Systemic Sclerosis-Associated Pulmonary Hypertension: Spectrum and Impact
20 May 2021-Vol. 11, Iss: 5, pp 911
TL;DR: In this paper, the authors proposed a screening modality and risk assessment to identify patients early in their disease course and promptly refer them to expert centers for a hemodynamic assessment and formal diagnosis of systemic sclerosis-associated pulmonary arterial hypertension (SSc-PAH).
Abstract: Systemic sclerosis-associated pulmonary arterial hypertension (SSc-PAH) is a catastrophic complication of one of the most common and devastating autoimmune diseases. Once diagnosed, it becomes the leading cause of mortality among this patient population. Screening modalities and risk assessments have been designed and validated by various organizations and societies in order to identify patients early in their disease course and promptly refer them to expert centers for a hemodynamic assessment and formal diagnosis. Moreover, several large multicenter clinical trials have now included patients with SSc-PAH to assess their response to therapy. Despite an improved understanding of the condition and significant advances in supportive and targeted therapy, outcomes have remained far from optimal. Therefore, rigorous phenotyping and search for novel therapies are desperately needed for this devastating condition.
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TL;DR: In this paper, a revision actualizada de las principales manifestaciones cardiacas de las enfermedades reumaticas, asi como su manejo terapeutico.
Abstract: Resumen Las enfermedades reumaticas autoinmunes son trastornos inflamatorios que pueden afectar a multiples organos, entre los que se incluye el corazon El elevado riesgo de aparicion de patologia cardiovascular en estos pacientes no solo se debe a la presencia de factores de riesgo cardiovascular tradicionales, sino tambien a la inflamacion cronica y la autoinmunidad Todas las estructuras cardiacas pueden verse afectadas durante el curso de la enfermedad (valvulas, sistema de conduccion, miocardio, endocardio, pericardio y arterias coronarias), por lo que las complicaciones cardiacas incluyen una amplia variedad de manifestaciones clinicas La afectacion cardiaca se asocia con un pronostico desfavorable, por lo que es esencial detectar la lesion cardiaca subclinica en pacientes asintomaticos e instaurar el tratamiento de forma precoz Este documento ofrece una revision actualizada de las principales manifestaciones cardiacas de las enfermedades reumaticas, asi como su manejo terapeutico
2 citations
09 Jun 2021
TL;DR: The contents of this special issue of Diagnostics dedicated to Diagnosis and Treatment of Pulmonary Hypertension (PH) are described in detail in this article and discussed in detail.
Abstract: With great satisfaction, we now share with you the contents of this Special Issue of Diagnostics dedicated to Diagnosis and Treatment of Pulmonary Hypertension (PH) [...]
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TL;DR: In this paper, the authors defined the following terms: ALAT, alanine aminotransferase, ASAT, aspartate AMINOTE, and APAH, associated pulmonary arterial hypertension.
Abstract: ALAT
: alanine aminotransferase
ASAT
: aspartate aminotransferase
APAH
: associated pulmonary arterial hypertension
BAS
: balloon atrial septostomy
BMPR2
: bone morphogenetic protein receptor 2
BNP
: brain natriuretic peptide
BPA
: balloon pulmonary angioplasty
BREATHE
: Bosentan
5,224 citations
TL;DR: The endothelin-receptor antagonist bosentan is beneficial in patients with pulmonary arterial hypertension and is well tolerated at a dose of 125 mg twice daily.
Abstract: Methods In this double-blind, placebo-controlled study, we randomly assigned 213 patients with pulmonary arterial hypertension (primary or associated with connective-tissue disease) to receive placebo or to receive 62.5 mg of bosentan twice daily for 4 weeks followed by either of two doses of bosentan (125 or 250 mg twice daily) for a minimum of 12 weeks. The primary end point was the degree of change in exercise capacity. Secondary end points included the change in the Borg dyspnea index, the change in the World Health Organization (WHO) functional class, and the time to clinical worsening. Results At week 16, patients treated with bosentan had an improved six-minute walking distance; the mean difference between the placebo group and the combined bosentan groups was 44 m (95 percent confidence interval, 21 to 67; P<0.001). Bosentan also improved the Borg dyspnea index and WHO functional class and increased the time to clinical worsening. Conclusions The endothelin-receptor antagonist bosentan is beneficial in patients with pulmonary arterial hypertension and is well tolerated at a dose of 125 mg twice daily. Endothelin-receptor antagonism with oral bosentan is an effective approach to therapy for pulmonary arterial hypertension. (N Engl J Med
2,443 citations
TL;DR: The 6th WSPH Task Force proposed to include pulmonary vascular resistance ≥3 Wood Units in the definition of all forms of pre-capillary PH associated with mPAP >20 mmHg, and included in group 1 of a subgroup “pulmonary arterial hypertension (PAH) long-term responders to calcium channel blockers”.
