IgG4-related disease with multiorgan involvement: a case-based review

Tarte, Nikhil N.; Ravipati, Chandana Shilpa; Rocha, José Aurillo; Rinker, Elizabeth; Patel, Nirupa J.

doi:10.1007/s00296-021-04848-w

Cited by 3 publications

(6 citation statements)

References 20 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…9,11,12 Multiple laboratory abnormalities can be found in IgG4-RD, typically related to the specific organ involved. 2,10 In this case, the analytical study revealed an iron deficiency anemia probably due to the impaired iron intake associated with chronic inflammation, as the patient had maintained a vegetarian diet for several years and blood tests showed a small cell hypochromic anemia with decreased reticulocytes without decreased ferritin, with elevated erythrocyte sedimentation rate (ESR) and Creactive protein (CRP). The anemia was improved by iron supplementation, and after starting treatment with prednisolone and rituximab.…”

Section: Discussionmentioning

confidence: 93%

“…[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. 1 Considering the increased risk of cancer in patients diagnosed with IgG4-RD, 9,11,12 this progression raised the concern of a neoplastic transformation, prompting an urgent upper digestive endoscopy with biopsy, which was crucial to enable the exclusion of a gastric malignancy.…”

Section: Discussionmentioning

confidence: 99%

“…However, its chronicity, radiologic progression, high recurrence rates, and multiorgan fibroinflammatory involvement is well documented in previous literature 3–5 . 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 1 .…”

Section: Discussionmentioning

confidence: 99%

“…2 Second-line therapy consists of conventional immunosuppressants such as azathioprine, mycophenolate mofetil and cyclosporine or CD20 depletion with rituximab. 2,10 The ability of IgG4-RD to mimic infective, inflammatory and neoplastic processes compels the medical community to consider IgG4-RD as a differential diagnosis in multisystemic processes, especially in patients with atypical presentations.…”

Section: Introductionmentioning

confidence: 99%

“…Corticosteroid therapy (CCT) is quickly effective, but disease outbreaks are common after gradual dose reduction 2 . Second‐line therapy consists of conventional immunosuppressants such as azathioprine, mycophenolate mofetil and cyclosporine or CD20 depletion with rituximab 2,10 …”

Section: Introductionmentioning

confidence: 99%

See 4 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

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.

show abstract

Section: Discussionmentioning

confidence: 93%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 3 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

Clinical features and relapse risks factors of IgG4 related disease: a single-center retrospective study

Zhang,

Zeng,

Tian

et al. 2023

Clin Exp Med

View full text Add to dashboard Cite

Active IgG4-related disease with bone marrow involvement: a report of 2 cases and case-based review

Liu

Wang

2022

Eur J Med Res

View full text Add to dashboard Cite

IgG4-related disease (IgG4-RD) is a systemic fibro-inflammatory disease, histopathologically characterized by dense lymphoplasmacytic infiltration rich in IgG4-positive plasma cells in affected organs. Classic hematologic presentations including lymphadenopathy, eosinophilia and polyclonal hypergammaglobulinemia are common, whereas bone marrow involvement of IgG4-RD is rarely reported. Here we present two patients of multi-organ IgG4-RD with bone marrow involvement, one on bone marrow biopsy, and the other on PET/CT. Presentations of other organ involvement included biopsy-proven IgG4-related tubulointerstitial nephritis, lymphadenopathies, submaxillary glands, arteritis, asthma, dysosmia, and constitutional symptoms. Bone marrow involvement was initially suspected due to leukopenia, anemia and thrombocytopenia in case#1, and was finally confirmed by histological evidence of significant IgG4-positive plasma cells infiltration in bone marrow. In case#2, we incidentally observed high uptakes of multi-bone marrow on 18F-FDG-PET/CT, with the maximum SUV value similar to that of the kidneys, submaxillary glands and hilar, mediastinal lymph nodes by 18F-FDG-PET/CT. Symptoms and all the hematologic presentation improved rapidly in both patients after steroids initiation. These two cases illustrate the rare bone marrow involvement in active IgG4-RD accompanied by other hematologic syndromes. The significance of disease pathogenesis is worthy of further study.

show abstract

IgG4-related disease with multiorgan involvement: a case-based review

Cited by 3 publications

References 20 publications

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

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

Clinical features and relapse risks factors of IgG4 related disease: a single-center retrospective study

Active IgG4-related disease with bone marrow involvement: a report of 2 cases and case-based review

Contact Info

Product

Resources

About