Real-world clinical data support the use of Opsumit (macitentan) to treat people with pulmonary arterial hypertension (PAH) due to scleroderma (PAH-SSc). The findings revealed similar safety results and comparable therapeutic efficacy in people with PAH-SSc as seen in patients with idiopathic or hereditary…
A recently discovered variant of the IFNL3 gene increases the risk of pulmonary fibrosis (PF) in people with scleroderma, a study reveals. In contrast, no such link was found between this variant and worsened skin fibrosis in these patients. Findings also revealed markedly increased serum levels of…
People with systemic sclerosis (SSc) show a wide range of eye complications, with dry eye disease, eyelid skin alterations, and retinal abnormalities as the most common, a Hungarian study shows. The variety of eye complications found in these patients reflect the heterogeneity, or diversity, of SSc —…
People with scleroderma can develop autoimmune diseases like neuromyelitis optica (NMO), a condition that affects the nervous system — especially the nerves of the eye and spinal cord, a case report suggests. Correctly diagnosing a co-occurring autoimmune disease help to ensure that scleroderma patients receive proper care early,…
Patients with scleroderma may need longer-term treatment with Adempas (riociguat) to heal their digital ulcers, results of a Phase 2 clinical trial suggest. Findings were published in the study, “A multicenter randomized, double-blind, placebo-controlled pilot study to assess the efficacy and safety of riociguat in systemic…
Treatment for one year with the immunosuppressant Cytoxan (cyclophosphamide) improves lung function in scleroderma patients with interstitial lung disease (SSc-ILD) — but these benefits wear off after stopping treatment, data from Phase 3 trials show. The study, “Cyclophosphamide for Systemic Sclerosis-related Interstitial Lung Disease: A Comparison of…
Immunosuppressive treatment of early-stage interstitial lung disease (ILD) in people with systemic sclerosis may be a potential therapeutic option to prevent ILD progression, a study suggests. The study, “Association between immunosuppressive therapy and course of mild interstitial lung disease…
The U.S. Food and Drug Administration (FDA) has granted breakthrough therapy designation to Ofev (nintedanib) as a potential therapy for progressive fibrosing interstitial lung disease (ILD) — which also affects systemic sclerosis patients — the treatment’s developer, Boehringer Ingelheim, has announced. Breakthrough therapy designation is given to investigational compounds that have…
Referral to specialized centers, focusing on skin lesions as well as joint and neurological manifestations, and treatment with corticosteroids and disease-modifying antirheumatic drugs (DMARDs), are among new expert recommendations for juvenile localized scleroderma. The study supporting those recommendations, “Consensus-based recommendations for the management of juvenile localized…
The first person has been enrolled in a clinical trial evaluating the investigational therapy bermekimab in adults with scleroderma, XBiotech, the developer of bermekimab, announced. Although the cause of scleroderma is unknown, the disease is thought to be at least partly driven by abnormally high levels of an inflammatory…
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