Risk assessment in patients with systemic sclerosis and pulmonary arterial hypertension
The current treatment of patients with pulmonary arterial hypertension (PAH) is founded on repeated risk stratification at diagnosis and during follow-up. The 2015 European pulmonary hypertension guidelines propose a multidimensional risk assessment strategy based on nine items and 13 distinct variables in order to predict the estimated 1-year mortality [1, 2]. Recently, several large registry studies have shown that simplified tools can be used to predict outcomes and guide treatment decisions [3–6]. These studies have focused largely on 6-min walking distance (6MWD), World Health Organization (WHO) functional class (FC), right atrial pressure (RAP), cardiac index (CI) and brain natriuretic peptides (BNP or NT-proBNP). Low-risk criteria were defined as WHO FC I or II, 6MWD>440 m, RAP
ConclusionIn predicting one ‐year survival in newly diagnosed SSc‐PAH, the REVEAL prognostic equation and risk score provide very good discrimination but poor calibration. REVEAL prediction scores should be interpreted with caution in newly diagnosed SSc‐PAH patients, particularly those with higher predicted risk resulti ng from a low 6MWD or a high BNP.This article is protected by copyright. All rights reserved.
ConclusionAfrican-Brazilians have distinct characteristics according to clinical subset and an overall more severe SSc than whites, similar to the blacks from other countries.Key Points• African-Brazilian SSc patients were associated with severe interstitial lung disease and nucleolar ANA pattern when compared to white SSc patients.• When disease subsets were considered, African-Brazilian patients with diffuse SSc presented association with pulmonary hypertension, heart involvement, nucleolar ANA pattern, and anti-fibrillarin antibodies.• White SSc patients were associated with centromeric ANA pattern.•...
The objective of this study was to determine the correlation of visual HRCT findings with severity, progression and mortality of SSc-ILD, and specifically whether HRCT findings and lung function measurements provide redundant or complementary measures of SSc-ILD severity and prognosis.
We report the perioperative management of a patient with pulmonary hypertension under new-generation treatments who underwent laparoscopic surgery. Preoperatively, arterial catheter, central venous line, and transesophageal echocardiography probe were inserted in addition to standard monitoring. Intraoperatively, inhaled nitric oxide was used because of increasing pressure in the right heart chambers related to the Trendelenburg position and the pneumoperitoneum. The operation finally lasted
Conclusion Ventilator induced lung injury is characterized by compromised vascular endothelial barrier protection and the production of edemagenic agents in response to mechanical stretch that may lead to overdistention depending upon tidal volume and ventilation frequency (Dos Santos and S.sky, 2000; Lionetti et al., 2005; Birukova et al., 2006). This mechanical-force initiated cellular injury results in cytoskeletal rearrangement. FAs play a central role in mechanotransduction and cytoskeletal rearrangement (De et al., 2010; Ladoux and Nicolas, 2012; Iskratsch et al., 2014; De, 2018). Here, we present the known picture ...
Systemic sclerosis (SSc) is a chronic disease of the connective tissue with an estimated prevalence of 5.8 per 100 000 in a multiethnic population living in Europe . The disease mechanisms are highly complex, affecting multiple organs, including the pulmonary system. Severe pulmonary complications include SSc-associated interstitial lung disease (ILD) and pulmonary hypertension (PH), which are also associated with premature mortality [2, 3]. Early diagnosis and monitoring of pulmonary complications by spirometry, body plethysmography and pulmonary diffusing capacity are critical to lung health in SSc patients [4, 5].
CONCLUSION: Doppler resting and exercise echocardiography may provide a reliable, noninvasive method for determining resting and exercise sPAP, mPAP, and PVR in SSc patients, although it may underestimate or overestimate these values in some individuals. Doppler echocardiography does not replace RHC for definite hemodynamic assessment of suspected PH. PMID: 30974403 [PubMed - as supplied by publisher]
Authors: Rizzi M, Radovanovic D, Airoldi A, Cristiano A, Frassanito F, Gaboardi P, Saad M, Atzeni F, Sarzi-Puttini P, Santus P Abstract Systemic sclerosis (SSc) is an autoimmune disease characterised by tissue fibrosis leading to vascular injury. Nitric oxide (NO) has been implicated in the pathogenesis of autoimmune diseases. A deficiency in basal NO production by the constitutive endothelial isoform of nitric oxide synthase may promote vasoconstriction and vascular wall thickening. In January 2017, we searched the PubMed/Medline, Cochrane Library and Enbase/Medline databases for studies analysing physio-pathologi...
Various etiologies of pulmonary hypertension (PH) and their comorbidities may impact post-lung transplantation (LTx) outcomes. We hypothesized that primary PH, scleroderma PH, and other secondary PH differ in post-tx survival.
ConclusionIn a large SSc ‐ILD cohort, a significant proportion of patients have coexisting PH, which often occurs early after diagnosis; most patients were treated with ILD and PAH therapies, and survival was good. Patients with SSc‐ILD should be evaluated for coexisting PH.This article is protected by copyright. All rights reserved.