MCID: PLM189
MIFTS: 26

Pulmonary Arterial Hypertension Associated with Connective Tissue Disease

Categories: Rare diseases, Respiratory diseases

Aliases & Classifications for Pulmonary Arterial Hypertension Associated with Connective Tissue...

MalaCards integrated aliases for Pulmonary Arterial Hypertension Associated with Connective Tissue Disease:

Name: Pulmonary Arterial Hypertension Associated with Connective Tissue Disease 59 17
Pah Associated with Connective Tissue Disease 59

Characteristics:

Orphanet epidemiological data:

59
pulmonary arterial hypertension associated with connective tissue disease
Inheritance: Not applicable; Prevalence: 1-9/1000000 (France),<1/1000000 (France),1-9/1000000 (Spain),1-9/100000 (United Kingdom),1-9/1000000 (United Kingdom),1-9/1000000 (Czech Republic),1-9/1000000 (Europe); Age of onset: All ages; Age of death: any age;

Classifications:

Orphanet: 59  
Rare respiratory diseases


External Ids:

ICD10 via Orphanet 34 I27.2
Orphanet 59 ORPHA275798

Summaries for Pulmonary Arterial Hypertension Associated with Connective Tissue...

MalaCards based summary : Pulmonary Arterial Hypertension Associated with Connective Tissue Disease, also known as pah associated with connective tissue disease, is related to connective tissue disease and pulmonary hypertension. An important gene associated with Pulmonary Arterial Hypertension Associated with Connective Tissue Disease is HLA-B (Major Histocompatibility Complex, Class I, B), and among its related pathways/superpathways are Calcium signaling pathway and Endothelins. The drugs Tezosentan and Bosentan have been mentioned in the context of this disorder. Affiliated tissues include heart and lung.

Related Diseases for Pulmonary Arterial Hypertension Associated with Connective Tissue...

Graphical network of the top 20 diseases related to Pulmonary Arterial Hypertension Associated with Connective Tissue Disease:



Diseases related to Pulmonary Arterial Hypertension Associated with Connective Tissue Disease

Symptoms & Phenotypes for Pulmonary Arterial Hypertension Associated with Connective Tissue...

Drugs & Therapeutics for Pulmonary Arterial Hypertension Associated with Connective Tissue...

Drugs for Pulmonary Arterial Hypertension Associated with Connective Tissue Disease (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):


# Name Status Phase Clinical Trials Cas Number PubChem Id
1 Tezosentan Investigational Phase 3 180384-57-0
2
Bosentan Approved, Investigational 147536-97-8 104865
3 Antihypertensive Agents
4 Endothelin Receptor Antagonists

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 A Pilot Study to Evaluate the Effect of Bosentan in Patients With Exercise Induced Pulmonary Arterial Hypertension Associated With Connective Tissue Disease Unknown status NCT00864201 Phase 3 bosentan
2 A Phase 3, Randomized, Double-blind, Placebo-controlled Study to Evaluate the Efficacy and Safety of Ralinepag When Added to PAH Standard of Care or PAH Specific Background Therapy in Subjects With WHO Group 1 PAH Recruiting NCT03626688 Phase 3 Ralinepag;Placebo
3 A National, Observational, Multi-center Registry to Examine the Characteristics of Patients With Systematic Sclerosis Digital Ulcers and Assess Bosentan Treatment. Completed NCT02798055 Bosentan group

Search NIH Clinical Center for Pulmonary Arterial Hypertension Associated with Connective Tissue Disease

Genetic Tests for Pulmonary Arterial Hypertension Associated with Connective Tissue...

Anatomical Context for Pulmonary Arterial Hypertension Associated with Connective Tissue...

MalaCards organs/tissues related to Pulmonary Arterial Hypertension Associated with Connective Tissue Disease:

41
Heart, Lung

Publications for Pulmonary Arterial Hypertension Associated with Connective Tissue...

Articles related to Pulmonary Arterial Hypertension Associated with Connective Tissue Disease:

