Cosimo Bruni1, Laura Cometi2, Antonietta Gigante3, Edoardo Rosato3, Marco Matucci-Cerinic2. 1. Div. Rheumatology, Department of Experimental and Clinical Medicine, AOU Careggi, University of Florence, Florence, Italy. Electronic address: cosimobruni85@gmail.com. 2. Div. Rheumatology, Department of Experimental and Clinical Medicine, AOU Careggi, University of Florence, Florence, Italy. 3. Department of Translational and Precision Medicine, Sapienza University of Rome, Rome, Italy.
Abstract
OBJECTIVE: In Systemic Sclerosis (SSc), vasculopathy is the background of major vascular complications (MVCs), like digital ulcers (DUs), pulmonary arterial hypertension (PAH) and scleroderma renal crisis (SRC). We aimed to identify the predictors and to test the primary preventive effect of vasoactive/vasodilating drugs (VVD) for the development of MVCs in SSc MVCs-naïve patients. METHODS: patients fulfilling the ACR/EULAR 2013 classification criteria for SSc without history of MVCs were eligible. Data about clinical manifestations, laboratory and instrumental assessments and treatments were retrospectively collected at baseline and latest available follow-up. RESULTS: 134 SSc patients were enrolled (mean age 56.5 years ± 14.2, females 88.1%, limited subset 61.9%, ACA positivity 60.4%). In a mean of 43 ± 19 months of follow-up 12 (9.0%) patients developed at least 1 MVC (10 DU, 2 PAH and 1 SRC). Dyspnoea and arthritis at baseline were independent predictors for MVCs development (p = 0.012, and p = 0.002 respectively). No primary preventive effect of VVD on MVCs development was found. However, sildenafil reduced the renal resistive index increase (p = 0.042) and alprostadil slowed the DLco decline (p = 0.029). Both iloprost and angiotensin-receptor blockers (ARBs) delayed MVCs development, while angiotensin converting enzyme inhibitors (ACEi) determined an earlier onset of such MCVs. CONCLUSIONS: in SSc patients, our data confirm the role of arthritis and dyspnea as independent predictors of major vascular complications, in particular in MVCs-naïve patients. Prostanoids, sildenafil and ARBs, even in absence of a primary preventive action, might help in slowing disease progression and postponing the onset of MVCs.
OBJECTIVE: In Systemic Sclerosis (SSc), vasculopathy is the background of major vascular complications (MVCs), like digital ulcers (DUs), pulmonary arterial hypertension (PAH) and scleroderma renal crisis (SRC). We aimed to identify the predictors and to test the primary preventive effect of vasoactive/vasodilating drugs (VVD) for the development of MVCs in SSc MVCs-naïve patients. METHODS:patients fulfilling the ACR/EULAR 2013 classification criteria for SSc without history of MVCs were eligible. Data about clinical manifestations, laboratory and instrumental assessments and treatments were retrospectively collected at baseline and latest available follow-up. RESULTS: 134 SSc patients were enrolled (mean age 56.5 years ± 14.2, females 88.1%, limited subset 61.9%, ACA positivity 60.4%). In a mean of 43 ± 19 months of follow-up 12 (9.0%) patients developed at least 1 MVC (10 DU, 2 PAH and 1 SRC). Dyspnoea and arthritis at baseline were independent predictors for MVCs development (p = 0.012, and p = 0.002 respectively). No primary preventive effect of VVD on MVCs development was found. However, sildenafil reduced the renal resistive index increase (p = 0.042) and alprostadil slowed the DLco decline (p = 0.029). Both iloprost and angiotensin-receptor blockers (ARBs) delayed MVCs development, while angiotensin converting enzyme inhibitors (ACEi) determined an earlier onset of such MCVs. CONCLUSIONS: in SSc patients, our data confirm the role of arthritis and dyspnea as independent predictors of major vascular complications, in particular in MVCs-naïve patients. Prostanoids, sildenafil and ARBs, even in absence of a primary preventive action, might help in slowing disease progression and postponing the onset of MVCs.
Authors: Aurélie Philippe; Gunnar Kleinau; Jason Jannis Gruner; Sumin Wu; Daniel Postpieszala; David Speck; Harald Heidecke; Simon J Dowell; Gabriela Riemekasten; Peter W Hildebrand; Julian Kamhieh-Milz; Rusan Catar; Michal Szczepek; Duska Dragun; Patrick Scheerer Journal: Int J Mol Sci Date: 2022-04-02 Impact factor: 5.923
Authors: Rusan Catar; Melanie Herse-Naether; Nan Zhu; Philine Wagner; Oskar Wischnewski; Angelika Kusch; Julian Kamhieh-Milz; Andreas Eisenreich; Ursula Rauch; Björn Hegner; Harald Heidecke; Angela Kill; Gabriela Riemekasten; Gunnar Kleinau; Patrick Scheerer; Duska Dragun; Aurelie Philippe Journal: Int J Mol Sci Date: 2021-12-27 Impact factor: 5.923