Pulmonary arterial hypertension in systemic sclerosis: Diagnosis and treatment according to the European Society of Cardiology and European Respiratory Society 2015 guidelines

2018 ◽  
Vol 4 (1) ◽  
pp. 35-42 ◽  
Author(s):  
Nicola Giordano ◽  
Claudio Corallo ◽  
Chiara Chirico ◽  
Angelica Brazzi ◽  
Adriana Marinetti ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Systemic Sclerosis ◽  
Pulmonary Arterial Hypertension ◽  
Endothelial Dysfunction ◽  
Arterial Hypertension ◽  
Correct Diagnosis ◽  
European Respiratory Society ◽  
Long Term Outcome ◽  
Screening Programs ◽  
Pulmonary Arterial

Scleroderma (systemic sclerosis) is an autoimmune connective tissue disease which presents endothelial dysfunction and fibroblast dysregulation, resulting in vascular and fibrotic disorders. Pulmonary hypertension is frequent in patients with systemic sclerosis: the natural evolution of the disease can induce the development of different forms of pulmonary hypertension, representing one of the main causes of death. Among the different forms of pulmonary hypertension in systemic sclerosis, pulmonary arterial hypertension is the most frequent one (rate of occurrence is estimated between 7% and 12%). This pulmonary vascular complication should be treated with a combination of drugs that is able to counteract endothelial dysfunction, antagonizing the endothelin-1 system and replacing prostaglandin I2 and nitric oxide activity. A correct diagnosis is mandatory, because it is possible only for pulmonary arterial hypertension to use specific drugs that are able to control the symptomatic condition and the evolution of the disease. According to the most recent guidelines, for the patients with systemic sclerosis, also without pulmonary hypertension symptoms, echocardiography screening for the detection of pulmonary hypertension is recommended. Pulmonary arterial hypertension screening programs in systemic sclerosis patients is able to identify milder forms of the disease, allowing earlier management and better long-term outcome.

Prevalence and incidence of pulmonary arterial hypertension: 10-year follow-up of an unselected systemic sclerosis cohort

2017 ◽  
Vol 2 (3) ◽  
pp. 196-202 ◽  
Author(s):  
Els Vandecasteele ◽  
Karin Melsens ◽  
Kristof Thevissen ◽  
Michel De Pauw ◽  
Ellen Deschepper ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Incidence Rate ◽  
Specific Treatment ◽  
Early Screening ◽  
European Respiratory Society ◽  
Pulmonary Arterial ◽  
Incident Cases

Introduction Early screening for pulmonary arterial hypertension (PAH) in systemic sclerosis (SSc) improves outcome. Therefore, we evaluated the screening for PAH during the 10-year follow-up of an unselected prospective SSc cohort by calculating the prevalence and the incidence rate of PAH and we compared the screening before and after implementation of the 2009 European Society of Cardiology/European Respiratory Society (ESC/ERS) guidelines. Methods Data were evaluated from each SSc-specific visit of 362 consecutive SSc patients included in the SSc Cohort of the Ghent University between May 2006 and December 2015. Results Of the 362 included patients, 23.2% had limited SSc, 59.9% limited cutaneous SSc and 16.9% diffuse cutaneous SSc. At baseline, one patient was already on PAH-specific treatment and eight patients were diagnosed with PAH, implicating a baseline PAH prevalence of 2.5% (9/362). During follow-up (median of 18 months [interquartile range: 0-54 months]), nine patients were diagnosed with incidental PAH, resulting in an incidence rate of 9.3/1000 person-years, 95% confidence intervals (95% CI): 4.3-17.7. Before the ESC/ERS guidelines, five PAH patients, all already diagnosed with prevalent PAH, were included in the cohort. After 2009, 13 patients (4 prevalent cases) were diagnosed with PAH, making the yearly incidence around 1% (0.82%-2.00%). Conclusions During 10-year follow-up in a cohort of 362 unselected SSc patients, the cumulative prevalence of PAH is 5% (18/362) and the incidence rate 9.3/1000 person-years, 95% CI: 4.3-17.7. Before implementation of the 2009 ESC/ERS screening algorithm, there were no incident cases.

