5 Riedel's thyroiditis, and 20 goiters, as controls), morphologically, and immunohistochemically. Read More

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Idiopathic fibrosing mediastinitis: spectrum of imaging findings with emphasis on its association with IgG4-related disease.

Clin Imaging 2015 Nov-Dec;39(6):993-9. Epub 2015 Jul 16.

Department of Respiratory Medicine, Hospital Universitario Ramón y Cajal, 28034 Madrid, Spain.

Purpose: The purpose of this study was to review the clinicoradiological features of idiopathic fibrosing mediastinitis (FM).

Methods: The records of six patients with idiopathic FM were retrospectively reviewed.

Results: The most common presenting symptom was cough. Read More

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Idiopathic retroperitoneal fibrosis: clinicopathologic features and outcome analysis.

Clin Rheumatol 2016 Feb 25;35(2):401-7. Epub 2015 Jul 25.

Department of Radiology, Marshfield Clinic, Marshfield, WI, USA.

To describe clinical features and outcomes of 26 patients with idiopathic retroperitoneal fibrosis from a single center, we reviewed medical records of consecutive patients with idiopathic retroperitoneal fibrosis evaluated at our facility from January 1, 1998 to December 31, 2013 for clinical features, laboratory and radiographic findings, management, and outcomes. Twenty-six patients met criteria for idiopathic retroperitoneal fibrosis and were included in the study. Median age at diagnosis was 58 years; male-female ratio was 3. Read More

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February 2016