Vasoactive Therapy in Systemic Sclerosis: Real-life Therapeutic Practice in More Than 3000 Patients

Abstract

Objective.Vasculopathy is a key factor in the pathophysiology of systemic sclerosis (SSc) and the main cause for Raynaud phenomenon (RP), digital ulcers (DU), and/or pulmonary arterial hypertension (PAH). It is so far unknown how patients with SSc are treated with vasoactive agents in daily practice. To determine to which extent patients with SSc were treated with different vasoactive agents, we used data from the German Network for Systemic Scleroderma registry.Methods.The data of 3248 patients with SSc were analyzed.Results.Patients were treated with vasoactive drugs in 61.1% of cases (1984/3248). Of these, 47.6% received calcium channel inhibitors, followed by 34.2% treated with angiotensin-converting enzyme (ACE) inhibitors, 21.1% treated with intravenous (IV) prostanoids, 10.1% with pentoxifylline, 8.8% with angiotensin 1 receptor antagonists (AT1RA), 8.7% with endothelin 1 receptor antagonists (ET1RA), 4.1% with phosphodiesterase type 5 (PDE5) inhibitors, and 5.3% with others. Patients with RP received vasoactive therapy in 63.3% of cases, with DU in 70.1%, and with PAH in 78.2% of cases. Logistic regression analysis revealed that patients with PAH were significantly more often treated with PDE5 inhibitors and ET1RA, and those with DU with ET1RA and IV prostanoids. In addition, 41.8% of patients were treated with ACE inhibitors and/or AT1RA. Patients registered after 2009 received significantly more often ET1RA, AT1RA, and IV prostanoids compared with patients registered prior to 2005.Conclusion.These data clearly indicate that many patients with SSc do not yet receive sufficient vasoactive therapy. Further, in recent years, a marked change of treatment regimens can be observed.

Treatment modalities and drug survival in a systemic sclerosis real-life patient cohort

“…Among more advanced vasoactive agents, ERAs were the treatment of choice in the majority of cases. In our study, the percentage of sildenafil-and iloprost-treated patients was significantly lower compared to other large published SSc cohorts [20,21]. A possible explanation is that these two drugs are not approved by the national insurance system for the management of digital vasculopathy in Greece and their prescription requires special permission from a national committee, thus limiting their ordinary use.…”

Serum concentration of beta-trace protein (BTP) as a potential biomarker of systemic sclerosis

“…Vasoactive therapy includes e.g. intravenous prostanoids and prostacyclin analogs [7,21]. Prostaglandins are rapidly metabolized in vivo, which is usually associated with a significant decrease in their biological activity; therefore their pharmacological analogs are used.…”

“…Dlatego w wielu rekomendacjach dotyczących SSc terapia rozszerzająca naczynia jest podstawowym postępowaniem w celu ograniczenia uszkodzenia komórek śródbłonka i powikłań naczyniowych, takich jak zapalenie, niedotlenienie tkanek i owrzodzenia [20]. Terapia naczyniowa obejmuje dożylne prostanoidy i analogi prostacykliny [7,21]. Prostaglandyny są szybko metabolizowane in vivo, co wiąże się ze znacznym zmniejszeniem ich aktywności biologicznej, dlatego stosuje się ich analogi farmakologiczne.…”

Versorgungsrealität der stationären vasoaktiven Therapie mit Prostazyklinderivaten bei Patienten mit akralen Durchblutungsstörungen bei systemischer Sklerose in Deutschland

“…Die systemische Sklerose (SSc) ist eine Multisystemerkrankung, die sich nicht nur an der Haut, sondern auch kardiopulmonal, renal, gastrointestinal und am Bewegungsapparat manifestieren kann [4]. Das sekundäre Raynaud-Syndrom (Raynaud-Phänomen [RP]), das bei mehr als 93 % der Patienten als [15] initiales Symptom vorhanden ist, kann -je nach Subtyp -den weiteren Organbeteiligungen Jahre bis Jahrzehnte vorausgehen und die Patienten in ihrer Lebensqualität massiv beeinträchtigen. Aufgrund rezidivierender Attacken können hypoxische Gewebeschäden auftreten, die sich in Ulzerationen, Fissuren, "pitting scars" oder digita-len Nekrosen äußern.…”