scholarly journals The FGL2 prothrombinase contributes to the pathological process of experimental pulmonary hypertension

2019 ◽  
Vol 127 (6) ◽  
pp. 1677-1687
Author(s):  
Cheng Fan ◽  
Jue Wang ◽  
Chaoqin Mao ◽  
Wenzhu Li ◽  
Kun Liu ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Endothelial Dysfunction ◽  
Arterial Hypertension ◽  
Vascular Remodeling ◽  
Thrombus Formation ◽  
Idiopathic Pulmonary Arterial Hypertension ◽  
Potential Therapeutic Target ◽  
Pulmonary Arterial

In situ thrombus formation is one of the major pathological features of pulmonary hypertension (PH). The mechanism of in situ thrombosis has not been clearly identified. Fibrinogen-like protein 2 (FGL2) prothrombinase is an immune coagulant that can cleave prothrombin to thrombin, which then converts fibrinogen into fibrin. This mechanism triggers in situ thrombus formation directly, bypassing both the intrinsic and extrinsic coagulation pathways. FGL2 prothrombinase is mainly expressed in endothelial cells and mediates multiple pathological processes. This implies that it may also play a role in PH. In this study, we examined the expression of FGL2 in idiopathic pulmonary arterial hypertension (IPAH) patients, and in monocrotaline-induced rat and hypoxia-induced mouse PH models. Fgl2−/− mice were used to evaluate the development of PH and explore associated pathological changes. These included in situ thrombosis, vascular remodeling, and endothelial apoptosis. Following these analyses, we examined possible signaling pathways downstream of FGL2 in PH. We show FGL2 is upregulated in pulmonary vascular endothelium in human IPAH and in two animal PH models. Genetic knockout of Fgl2 limited the development of PH, indicated by decreased in situ thrombus formation, less vascular remodeling, and reduced endothelial dysfunction. In addition, loss of FGL2 downregulated PAR1 (proteinase-activated receptor 1) expression and decreased the overactivation and consumption of platelets in hypoxia-induced PH. These results indicate FGL2 participate in the development of PH and loss of FGL2 could attenuate PH by reducing in situ thrombosis and suppressing PAR1 signaling. Thus we provide evidence that suggests FGL2 prothrombinase presents a potential therapeutic target for clinical treatment of PH. NEW & NOTEWORTHY This is the first study to demonstrate that fibrinogen-like protein 2 (FGL2) participates in the pathological progression of pulmonary hypertension (PH) in human idiopathic pulmonary arterial hypertension, a monocrotaline rat PH model, and a hypoxia mouse PH model. Genetic knockout of Fgl2 significantly limited the development of PH indicated by reduced in situ thrombosis, vascular remodeling, and endothelial dysfunction, and suppressed PAR1 (proteinase-activated receptor 1) signaling and overactivation of platelets on PH. These results suggest FGL2 presents a potential therapeutic target for clinical treatment of PH.

Screening for pulmonary arterial hypertension

ESC CardioMed ◽  
2018 ◽  
pp. 2506-2507
Author(s):  
John Coghlan
Keyword(s):  
Pulmonary Hypertension ◽  
Portal Hypertension ◽  
Systemic Sclerosis ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Diagnostic Tools ◽  
Primary Screening ◽  
Class 1 ◽  
Pulmonary Arterial ◽  
Low Prevalence

Screening programmes for pulmonary hypertension are justifiable in some circumstances but not others. The inherent inaccuracies of the diagnostic tools and the low prevalence of pulmonary hypertension renders screening programmes ineffective unless the population evaluated with the primary screening tool (echocardiography) can be enriched. Nevertheless, significant progress has been made over the past 5 years and screening programmes are now strongly recommended (class 1 recommendation) in asymptomatic systemic sclerosis, BMPR2 mutation carriers, first-degree relatives of patients with hereditable pulmonary hypertension, and patients with portal hypertension referred for transplantation.

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