Systemische Sklerose - eine systematische Übersicht

VASA ◽  
2011 ◽  
Vol 40 (1) ◽  
pp. 6-19 ◽  
Author(s):  
Klein-Weigel ◽  
Opitz ◽  
Riemekasten
Keyword(s):  
Pulmonary Hypertension ◽  
Systemic Sclerosis ◽  
Task Force ◽  
Systemische Sklerose ◽  
Systematische Übersicht ◽  
European Respiratory Society ◽  
High Association ◽  
Microscopic Features ◽  
European Society Of Cardiology ◽  
Genetic Burden

Due to its high association with Raynaud’s phenomenon systemic sclerosis (SSc) is probably the most common connective tissue disease seen by vascular specialists. In part 1 of our systematic overview we summarize classification concepts of scleroderma disorders, the epidemiologic and genetic burden, the complex pathophysiologic background, and the clinical features and the stage-dependent capillary microscopic features of SSc. Furthermore, we address the diagnostic recommendations propagated by the German Network for Systemic Sclerosis and the Task Force for Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology, the European Respiratory Society, and the International Society of Heart and Lung Transplantation.

Conclusion

2018 ◽  
Author(s):  
Marc Humbert
Keyword(s):  
Pulmonary Hypertension ◽  
European Society ◽  
Hypertension Management ◽  
Evidence Based ◽  
European Respiratory Society ◽  
Fast Growing ◽  
Recent Advances ◽  
European Society Of Cardiology ◽  
Future Work

Pulmonary hypertension is a fast-growing field in cardiopulmonary medicine. Thanks to recent advances summarized in the 2015 European Society of Cardiology/European Respiratory Society guidelines, evidence-based pulmonary hypertension management can now be provided to all pulmonary hypertension patients. There are, however, important gaps of evidence which should be clarified in future work.

scholarly journals Pulmonary endarterectomy in the management of chronic thromboembolic pulmonary hypertension

2017 ◽  
Vol 26 (143) ◽  
pp. 160111 ◽  
Author(s):  
David Jenkins ◽  
Michael Madani ◽  
Elie Fadel ◽  
Andrea Maria D'Armini ◽  
Eckhard Mayer
Keyword(s):  
Pulmonary Hypertension ◽  
Treatment Algorithm ◽  
Pulmonary Arteries ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Endarterectomy ◽  
European Respiratory Society ◽  
Complex Procedure ◽  
Thromboembolic Pulmonary Hypertension ◽  
Definition Of ◽  
European Society Of Cardiology

Chronic thromboembolic pulmonary hypertension (CTEPH) is a type of pulmonary hypertension, resulting from fibrotic transformation of pulmonary artery clots causing chronic obstruction in macroscopic pulmonary arteries and associated vascular remodelling in the microvasculature.Pulmonary endarterectomy (PEA) offers the best chance of symptomatic and prognostic improvement in eligible patients; in expert centres, it has excellent results. Current in-hospital mortality rates are <5% and survival is >90% at 1 year and >70% at 10 years. However, PEA, is a complex procedure and relies on a multidisciplinary CTEPH team led by an experienced surgeon to decide on an individual's operability, which is determined primarily by lesion location and the haemodynamic parameters. Therefore, treatment of patients with CTEPH depends largely on subjective judgements of eligibility for surgery by the CTEPH team.Other controversies discussed in this article include eligibility for PEA versus balloon pulmonary angioplasty, the new treatment algorithm in the European Society of Cardiology/European Respiratory Society guidelines and the definition of an “expert centre” for the management of this condition.

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.

scholarly journals Echocardiographic Probability of Pulmonary Hypertension in Myeloproliferative Neoplasms Patients in Chiang-Mai University

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 5390-5390
Author(s):  
Yanika Jindamai ◽  
Ekarat Rattarittamrong ◽  
Arintaya Phrommintikul ◽  
Lalita Yongsmith ◽  
Thanawat Rattanathammethee ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Conflicts Of Interest ◽  
Task Force ◽  
Myeloproliferative Neoplasms ◽  
White Blood Cells ◽  
Philadelphia Chromosome ◽  
University Hospital ◽  
Chiang Mai ◽  
European Respiratory Society ◽  
Number Of Patients

