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Immune landscape in systemic sclerosis

Major Category: Research and Disease Areas

Subcategory: Loss of Tolerance

By Bhairav Paleja (Team Lead), Ahmad Lajam, Camillus Chua, Lai Liyun, Pavanish Kumar, Sharifah Nur Hazirah

Systemic sclerosis (SSc) is a multisystem connective tissue disease characterised by excessive extracellular matrix deposition, microvasculopathy and autoimmunity. Excessive collagen accumulation results in fibrosis of several organs such as skin and lungs. Pulmonary arterial hypertension (PAH) and interstitial lung diseases are major causes of death in SSc. The extent of organ involvement, severity of disease, progression of disease and prognosis is heterogenous in each patient. This necessitates identification of biomarkers that can help in diagnosis and prognosis of the disease. Furthermore, identifying biomarkers will also aid in understanding of disease pathogenesis in SSc. A variety of immunological abnormalities of T and B cells have been detected in SSc, however there is lack of systematic studies of functional relations between immune cell subsets and their role in pathogenesis of SSc. This lack of mechanistic knowledge hampers targeted intervention. The current study aims at an in depth analysis of systemic immune composition in SSc, using high dimensional mass-cytometry based immunophenotyping, with the potential to delineate mechanisms of pathogenesis and identify diagnostic and/or therapeutic targets.