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Clinical and Molecular Allergy

, 14:13

Clinical Immunology


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.

KeywordsMastocytosis Systemic sclerosis Mastcells Fibrosis AbbreviationsSScsystemic sclerosis


MCTmast cells tryptase-positive chymase-negative

MCTCmast cells tryptase-positive chymase-positive

ANAantinuclear antibodies

anti-SS-Aanti-Sjögren’s-syndrome-related antigen A

anti-SS-Banti-Sjögren’s-syndrome-related antigen B

anti-Smanti-Smith antibodies

anti-Scl-70anti-topoisomerase I antibodies

anti-Jo1anti-intracellular enzyme histidyl tRNA synthetase antibodies

CKcreatine kinase

LDHlactate dehydrogenase

SGOTserum glutamic-oxaloacetic transaminase

SGPTserum glutamic-pyruvic transaminase

HLAhuman leukocyte antigen

CTcomputerized tomography

CDcluster of differentiation

PETpositron emission tomography

TGF-βtransforming growth factor β

PDGFplatelet-derived growth factor

VEGFvascular endothelial growth factor


GM-CSFgranulocyte-macrophage colony-stimulating factor

IPFidiopathic pulmonary fibrosis

SSc-ILDsystemic sclerosis associated interstitial lung disease

c-kittyrosine-protein kinase kit

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Autor: Gianluca Bagnato - William Neal Roberts - Davide Sciortino - Donatella Sangari - Santa Cirmi - Roneka L. Ravenell - Michel


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