Mastocytosis and systemic sclerosis: a clinical association.

Davide Sciortino,Gianfilippo Bagnato,Roneka L. Ravenell,Gianluca Bagnato,William Neal Roberts,Sebastiano Gangemi,Donatella Sangari,Michele Navarra,Santa Cirmi

doi:10.1186/s12948-016-0050-3

Davide Sciortino, Gianfilippo Bagnato + Show 7 more

Open Access

https://doi.org/10.1186/s12948-016-0050-3

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Abstract

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.

Highlights

Systemic sclerosis (SSc) is a complex autoimmune disease characterized by vascular alterations and autoimmune activation leading to widespread organ fibrosis
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
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

Summary

Conclusions

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 onset of SSc in this case in turn was subsequently accompanied by the progression of the cutaneous form of mastocytosis into the widespread systemic phenotype This case raises the possibility that, the link between mast cell activity and fibrotic disease may be understood as a bidirectional one, or a feedback loop. Nintedanib is in clinical trial for ILD due to SSc. If tyrosine kinase inhibitors are found to play a role in therapeutic regimens aimed at fibrotic diseases such as idiopathic pulmonary fibrosis (IPF) and systemic sclerosis associated interstitial lung disease (SSc–ILD), it may be that they work in part by inhibition of slower, chronic, non-anaphylactic transdegraulation of mast cells, among other mechanisms. Author details 1 Division of Rheumatology, Department of Clinical and Experimental Medicine, University of Messina, Messina, Italy.

Background

Findings