1. The incidence rate of pulmonary arterial hypertension and scleroderma renal crisis in systemic sclerosis patients with digital ulcers on endothelin antagonist receptors (ERAs) and phosphodiesterase-5 inhibitors (PDE5i)
- Author
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A. Argibay, Carmen Pilar Simeón-Aznar, B. Marí-Alfonso, Luis Trapiella-Martínez, Ana Belén Madroñero-Vuelta, Dolores Colunga-Argüelles, Norberto Ortego-Centeno, Mayka Freire, Adela Marín-Ballvé, Alfredo Guillén-Del-Castillo, Manuel Rubio-Rivas, A.J. Chamorro, Cristina Gonzalez-Echavarri, Jose Antonio Todolí-Parra, Antoni Castro-Salomó, Gema María Lledó, José Antonio Vargas-Hitos, Xavier Pla-Salas, Vicent Fonollosa-Pla, Melani Pestaña-Fernández, Carles Tolosa-Vilella, María Esther Sánchez-García, and Luis Sáez-Comet
- Subjects
Endothelin Receptor Antagonists ,Male ,medicine.medical_specialty ,Scleroderma Renal Crisis ,030204 cardiovascular system & hematology ,Systemic scleroderma ,Gastroenterology ,Scleroderma ,endothelin antagonist receptors, phosphodiesterase-5 inhibitors, prevention, pulmonary arterial hypertension, scleroderma renal crisis, treatment ,Fingers ,03 medical and health sciences ,0302 clinical medicine ,Rheumatology ,Internal medicine ,Statistical significance ,Skin Ulcer ,medicine ,Humans ,Pharmacology (medical) ,Registries ,skin and connective tissue diseases ,030203 arthritis & rheumatology ,Pulmonary Arterial Hypertension ,Scleroderma, Systemic ,integumentary system ,business.industry ,Incidence ,Antagonist ,Retrospective cohort study ,Acute Kidney Injury ,Middle Aged ,Phosphodiesterase 5 Inhibitors ,medicine.disease ,Treatment Outcome ,Spain ,cGMP-specific phosphodiesterase type 5 ,Blood Vessels ,Female ,Endothelin receptor ,business - Abstract
Introduction Endothelin antagonist receptors (ERAs) and phosphodiesterase-5 inhibitors (PDE5i) are beneficial in pulmonary arterial hypertension (PAH) and digital ulcers (DU) and prevent from DU recurrences. Our study aimed to determine the difference in the incidence rate of PAH and scleroderma renal crisis (SRC) in patients with SSc and DU (SSc-DU) under ERAs/PDE5i or without treatment. Methods We conducted a retrospective cohort study including SSc-DU patients from the Spanish Scleroderma Registry (RESCLE). The primary outcome was the incidence rate of PAH and SRC in patients under ERAs/PDE5i or not. Results Some 544 patients out of 1817 (29.9%) in the RESCLE database had DU, 221 (40.6%) under ERAs/PDE5i and 323 (59.4%) not. The incidence rate (95% CI) difference between patients under treatment or not under did not reach statistical significance in PAH [−0.1 (−4.8, 4.69), P = 0.988] or in SRC [0.7 (−2.2, 3.7), P = 0.620]. However, the time from the first DU to the diagnosis of SRC was delayed in treated patients [mean (s.d.) 7.6 (5.8) years vs 2.9 (5.3); P = 0.021]. The dcSSc subset was more prevalent in the treatment group (36 vs 26%; P = 0.018), along with anti-topoisomerase I antibodies (34 vs 18%; P Conclusion There was no difference in the incidence rate of PAH and SRC between groups. However, treatment with ERAs and/or PDE5i appeared to delay the occurrence of SRC.
- Published
- 2021