High-dose immunosuppressive therapy for severe systemic sclerosis: initial outcomes

High-dose immunosuppressive therapy for severe systemic sclerosis: initial outcomes.

Systemic sclerosis (SSc) is a multisystem disease of presumed autoimmune pathogenesis for which no proven effective treatment exists. High-dose immunosuppressive therapy (HDIT) has been proposed as an investigational treatment for severe autoimmune diseases. Nineteen patients with poor-prognosis SSc underwent HDIT. The median age was 40 years (range, 23-61 years), the median modified Rodnan ski...

High-Dose Immunosuppressive Therapy and Autologous Hematopoietic Stem Cell Transplantation for Treatment of Severe Systemic Sclerosis

High-dose immunosuppressive therapy and autologous hematopoietic cell transplantation for severe systemic sclerosis: long-term follow-up of the US multicenter pilot study.

More effective therapeutic strategies are required for patients with poor-prognosis systemic sclerosis (SSc). A phase 2 single-arm study of high-dose immunosuppressive therapy (HDIT) and autologous CD34-selected hematopoietic cell transplantation (HCT) was conducted in 34 patients with diffuse cutaneous SSc. HDIT included total body irradiation (800 cGy) with lung shielding, cyclophosphamide (1...

Association Between Initial Oral Therapy and Outcomes in Systemic Sclerosis-Related Pulmonary Arterial Hypertension.

OBJECTIVE To compare time to clinical worsening (TTCW) based on initial oral therapy for pulmonary arterial hypertension (PAH) in patients with systemic sclerosis (SSc)-related PAH. METHODS Using data from the Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma (PHAROS) registry (a multicenter prospective observational study enrolling SSc patients with incident pulmon...

High frequency skin ultrasonography in systemic sclerosis

Background: Systemic sclerosis is an autoimmune connective tissue disorder which is characterised by cutaneous and internal organ fibrosis. Raynaud’s phenomenon is the earliest feature and may precede the onset of the disease by months to years. The heart, lungs, gastrointestinal tract, kidneys and other organs may be involved. Our aim was to evaluate skin thickness and echogenicity in systemic...