Abstract: Since the 1st World Symposium on Pulmonary Hypertension (WSPH) in 1973, pulmonary hypertension (PH) has been arbitrarily defined as mean pulmonary arterial pressure (mPAP) ≥25 mmHg at rest, measured by right heart catheterisation. Recent data from normal subjects has shown that normal mPAP was 14.0±3.3 mmHg. Two standard deviations above this mean value would suggest mPAP >20 mmHg as above the upper limit of normal (above the 97.5th percentile). This definition is no longer arbitrary, but based on a scientific approach. However, this abnormal elevation of mPAP is not sufficient to define pulmonary vascular disease as it can be due to an increase in cardiac output or pulmonary arterial wedge pressure. Thus, this 6th WSPH Task Force proposes to include pulmonary vascular resistance ≥3 Wood Units in the definition of all forms of pre-capillary PH associated with mPAP >20 mmHg. Prospective trials are required to determine whether this PH population might benefit from specific management. Regarding clinical classification, the main Task Force changes were the inclusion in group 1 of a subgroup “pulmonary arterial hypertension (PAH) long-term responders to calcium channel blockers”, due to the specific prognostic and management of these patients, and a subgroup “PAH with overt features of venous/capillaries (pulmonary veno-occlusive disease/pulmonary capillary haemangiomatosis) involvement”, due to evidence suggesting a continuum between arterial, capillary and vein involvement in PAH.
2,358 citations
TL;DR: Among the 222 patients completing one year of treatment with sildenafil monotherapy, the improvement from baseline at one year in the distance walked in six minutes was 51 m, and the incidence of clinical worsening did not differ significantly between the patients treated with s Bildenafils and those treated with placebo.
Abstract: background Sildenafil inhibits phosphodiesterase type 5, an enzyme that metabolizes cyclic guanosine monophosphate, thereby enhancing the cyclic guanosine monophosphate– mediated relaxation and growth inhibition of vascular smooth-muscle cells, including those in the lung. methods In this double-blind, placebo-controlled study, we randomly assigned 278 patients with symptomatic pulmonary arterial hypertension (either idiopathic or associated with connective-tissue disease or with repaired congenital systemic-to-pulmonary shunts) to placebo or sildenafil (20, 40, or 80 mg) orally three times daily for 12 weeks. The primary end point was the change from baseline to week 12 in the distance walked in six minutes. The change in mean pulmonary-artery pressure and World Health Organization (WHO) functional class and the incidence of clinical worsening were also assessed, but the study was not powered to assess mortality. Patients completing the 12-week randomized study could enter a long-term extension study. results The distance walked in six minutes increased from baseline in all sildenafil groups; the mean placebo-corrected treatment effects were 45 m (+13.0 percent), 46 m (+13.3 percent), and 50 m (+14.7 percent) for 20, 40, and 80 mg of sildenafil, respectively (P<0.001 for all comparisons). All sildenafil doses reduced the mean pulmonary-artery pressure (P = 0.04, P = 0.01, and P<0.001, respectively), improved the WHO functional class (P = 0.003, P<0.001, and P<0.001, respectively), and were associated with side effects such as flushing, dyspepsia, and diarrhea. The incidence of clinical worsening did not differ significantly between the patients treated with sildenafil and those treated with placebo. Among the 222 patients completing one year of treatment with sildenafil monotherapy, the improvement from baseline at one year in the distance walked in six minutes was 51 m. conclusions Sildenafil improves exercise capacity, WHO functional class, and hemodynamics in patients with symptomatic pulmonary arterial hypertension.
2,211 citations
TL;DR: It is shown that PAH is detected late in the course of the disease, with a majority of patients displaying severe functional and hemodynamic compromise, and this contemporary registry highlights current practice.
Abstract: Rationale: Pulmonary arterial hypertension (PAH) is an orphan disease for which the trend is for management in designated centers with multidisciplinary teams working in a shared-care approach.Objective: To describe clinical and hemodynamic parameters and to provide estimates for the prevalence of patients diagnosed for PAH according to a standardized definition.Methods: The registry was initiated in 17 university hospitals following at least five newly diagnosed patients per year. All consecutive adult (⩾ 18 yr) patients seen between October 2002 and October 2003 were to be included.Main Results: A total of 674 patients (mean ± SD age, 50 ± 15 yr; range, 18–85 yr) were entered in the registry. Idiopathic, familial, anorexigen, connective tissue diseases, congenital heart diseases, portal hypertension, and HIV-associated PAH accounted for 39.2, 3.9, 9.5, 15.3, 11.3, 10.4, and 6.2% of the population, respectively. At diagnosis, 75% of patients were in New York Heart Association functional class III or IV. ...
1,759 citations