Telangiectasic mastocytosis with systemic sclerosis

Frigui, M.; Dupin, Nicolas; Carlotti, A.; Bussone, Guillaume; Pestre, Vincent; Charles, Pierre; Crabol, Yoann; Bérezné, Alice; Guillevin, Loı̈c; Mouthon, Luc

doi:10.1016/j.lpm.2012.10.014

Cited by 7 publications

(3 citation statements)

References 9 publications

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“…Mast cell infiltration was detected from lip biopsy in very early stages of SSc and preceded the onset of skin changes and more recently cutaneous mastocytosis was found to precede the onset of SSc in a 36‐year‐old woman suggesting that mast cells may play an early role in the onset of SSc. Mast cell infiltration has also been detected late in the development of SSc and in a subset of SSc patients with localized scleroderma‐like lesions . An increase in mast cell numbers has also been detected in SSc skin .…”

Section: Mast Cellsmentioning

confidence: 99%

The immunopathogenesis of fibrosis in systemic sclerosis

Brown

O’Reilly

2018

Clinical and Experimental Immunology

141

113

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Summary Systemic sclerosis (SSc) is an idiopathic systemic autoimmune disease. It is characterized by a triad of hallmarks: immune dysfunction, fibrosis and vasculopathy. Immune dysfunction in SSc is characterized by the activation and recruitment of immune cells and the production of autoantibodies and cytokines. How immune abnormalities link the fibrosis and vasculopathy in SSc is poorly understood. A plethora of immune cell types are implicated in the immunopathogenesis of SSc, including T cells, B cells, dendritic cells, mast cells and macrophages. How these different cell types interact to contribute to SSc is complicated, and can involve cell‐to‐cell interactions and communication via cytokines, including transforming growth factor (TGF)‐β, interleukin (IL)‐6 and IL‐4. We will attempt to review significant and recent research demonstrating the importance of immune cell regulation in the immunopathogenesis of SSc with a particular focus on fibrosis.

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Section: Mast Cellsmentioning

confidence: 99%

The immunopathogenesis of fibrosis in systemic sclerosis

Brown

O’Reilly

2018

Clinical and Experimental Immunology

141

113

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“…A previous case series study described this rare association in which systemic sclerosis precedes the onset of cutaneous mastocytosis [ 14 ], perhaps suggesting some underlying stimulation of the mast cell compartment as a feature of the SSc. The investigators reported two cases of skin lesions associated with mast-cell infiltrates compatible with the diagnosis of cutaneous mastocytosis at 30 and 6 years after the onset of SSc.…”

Section: Discussionmentioning

confidence: 93%

Mastocytosis and systemic sclerosis: a clinical association

et al. 2016

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BackgroundSystemic sclerosis (SSc) is a complex autoimmune disease characterized by vascular alterations and autoimmune activation leading to widespread organ fibrosis. At the early stage of disease when organ involvement and extent of disease are emerging, mast cells may have some role, as implied by both symptoms and histologic evidence.Case presentationA female patient diagnosed with cutaneous mastocytosis experienced the onset of systemic sclerosis after 15 years followed by the switch of mastocytosis to the systemic phenotype. A literature review on the evidences related to mast-cells activation in systemic sclerosis is presented below.ConclusionsFor clinicians, more attention must be paid to the potential association between systemic sclerosis and cancer. This case suggests that a proliferative disease in the mast cell compartment—though representing a rare association—may not be completely unexpected in SSc and perhaps excess mast cell activity can serve a pathogenic role in promoting fibrotic disease.

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“…Mast cells also express KIT receptor, so imatinib may have partially effect on mast cells in the treatment for SSc. Moreover, some reports described SSc cases associated mastocytosis, which is the proliferative disease in the mast cell compartment [41,42] .…”

Section: Mast Cells In Systemic Sclerosismentioning

confidence: 99%

Mast cells in collagen diseases

Mikita

Inaba²,

Yoshimasu³

et al. 2017

Trends Immunother

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Mast cells are involved in many immune reactions and diseases through 1) the expressions of several receptors, 2) productions of various mediators such as histamine, cytokines, and chemokines, 3) direct in teractions with immune cells. Besides allergic diseases, the involvement of mast cells has been also in vestigated in autoimmune diseases such as bullous pemphigoid, rheumatoid arthritis, and multiple sclerosis. Moreover, several studies reported the involvement of mast cells in collagen diseases. In this article, we review recent findings about the role of mast cells especially in systemic lupus erythematosus and systemic sclerosis. In these diseases, mast cells seem to be involved in local inflammation and tissue damage in the targeted organ or local immunosuppression rather than the development of autoimmunity including production of autoantibodies.

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Telangiectasic mastocytosis with systemic sclerosis

Cited by 7 publications

References 9 publications

The immunopathogenesis of fibrosis in systemic sclerosis

The immunopathogenesis of fibrosis in systemic sclerosis

Mastocytosis and systemic sclerosis: a clinical association

Mast cells in collagen diseases

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