scholarly journals SAT0508 Ace-inhibitors in arterial hypertension in ssc patients display a risk factor for scleroderma renal crisis – a eustar analysis

2018 ◽  
Author(s):  
S. Adler ◽  
P.A. Varisco ◽  
O. Distler ◽  
L. Buetikofer ◽  
O. Kowal-Bielecka ◽  
...  
Keyword(s):  
Risk Factor ◽  
Arterial Hypertension ◽  
Ace Inhibitors ◽  
Scleroderma Renal Crisis ◽  
Renal Crisis

scholarly journals ACE inhibitors in SSc patients display a risk factor for scleroderma renal crisis—a EUSTAR analysis

2020 ◽  
Vol 22 (1) ◽  
Author(s):  
Lukas Bütikofer ◽  
◽  
Pierre A. Varisco ◽  
O. Distler ◽  
O. Kowal-Bielecka ◽  
...  
Keyword(s):  
Risk Factor ◽  
Ace Inhibitors ◽  
Scleroderma Renal Crisis ◽  
Renal Crisis

Exposure to ACE inhibitors prior to the onset of scleroderma renal crisis—Results from the International Scleroderma Renal Crisis Survey

2014 ◽  
Vol 43 (5) ◽  
pp. 666-672 ◽  
Author(s):  
Marie Hudson ◽  
Murray Baron ◽  
Solène Tatibouet ◽  
Daniel E. Furst ◽  
Dinesh Khanna
Keyword(s):  
Ace Inhibitors ◽  
Scleroderma Renal Crisis ◽  
Renal Crisis

scholarly journals An International, Web-Based, Prospective Cohort Study to Determine Whether the Use of ACE Inhibitors prior to the Onset of Scleroderma Renal Crisis Is Associated with Worse Outcomes—Methodology and Preliminary Results

2010 ◽  
Vol 2010 ◽  
pp. 1-7 ◽  
Author(s):  
Marie Hudson ◽  
Murray Baron ◽  
Ernest Lo ◽  
Joanna Weinfeld ◽  
Daniel E. Furst ◽  
...  
Keyword(s):  
Cohort Study ◽  
Ace Inhibitors ◽  
Scleroderma Renal Crisis ◽  
Web Based ◽  
Number Of Patients ◽  
Cohort Study Design ◽  
One Year ◽  
Incident Cases ◽  
E Mail ◽  
Renal Crisis

Background. To describe the methodology of a study designed to determine whether systemic sclerosis (SSc) patients with incident scleroderma renal crisis (SRC) on angiotensin converting enzyme (ACE) inhibitors prior to the onset of SRC have worse outcomes.Methods. Prospective, international cohort study of SRC subjects identified through an ongoing web-based survey. Every second Friday afternoon, an e-mail was sent to 589 participating physicians to identify new cases of SRC. Death or dialysis at one year after the onset of SRC will be compared in patients exposed or not to ACE inhibitors prior to the onset of SRC.Results. Fifteen months after the start of the survey, we had identified 76 incident cases of SRC. Of these, 66 (87%) had a hypertensive SRC and 10 (13%) a normotensive SRC. Twenty-two percent (22%) of the patients were on an ACE inhibitor immediately prior to the onset of the SRC. To date, we have collected one-year follow-up data on approximately 1/3 of the cohort. Of these, over 50% have died or remain on dialysis at one year.Conclusion. An international, web-based cohort study design is a feasible method of recruiting a substantial number of patients to study an infrequent vascular manifestation of SSc.

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)

Rheumatology ◽  
2020 ◽  
Author(s):  
Melani Pestaña-Fernández ◽  
Manuel Rubio-Rivas ◽  
Carles Tolosa-Vilella ◽  
Alfredo Guillén-Del-Castillo ◽  
Dolores Colunga-Argüelles ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Treatment Group ◽  
Arterial Hypertension ◽  
Incidence Rate ◽  
Scleroderma Renal Crisis ◽  
Digital Ulcers ◽  
Phosphodiesterase 5 Inhibitors ◽  
Pulmonary Arterial ◽  
Phosphodiesterase 5 ◽  
Renal Crisis

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 < 0.001) and tendon friction rubs (12 vs 6%; P = 0.038), whereas the lcSSc subset was more prevalent in the no-treatment group (57 vs 66%; P = 0.031) along with ACA (37 vs 46%; P = 0.031). 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.

scholarly journals Scleroderma Renal Crisis in a Case of Mixed Connective Tissue Disease Treated Successfully with Angiotensin-Converting Enzyme Inhibitors

2021 ◽  
Vol 2021 ◽  
pp. 1-6
Author(s):  
Jomana Madieh ◽  
Iman Khamayseh ◽  
Alaa Hrizat ◽  
Abdurrahman Hamadah ◽  
Kamel Gharaibeh
Keyword(s):  
Connective Tissue ◽  
Connective Tissue Disease ◽  
Ace Inhibitors ◽  
Lupus Erythematosus ◽  
Mixed Connective Tissue Disease ◽  
Enzyme Inhibitors ◽  
Angiotensin Converting Enzyme Inhibitors ◽  
Scleroderma Renal Crisis ◽  
Converting Enzyme Inhibitors ◽  
Renal Crisis

Mixed connective tissue disease (MCTD) is a rheumatic disease syndrome with overlapping features of scleroderma, systemic lupus erythematosus, and polymyositis. An extremely rare but serious complication that can occur in MCTD is scleroderma renal crisis (SRC). There have been different approaches to the treatment of SRC associated with MCTD. We present a case of MCTD with chronic features of Raynaud’s phenomenon, dermatomyositis, and thrombocytopenia complicated with acute SRC which showed a great response to ACE inhibitors. Here, we advise the early and aggressive use of ACE inhibitors as soon as SRC is suspected.

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