Abstract
Treatment of early diffuse cutaneous systemic sclerosis (dcSSc) patients can be challenging especially when organ involvement is present. Comprehensive screening is therefore necessary to determine the nature and extent of heart, lung and kidney involvement. This should include ECG, cardiac MRI, thoracic HRCT, lung function testing, right heart catheterisation and laboratory tests including NT-proBNP and hs-cTnI. Such patients may be considered for autologous hematopoietic stem cell transplantation (HSCT) if they do not respond to conventional immunosuppressive medication and have other features of poor prognosis. Clinical decision making may be difficult however in those patients with cardiac involvement who are deemed at risk of serious adverse events from HSCT including treatment-related death from heart/lung failure due to cardiotoxicity of high dose cyclophosphamide, cytokine-release syndrome from ATG and/or fluid overload.
We describe two cases of poor prognosis dcSSc with subclinical myocardial involvement who underwent autologous stem cell transplantation with favourable outcomes. These cases demonstrate that HSCT may be considered even in dcSSc patients with cardiac involvement but are probably best managed in tertiary referral centers.
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