Proportion Of Systemic Sclerosis Patients Research Articles

Iloprost Duration for Digital Ulcers in Systemic Sclerosis: French Retrospective Study at Two Centers and Literature Review.

ObjectiveIschemic digital ulcers (DUs) are frequent and severe complications of systemic sclerosis (SSc). Treatment options for SSc-related digital vasculopathy are based on aggressive vasodilation, with the objective to improve blood flow in ischemic areas. Intravenous prostanoids are recommended to treat active DUs. However, the level of evidence for the duration of 5 days is low. Therefore, the aim of this study was to determine whether prolonging the infusion beyond 5 days increases the rate of healing of active DUs in SSc.MethodsThis is an observational longitudinal retrospective bicenter study from 2000 to 2017. The objective was to compare the healing rate and time (defined by a healing of at least 50% of DUs) between two durations of iloprost administration: 5 days or less, or more than 5 days.ResultsForty-one patients, with a mean age of 47 ± 15 years at diagnosis and 32 (78%) females have been included. Systemic sclerosis was diffuse in 10 (24%) cases and 13 (32%) had an interstitial lung disease. A total of 243 iloprost infusions for DUs were performed: 140 infusions for 5 days or less, and 103 infusions for more than 5 days (prolonged duration). Patients with active DUs which received >5 days of iloprost had higher modified Rodnan skin scale at the time of iloprost infusion (median 33 vs. 15; p < 0.05), more interstitial lung disease (44 vs. 27%; p < 0.05), more anti-topoisomerase I antibody positivity (59 vs. 44%; p < 0.05), and received more previous cyclophosphamide therapy (48 vs. 19%; p < 0.05). While the number of active DUs before iloprost infusion was not significantly different among those who received ≤5 days and >5 days of iloprost, the time to healing after iloprost infusion significantly decreased in SSc patients who received >5 days iloprost infusion: 48 [7–392] vs. 91 [9–365] days (p < 0.05). The proportion of SSc patients with healed DUs tended to increase in patients with >5 days iloprost infusion (log rank = 0.06). The number of patients with complete DU healing at day 90 was significantly increased in SSc who received >5 days of iloprost: 53 (51%) vs. 52 (37%) (p < 0.05). In addition, the time to healing was not significantly associated with the use of calcium channel blockers, endothelin receptor antagonists or a combination of PDE-5 inhibitors.ConclusionProlonging duration of iloprost >5 days could improve the healing rate and the time to healing of SSc-related DUs. Prospective randomized studies are needed to confirm these data and define the optimal duration of iloprost therapy.

Esophageal Involvement and Determinants of Perception of Esophageal Symptoms Among South Koreans With Systemic Sclerosis.

Background/AimsOur study aims to characterize esophageal motor function; evaluate the relationships among esophagogastroduodenoscopy (EGD), high-resolution manometry (HRM), and 24-hour esophageal multichannel intraluminal impedance monitoring combined with pH-metry (MII-pH); and elucidate the determinants of esophageal symptom perception in South Koreans with systemic sclerosis (SSc).MethodsWe reviewed prospectively collected HRM (n = 46), EGD (n = 41), and MII-pH (n = 37) data from 46 consecutive patients with SSc (42 females; mean age 50.1 years) who underwent esophageal tests between June 2013 and September 2018.ResultsThe most common HRM diagnosis was normal (39.1%), followed by ineffective esophageal motility (23.9%) and absent contractility (21.7%). Erosive esophagitis was observed in 12.2% of total SSc patients, with a higher frequency in patients with absent contractility than those with normal motility (44.5% vs 0.0%, P = 0.01). Pathologic acid exposure was observed in 6 patients (20.0%) and positive symptom association in 18 patients (60.0%) in MII-pH tests of symptomatic patients. The proportion of SSc patients with esophageal symptoms not explained by reflux or mucosal or motor esophageal abnormalities was 33.0%.ConclusionsEsophageal involvement among South Koreans with SSc was characterized by heterogeneous motility patterns, with a higher prevalence of normal motility and lower prevalence of erosive esophagitis. Reflux hypersensitivity or functional heartburn might be partly attributed to the perception of esophageal symptoms in SSc patients who have neither gastroesophageal reflux disease nor esophageal dysmotility.

Pulmonary arterial hypertension screening practices in scleroderma patients among Canadian rheumatologists.

