دورية أكاديمية
أعرض في EDS

Idiopathic hyalinizing fibrosclerosis: A systemic steroid-resistant condition distinct from IgG4-related disease.

التفاصيل البيبلوغرافية
العنوان: Idiopathic hyalinizing fibrosclerosis: A systemic steroid-resistant condition distinct from IgG4-related disease.
المؤلفون: Zen Y; Institute of Liver Studies, King's College Hospital, London, SE5 9RS, UK. Electronic address: yoh.1.zen@kcl.ac.uk., Joshi D; Institute of Liver Studies, King's College Hospital, London, SE5 9RS, UK.
المصدر: Human pathology [Hum Pathol] 2024 Sep; Vol. 151, pp. 105638. Date of Electronic Publication: 2024 Aug 13.
نوع المنشور: Case Reports; Journal Article
اللغة: English
بيانات الدورية: Publisher: W B Saunders Country of Publication: United States NLM ID: 9421547 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1532-8392 (Electronic) Linking ISSN: 00468177 NLM ISO Abbreviation: Hum Pathol Subsets: MEDLINE
أسماء مطبوعة: Publication: Philadelphia, PA : W B Saunders
Original Publication: Philadelphia, W B. Saunders Co.
مواضيع طبية MeSH: Immunoglobulin G4-Related Disease*/pathology , Immunoglobulin G4-Related Disease*/drug therapy , Immunoglobulin G4-Related Disease*/diagnosis , Immunoglobulin G4-Related Disease*/complications , Immunoglobulin G4-Related Disease*/immunology , Fibrosis*/pathology , Immunoglobulin G*/blood, Humans ; Male ; Female ; Middle Aged ; Adult ; Sclerosis/pathology ; Diagnosis, Differential ; Drug Resistance ; Cholangitis, Sclerosing/pathology ; Cholangitis, Sclerosing/immunology ; Cholangitis, Sclerosing/drug therapy ; Cholangitis, Sclerosing/diagnosis ; Biopsy ; Steroids/therapeutic use ; Biomarkers/blood ; Biomarkers/analysis ; Immunohistochemistry
مستخلص: Since the concept of IgG4-related disease (IgG4-RD) was proposed, that diagnosis has been considered in idiopathic fibroinflammatory diseases in various organs, particularly in cases with multi-organ involvement. We have recently encountered three cases of fibrosing disease of uncertain etiology with shared microscopic appearances. Case 1 (56-year-old man) had an irregular mass at the base of mesentery. Case 2 (29-year-old woman) presented with obstructive jaundice due to an ill-defined mass at the hepatic hilum and two lung nodules. Case 3 (53-year-old man) had multiple solid nodules in the mediastinum, peritoneum, retroperitoneum, and mesentery; he also had diffuse irregular narrowing of the intra- and extra-hepatic bile ducts in keeping with sclerosing cholangitis. Serum IgG4 concentrations were not elevated. Biopsies from the nodular lesions showed extensive hyalinizing fibrosis with an only focal lymphoplasmacytic infiltrate. Thick collagenous bundles are arranged in an irregular or partly whorl pattern. Typical storiform fibrosis or obliterative phlebitis was not observed. The number of IgG4-positive plasma cells was <10 cells/high-power field; the ratio of IgG4/IgG-positive plasma cells was <30%. After the histological diagnosis of sclerosing mesenteritis, pulmonary hyalinizing granuloma, and mediastinal fibrosis was made, they were treated with a trial of steroids, but none showed a significant response. In conclusion, a hyalinizing fibrotic condition can occur at various anatomical sites. They have shared microscopic findings, and are steroid-resistant. Although the clinical presentation may mimic IgG4-RD, the two conditions are likely distinct. We would propose a diagnostic term of 'idiopathic hyalinizing fibrosclerosis' for this under-recognized, rare, systemic condition.
Competing Interests: Declaration of competing interest None to declare.
(Copyright © 2024 Elsevier Inc. All rights reserved.)
فهرسة مساهمة: Keywords: Hyalinizing granuloma; Mediastinal fibrosis; Mesenteritis; Multifocal fibrosclerosis; Sclerosing cholangitis
المشرفين على المادة: 0 (Immunoglobulin G)
0 (Steroids)
0 (Biomarkers)
تواريخ الأحداث: Date Created: 20240811 Date Completed: 20240822 Latest Revision: 20240822
رمز التحديث: 20240823
DOI: 10.1016/j.humpath.2024.105638
PMID: 39128556
قاعدة البيانات: MEDLINE
الوصف
تدمد:1532-8392
DOI:10.1016/j.humpath.2024.105638