Introduction: Pulmonary hypertension (PHT) is an emerging complication of myeloproliferative neoplasms (MPNs). The aim of this study was to determine the prevalence and risk factors of high echocardiographic probability of PHT according to 2015 The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS) Guideline. The echocardiographic probability of PHT was assessed by peak tricuspid regurgitation velocity (TRV max) and echocardiographic signs suggesting PHT. Methods: This was a cross-sectional study conducted in Chiang-Mai University Hospital during January 2019 and July 2019. Patients aged 18 years or older with Philadelphia chromosome negative MPNs including polycythemia vera (PV), essential thrombocythemia (ET), and myelofibrosis (MF) were eligible after obtaining informed consents. Patients who were known to have a preexisting PHT from any causes were excluded. All enrolled patients had an echocardiography performed by a cardiologist for evaluating the probability of PHT according to 2015 ESC/ERS guideline. Primary outcome of the study was the prevalence of MPNs patients who had high echocardiographic probability of PHT. Secondary outcomes were risk factor of high echocardiographic probability of PHT. Results: A total of 23 patients with a median age of 64 years (range 45-87) were enrolled. There were 14 PV (60.9%), 8 ET (34.8%), 1 MF (4.3%) patients included. There were13 male patients (56.5%). The most common driver mutation was JAK2V617F mutation (19 patients, 82.6%). Common co-morbid diseases were hypertension (26.1%), combined hypertension and dyslipidemia (13%), and combined hypertension and diabetes (8.7%), respectively. The majority of patients (73.9%) did not have history of thrombosis. However, 57.2% and 62.5% of PV and ET patients were classified as high risk of thrombosis. Twenty two patients (95.6%) received both antiplatelets and cytoreductive agents with 9 patients (39.1%) also underwent phlebotomy. Hydroxyurea was only cytoreductive drug prescribed in this study. The median time from diagnosis to echocardiogram evaluation was 66 months (range 9-6,242 months). Median (range) hemoglobin was 11.7 g/dl (9.7-16.8), median white blood cells count was 7.5x109/L (4.0-24.1), and median platelet count was 374x109/L (171-931). No patient with high echocardiographic probability of PHT was detected. One patient (4.3%) patient had intermediate probability and 22 (95.7%) patients had low probability. The median value of TRV max was 2.42 m/s (range 1.93-2.90). Conclusions: No MPNs patients (0/23) with high echocardiographic probability of PHT detected in this study. Further study with higher number of patients is warrant for determine the prevalence of PHT in Thai MPN patients. Table Disclosures No relevant conflicts of interest to declare.

Systemische Sklerose - eine systematische Übersicht

VASA ◽  
2011 ◽  
Vol 40 (1) ◽  
pp. 20-30 ◽  
Author(s):  
Opitz ◽  
Klein-Weigel ◽  
Riemekasten
Keyword(s):  
Connective Tissue ◽  
Systemische Sklerose ◽  
Systematische Übersicht ◽  
Scleroderma Renal Crisis ◽  
Digital Ulcers ◽  
European Respiratory Society ◽  
First Line Therapy ◽  
European League Against Rheumatism ◽  
Therapeutic Value ◽  
Pulmonary Arterial

Here we give an overview over treatment recommendations propagated by the European League Against Rheumatism (EULAR), EULAR Scleroderma Trials and Research Group, the German Network for Systemic Sclerosis, the European Respiratory Society, and the International Society of Heart and Lung Transplantation. As response to immunosuppressant (IS) therapy is usually weaker in systematic sclerosis (SSc) compared to other connective tissue disorders IS should be considered with caution. To prevent scleroderma renal crisis steroid doses should not exceed 15 mg/d. The definitive role of a number of new immunosuppressant drugs and the effects of autologous stem cell transplantation in systemic clerosis (SSc) have to be elucidated. Prostanoids, especially iloprost, are widely used as intravenous formulas for the treatment of severe Raynaud’s phenomenon (RP) and digital ulcers (DU). Calcium antagonists are of limited therapeutic value. Bosentan, an oral endothelin receptor antagonists (ETRA), was shown to prevent new DU, but failed to heal existing DU, while the oral phopshodiesterase inhibitor (PDI) Sildenafil reduces the occurrence of RP and might be effective in ulcer healing. Combination therapies of PDI with ETRA are currently evaluated. Therapy of pulmonary arterial hypertension (PAH) is usually started as oral monotherapy, frequently using an ETRA. When this first-line therapy is not tolerated ETRA is substituted by PDI. If treatment goals are not reached with monotherapy combinationtherapy is started, for example by adding a PDI to an existing ETRA. In general, treatment of PAH in patients with connective tissue disease follows the same algorithms as in idiopathic PAH.

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