Potential roles of non-lymphocytic cells in the pathogenesis of IgG4-related disease

Cai, Shaozhe; Hu, Ziwei; Chen, Yu; Zhong, Jixin; Dong, Lingli

doi:10.3389/fimmu.2022.940581

Cited by 5 publications

(4 citation statements)

References 88 publications

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“…Treatment with corticosteroids is effective in 90% of cases. Although the exact pathogenesis is unknown, it is considered to be an autoimmune proinflammatory condition mediated by fibroblasts (5).…”

Section: Discussionmentioning

confidence: 99%

“…Regarding the pathogenesis that caused this adverse event, radiation injury triggers inflammation and ultimately stimulates the transdifferentiation of fibroblasts into myofibroblasts, which produce excess collagen and other extracellular matrix components (7). The transition of fibroblasts into myofibroblasts is a common feature of fibrosis-related diseases and the underlying mechanism is regarded as one of the pathogenetic features of IgG4RD (5). Therefore, irradiation may have induced the recurrence of IgG4RD in the treated area.…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

A Unique Adverse Event of Radiotherapy in a Patient With IgG4-related Disease: A Case Report

AIMI,

MURAI,

TAKINO

et al. 2023

In Vivo

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

A Unique Adverse Event of Radiotherapy in a Patient With IgG4-related Disease: A Case Report

AIMI,

MURAI,

TAKINO

et al. 2023

In Vivo

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“…IgG4-RD occurs in a variety of organs, including the pancreas, bile duct, retroperitoneum, lacrimal gland, salivary gland, pituitary gland, prostate, lung, heart, kidney, aorta, and thyroid gland. The condition may be misdiagnosed as malignancy, infection, inflammatory disorders, or other immune-mediated disease (20). cardiovascular lesions include aortitis, arteritis, periaortitis, periarteritis, pericarditis, and inflammatory aneurysm (21).…”

Section: Meanwhile Tfh Cells Promote the Proliferation And Differenti...mentioning

confidence: 99%

IgG4-related disease with massive pericardial effusion diagnosed clinically using FDG-PETCT: a case report

Wei,

Qi,

Wei

et al. 2023

Front. Immunol.

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BackgroundIgG4-related disease (IgG4-RD) is a systemic inflammatory disease which involves various organs such as the pancreas, lacrimal gland, salivary gland, retroperitoneum, and so on. These organs can be affected concomitantly. 18-Fluorodeoxyglucose positron emission tomography computed tomography (FDG-PETCT) is a systemic examination which can identify active inflammation and detect multiple organ involvement simultaneously. Pericardial involvement is rare in IgG4-RD, early detection and treatment can greatly improve the prognosis of patients.Case summaryWe reported a 82-year-old female patient referred to our department complaining of chest tightness and abdominal fullness for 8 months and massive pericardial effusion for 2 months. A large amount of pericardial effusion was found during the hospitalization of Gastroenterology. Then she was transferred to cardiology. Although infectious, tuberculous, and neoplastic pericardial effusions were excluded, there was still no diagnosis. The patients were examined by FDG-PETCT which considered IgG4-RD. After coming to our department, the results of the patient’s laboratory tests showed that immunoglobulin subgroup IgG4 was 14.0 g/L. Then we performed a biopsy of the right submandibular gland. Pathological morphology and immunohistochemistry suggested IgG4-RD. Combined with level of IgG4, clinical, pathological and immunohistochemical results, we determined the final diagnosis of IgG4 related diseases. Then we gave glucocorticoid and immunosuppressant treatment. At the end, pericardial effusion was completely absorbed. As prednisone acetate was gradually reduced, no recurrence of the disease has been observed.ConclusionPericardial effusion can be the initial presentation in IgG4-RD. For patients with massive pericardial effusion of unknown cause, early detection of IgG4 is recommended, and PETCT may be helpful for obtaining the diagnosis.

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“…Although the etiology of this disease remains largely unknown, recent studies have shed light on the significant roles of T- and B-lymphocytes [ 2 – 5 ]. While there have been notable advances in understanding the involvement of immune cells in the pathology of IgG4-RD, limited insight presently exists regarding the implications of non-immune cells on IgG4-RD pathogenesis, as well as their distinctive characteristics [ 6 ]. Notably, unlike other fibrotic diseases such as systemic sclerosis and idiopathic pulmonary fibrosis, the fibrotic changes observed in IgG4-RD are generally reversible, indicating the existence of distinct profibrotic machinery [ 7 ].…”

Section: Introductionmentioning

confidence: 99%

Single-cell RNA sequencing of submandibular gland reveals collagen type XV-positive fibroblasts as a disease-characterizing cell population of IgG4-related disease

Tanaka,

Yamamoto,

Iwata

et al. 2024

Arthritis Res Ther

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Objectives IgG4-related disease (IgG4-RD) is a systemic autoimmune disease with an unknown etiology, affecting single/multiple organ(s). Pathological findings include the infiltration of IgG4-producing plasma cells, obliterative phlebitis, and storiform fibrosis. Although immunological studies have shed light on the dysregulation of lymphocytes in IgG4-RD pathogenesis, the role of non-immune cells remains unclear. This study aimed to investigate the demographics and characteristics of non-immune cells in IgG4-RD and explore potential biomarkers derived from non-immune cells in the sera. Methods We conducted single-cell RNA sequence (scRNA-seq) on non-immune cells isolated from submandibular glands of IgG4-RD patients. We focused on fibroblasts expressing collagen type XV and confirmed the presence of those fibroblasts using immunohistochemistry. Additionally, we measured the levels of collagen type XV in the sera of IgG4-RD patients. Results The scRNA-seq analysis revealed several distinct clusters consisting of fibroblasts, endothelial cells, ductal cells, and muscle cells. Differential gene expression analysis showed upregulation of COL15A1 in IgG4-RD fibroblasts compared to control subjects. Notably, COL15A1-positive fibroblasts exhibited a distinct transcriptome compared to COL15A1-negative counterparts. Immunohistochemical analysis confirmed a significant presence of collagen type XV-positive fibroblasts in IgG4-RD patients. Furthermore, immune-suppressive therapy in active IgG4-RD patients resulted in decreased serum levels of collagen type XV. Conclusions Our findings suggest that collagen type XV-producing fibroblasts may represent a disease-characterizing non-immune cell population in IgG4-RD and hold potential as a disease-monitoring marker.

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Potential roles of non-lymphocytic cells in the pathogenesis of IgG4-related disease

Cited by 5 publications

References 88 publications

A Unique Adverse Event of Radiotherapy in a Patient With IgG4-related Disease: A Case Report

A Unique Adverse Event of Radiotherapy in a Patient With IgG4-related Disease: A Case Report

IgG4-related disease with massive pericardial effusion diagnosed clinically using FDG-PETCT: a case report

Single-cell RNA sequencing of submandibular gland reveals collagen type XV-positive fibroblasts as a disease-characterizing cell population of IgG4-related disease

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