تقرير
IgG4-related disease: Case report and 6-year follow-up of an elusive diagnosis mimicking malignancy.
| العنوان: | IgG4-related disease: Case report and 6-year follow-up of an elusive diagnosis mimicking malignancy. |
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| المؤلفون: | Oliveira SM; Family Health Unit of Barrinha, Esmoriz Local Health Unit of the Aveiro Region Aveiro Portugal., Gomes I; Family Health Unit of Barrinha, Esmoriz Local Health Unit of the Aveiro Region Aveiro Portugal., Trigo I; Family Health Unit of Barrinha, Esmoriz Local Health Unit of the Aveiro Region Aveiro Portugal., Fonseca E; Department of Pathology, Centro Hospitalar Universitário de São João; Instituto de Investigação e Inovação em Saúde (i3S) and Institute of Molecular Pathology and Immunology University of Porto (Ipatimup); Faculty of Medicine of the University of Porto Porto Portugal., Lopes RN; Family Health Unit of Barrinha, Esmoriz Local Health Unit of the Aveiro Region Aveiro Portugal., Oliveira AS; Family Health Unit of Barrinha, Esmoriz Local Health Unit of the Aveiro Region Aveiro Portugal. |
| المصدر: | Clinical case reports [Clin Case Rep] 2024 May 10; Vol. 12 (5), pp. e8894. Date of Electronic Publication: 2024 May 10 (Print Publication: 2024). |
| نوع المنشور: | Case Reports |
| اللغة: | English |
| بيانات الدورية: | Publisher: John Wiley & Sons Country of Publication: England NLM ID: 101620385 Publication Model: eCollection Cited Medium: Print ISSN: 2050-0904 (Print) Linking ISSN: 20500904 NLM ISO Abbreviation: Clin Case Rep Subsets: PubMed not MEDLINE |
| أسماء مطبوعة: | Original Publication: Chichester, UK : John Wiley & Sons |
| مستخلص: | Key Clinical Message: IgG4-related disease is a rare and emerging pathology, characterized by the appearance of pseudotumors. Due to the ability to mimic other pathologies, it is essential to consider it as a differential diagnosis in multisystemic processes. The diagnosis is challenging, requiring a multidisciplinary approach, to minimize the associated morbidity and mortality. Abstract: IgG4-related disease (IgG4-RD) is a rare, emerging, systemic and chronic pathology, characterized by the appearance of pseudotumors resulting from tissue infiltration by IgG4-positive plasma cells that promote eosinophilic inflammation of the tissue with subsequent fibrosis. We present the case of a male, 45-year-old patient, with marked weight loss and skin pallor detected by his family doctor during a child health consultation of his daughter. When questioned, the patient referred complaints of postprandial discomfort in the left hypochondrium with a feeling of fullness, weight loss, chronic fatigue and hyperhidrosis that had lasted for a month. On physical examination, he was pale, and had pain at palpation of the left hypochondrium. Laboratory data showed increased inflammation markers, abdominal ultrasound and CT demonstrated numerous enlarged lymph nodes in the upper quadrants, raising concern for a malignant lymphoproliferative process. Serological, imaging, clinical and laparoscopic excisional biopsy revealed features of IgG4-related disease and excluded malignant lymphoproliferative disease. The immediate response to treatment with oral prednisolone 30 mg/day also contributed for diagnosis confirmation. Due to refractory disease after gradual prednisolone reduction, second-line therapy with rituximab was initiated. Over the 6 years of follow-up, the patient presented multiple exacerbations characterized by the emergence of systemic symptoms, being maintained under close clinical and imaging follow-up by reumathology, infectious diseases, and family medicine specialists. Competing Interests: The authors have no conflicts of interest to declare. (© 2024 The Authors. Clinical Case Reports published by John Wiley & Sons Ltd.) |
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| فهرسة مساهمة: | Keywords: IgG4‐related disease; chronic; immune‐mediated; pseudotumor; systemic |
| تواريخ الأحداث: | Date Created: 20240513 Latest Revision: 20240514 |
| رمز التحديث: | 20240514 |
| مُعرف محوري في PubMed: | PMC11087225 |
| DOI: | 10.1002/ccr3.8894 |
| PMID: | 38736580 |
| قاعدة البيانات: | MEDLINE |
Full Text Finder | تدمد: | 2050-0904 |
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| DOI: | 10.1002/ccr3.8894 |