Immunoglobulin G4-related disease: case report and literature review

Dong, Liu; Sheikh, Irfan; Wu, Xiaohong; Chen, Enguo; Ying, Kejing

doi:10.1007/s12026-021-09215-2

Cited by 4 publications

(8 citation statements)

References 38 publications

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“…2 However, its chronicity, radiologic progression, high recurrence rates, and multiorgan fibroinflammatory involvement is well documented in previous literature. [3][4][5] Furthermore, despite the effectiveness of glucocorticoids, about one-third of patients experience disease relapse, which may occur in the same organ being treated or in formerly uninvolved organ systems. 10 Previous studies have suggested that elevated Th1 levels, changes of regulatory CD4 + CD25 + T cells that primarily secrete the anti-inflammatory factor IL-10 and the fibrogenic factor TGF-ß and/or IgG4 production promoted by regulatory T-cell-mediated immune response might participate in the progression of IgG4-RD.…”

Section: Discussionmentioning

confidence: 99%

“…1,2 It is an immunemediated and multisystemic inflammatory disease that can affect virtually any organ or tissue. 2,3 A Japanese study estimated an incidence of 0.28-1.08 per 100,000 population, affecting mostly males over 50 years of age with a male/female ratio of 3:1. 3 The disease may mimic other prevalent pathologies due to the subacute development of a mass or organomegaly, leading to possible delayed diagnosis and unnecessary medical or surgical treatment.…”

Section: Introductionmentioning

confidence: 99%

“…2,3 A Japanese study estimated an incidence of 0.28-1.08 per 100,000 population, affecting mostly males over 50 years of age with a male/female ratio of 3:1. 3 The disease may mimic other prevalent pathologies due to the subacute development of a mass or organomegaly, leading to possible delayed diagnosis and unnecessary medical or surgical treatment. 4,5 Although almost any organ can be affected, common sites include the pancreato-biliary system, kidneys, retroperitoneum/ aorta, thorax, and salivary/lacrimal glands.…”

Section: Introductionmentioning

confidence: 99%

“…IgG4‐related disease (IgG4‐RD) is a rare, chronic and progressive clinical entity characterized by tissue infiltration through IgG4+ plasma cells that lead to eosinophilic inflammation and consequent storiform fibrosis, with the appearance of pseudotumor lesions 1,2 . It is an immune‐mediated and multisystemic inflammatory disease that can affect virtually any organ or tissue 2,3 . A Japanese study estimated an incidence of 0.28–1.08 per 100,000 population, affecting mostly males over 50 years of age with a male/female ratio of 3:1 3 …”

Section: Introductionmentioning

confidence: 99%

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IgG4‐related disease: Case report and 6‐year follow‐up of an elusive diagnosis mimicking malignancy

Oliveira,

Gomes,

Trigo

et al. 2024

Clinical Case Reports

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Key Clinical MessageIgG4‐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.AbstractIgG4‐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.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

IgG4‐related disease: Case report and 6‐year follow‐up of an elusive diagnosis mimicking malignancy

Oliveira,

Gomes,

Trigo

et al. 2024

Clinical Case Reports

View full text Add to dashboard Cite

show abstract

“…Third, not all patients had documented CSF levels of ANCA and IgG4. Those were increased in some reports of HP related to IgG4-RD ( 30 ) and AAV ( 31 ), making them valuable biomarkers for differentiating between IgG4-HP and ANCA-related HP.…”

Section: Discussionmentioning

confidence: 99%