Current international guidelines recommend annual screening for pulmonary arterial hypertension with transthoracic echocardiogram and pulmonary function testing in all patients with scleroderma (systemic sclerosis). Our objectives were to determine Canadian rheumatologists' screening practices for pulmonary arterial hypertension in patients with systemic sclerosis and identify reasons why current guideline recommendations may not be followed. A survey was emailed to all Canadian Rheumatology Association members. They self-identified as systemic sclerosis experts or non-experts and provided basic demographic data. Participants were asked how frequently they screened with transthoracic echocardiogram and pulmonary function testing and, if applicable, why they did not adhere to recommendations. A total of 71 rheumatologists participated, of whom 43 identified as non-experts. Overall, 81.4% ordered annual transthoracic echocardiogram and 77.6% annual pulmonary function testing. Rates of annual transthoracic echocardiogram testing were similar between experts and non-experts, whereas experts ordered annual pulmonary function testing more often. Clinicians with a higher proportion of systemic sclerosis patients in their practice were more likely to follow guidelines. There was an inverse relationship between years in practice and adherence to screening guidelines. The most common reason for not following screening guidelines was disagreement with recommendations, followed by unfamiliarity with guidelines. Pulmonary arterial hypertension screening rates remain sub-optimal in Canada but have improved since 2012. Failure to adopt guidelines is due to rheumatologists disagreeing with or not knowing current recommendations. Future studies should examine why rheumatologists disagree with guidelines and how to improve awareness.

Effect of season on clinical outcomes of Thai systemic sclerosis: Analysis of the Thai national healthcare database

Objectives: We aimed to determine rate of admission, mortality rate, and causes of death in systemic sclerosis (SSc) patients after stratifying by season.Method: A cross-sectional analysis was performed of patients over 15 in the national database with a primary diagnosis of SSc (ICD-10:M34) covered by the National Health Security Office hospitalized between 2014 and 2018. The seasons were stratified into hot, rainy, and Cool dry seasons.Results: Included were 2480 SSc patients with 3684 admissions: most (64.3%) were female. The respective mean age and median length of stay was 56.9 ± 12.3 years and 3 days (IQR 2–6). The admission rate was highest during the rainy season (1574 visits, 42.7%), followed by the cool dry season (1183 visits, 32.1%) then the hot season (927 visits, 25.2%). During the 13,180 person-days, 1660 SSc patients died resulting in a mortality rate of 12.1 per 100 person-days. The proportion of SSc patients who died in the Cool dry season was significantly higher than in the hot or rainy season (p = .04). Pulmonary involvement in SSc was the most common cause of death in the hot season, which is greater than in the other seasons (p = .004). By comparison, death due to cardiac involvement in SSc was common during the rainy season and cool dry seasons (p = .04).Conclusion: The admission rate among Thai SSc patients was greatest during the rainy season, while mortality was highest during the cool dry season. The most common causes of death were SSc-related irrespective of season, particularly cardiopulmonary involvement.

Pulmonary arterial hypertension screening of systemic sclerosis patients in clinical practice: an independent chart review

Patients with systemic sclerosis (SSc) are at increased risk of pulmonary arterial hypertension (PAH). Guidelines recommend annual screening with pulmonary function testing (PFT) and transthoracic echocardiogram (TTE). Through auditing the charts of 11 rheumatologists associated with McMaster University, we evaluated the proportion of SSc patients without PAH or pulmonary fibrosis who receive annual TTE, PFT, and dyspnea screening. Screening rates between self-identified SSc experts and non-experts were compared. In cases where screening tests were abnormal, charts were reviewed for evidence of cardiologist or respirologist referral. In total, 136 patients’ charts were included. Annual screening for dyspnea was very common (88% of patients, 119/134). Annual PAH screening via TTE (74%, 100/135) and PFT (79%, 107/136) was less common. Annual dyspnea screening, TTE, and PFT were more commonly performed by SSc experts than by non-experts (94% vs. 83%, p = 0.03; 85% vs. 61%, p = 0.002; 93% vs. 62%, p&lt;0.001, respectively). Nearly all patients with an abnormal TTE (10/11, 91%) and PFT (12/14, 86%) received appropriate referrals. Future research should explore reasons for differences in screening rates between SSc experts and non-experts. Given that rheumatologists screen for dyspnea more often than they order PFT and TTE, there may be barriers to ordering these tests that warrant further investigation.