(show top 50) (show all 63)
# Title Authors PMID Year
1
Simplified risk stratification for pulmonary arterial hypertension associated with connective tissue disease. 38
31385084 2019
2
Acute Hemodynamic Changes after Single Administration of Udenafil in Pulmonary Arterial Hypertension: a Phase IIa Study. 38
30808080 2019
3
Lung Disease in Antiphospholipid Syndrome. 38
31137066 2019
4
2018 TSOC guideline focused update on diagnosis and treatment of pulmonary arterial hypertension. 38
30926248 2019
5
Safety and feasibility audit of a home-based drug-transitioning approach for patients with pulmonary arterial hypertension: an observational study. 38
29641223 2018
6
The prognosis of pulmonary arterial hypertension associated with primary Sjögren's syndrome: a cohort study. 38
29419347 2018
7
Predictors of Favorable Responses to Immunosuppressive Treatment in Pulmonary Arterial Hypertension Associated With Connective Tissue Disease. 38
28904255 2018
8
Acute Iloprost Inhalation Improves Right Ventricle Function in Pulmonary Artery Hypertension: A Cardiac Magnetic Resonance Study. 38
30719004 2018
9
[Pulmonary hypertension in the elderly]. 38
28976395 2017
10
Pulmonary hypertension associated with antiphospholipid antibody: Call for a screening tool? 38
28944137 2017
11
Mechanism and prognostic role of qR in V1 in patients with pulmonary arterial hypertension. 38
28256215 2017
12
Replacing a phosphodiesterase-5 inhibitor with riociguat in patients with connective tissue disease-associated pulmonary arterial hypertension: a case series. 38
28671485 2017
13
Pulmonary arterial hypertension associated with connective tissue disease: Insights from Latvian PAH registry. 38
28110845 2017
14
Tadalafil in idiopathic or heritable pulmonary arterial hypertension (PAH) compared to PAH associated with connective tissue disease. 38
28279499 2017
15
Ambrisentan use for pulmonary arterial hypertension in a post-authorization drug registry: The VOLibris Tracking Study. 38
27282418 2017
16
Long-term outcomes of domiciliary intravenous iloprost in idiopathic and connective tissue disease-associated pulmonary arterial hypertension. 38
27651181 2017
17
Riociguat for the treatment of pulmonary arterial hypertension associated with connective tissue disease: results from PATENT-1 and PATENT-2. 38
27457511 2017
18
Biomarkers of tissue remodeling predict survival in patients with pulmonary hypertension. 38
27575785 2016
19
The Management of Supraventricular Tachyarrhythmias in Patients with Pulmonary Arterial Hypertension. 38
26643289 2016
20
Galectin-3 and aldosterone as potential tandem biomarkers in pulmonary arterial hypertension. 38
26869635 2016
21
[Value of intravascular ultrasound in the assessment of pulmonary vascular properties and mortality in patients with pulmonary artery hypertension associated with connective tissue diseases]. 38
26888841 2015
22
Combining bosentan and sildenafil in pulmonary arterial hypertension patients failing monotherapy: real-world insights. 38
26022952 2015
23
Echocardiographic Evaluation of the Relationship Between inflammatory factors (IL6, TNF╬▒, hs-CRP) and Secondary Pulmonary Hypertension in patients with COPD. A Cross sectional study. 38
26738368 2015
24
Associations Between Sildenafil Use and Changes in Days of Hospitalization in a Population With Pulmonary Arterial Hypertension Associated With Connective Tissue Disease. 38
25818118 2015
25
Efficacy and Safety of Long-Term Imatinib Therapy for Pulmonary Arterial Hypertension. 38
26043786 2015
26
Rapid initiation of intravenous epoprostenol infusion is the favored option in patients with advanced pulmonary arterial hypertension. 38
25844932 2015
27
Epoprostenol Therapy for Pulmonary Arterial Hypertension. 38
26101188 2015
28
Effect of pulmonary arterial hypertension-specific therapies on health-related quality of life: a systematic review. 38
24626791 2014
29
Epidemiology and long-term survival of pulmonary arterial hypertension in the Czech Republic: a retrospective analysis of a nationwide registry. 38
24629043 2014
30
Long-term results from the EARLY study of bosentan in WHO functional class II pulmonary arterial hypertension patients. 38
24525158 2014
31
Intravenous epoprostenol treatment of patients with connective tissue disease and pulmonary arterial hypertension at a single center. 38
23359006 2013
32
Pulmonary arterial hypertension associated with connective tissue disease: meta-analysis of clinical trials. 38
23906950 2013
33
Pulmonary hypertension complicated by pericardial effusion: a single center experience. 38
23258502 2013
34
Double-blind, placebo-controlled clinical trial with a rho-kinase inhibitor in pulmonary arterial hypertension. 38
23912836 2013
35
Sex differences in the diagnosis, treatment, and outcome of patients with pulmonary arterial hypertension enrolled in the registry to evaluate early and long-term pulmonary arterial hypertension disease management. 38
21757572 2012
36
Pulmonary hypertension associated with veno-occlusive disease in systemic sclerosis: Insight into the mechanism of resistance to vasodilator. 38
30532900 2012
37
Ocular safety of sildenafil citrate when administered chronically for pulmonary arterial hypertension: results from phase III, randomised, double masked, placebo controlled trial and open label extension. 38
22354598 2012
38
Pulmonary arterial dilatation in pulmonary hypertension: prevalence and prognostic relevance. 38
22433455 2012
39
Human pentraxin 3 (PTX3) as a novel biomarker for the diagnosis of pulmonary arterial hypertension. 38
23029266 2012
40
Frequency of pleural effusions in patients with pulmonary arterial hypertension associated with connective tissue diseases. 38
21212140 2011
41
Enlarged right-sided dimensions and fibrosis of the right ventricular insertion point on cardiovascular magnetic resonance imaging is seen early in patients with pulmonary arterial hypertension associated with connective tissue disease. 38
20919946 2011
42
Intensive immunosuppressive therapy improves pulmonary hemodynamics and long-term prognosis in patients with pulmonary arterial hypertension associated with connective tissue disease. 38
21873802 2011
43
Pulmonary arterial hypertension associated with connective tissue disease and immunosuppressive therapy. 38
21963571 2011
44
Management of pulmonary arterial hypertension in children. 38
21109183 2010
45
Long-term outcomes in children with pulmonary arterial hypertension treated with bosentan in real-world clinical settings. 38
21029834 2010
46
Predicting survival in pulmonary arterial hypertension: insights from the Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL). 38
20585012 2010
47
Assessment of endpoints in pulmonary arterial hypertension associated with connective tissue disease. 38
20375662 2010
48
Acute vasodilator effects of inhaled fasudil, a specific Rho-kinase inhibitor, in patients with pulmonary arterial hypertension. 38
20339976 2010
49
Characteristics and prospective 2-year follow-up of children with pulmonary arterial hypertension in France. 38
20226425 2010
50
The cost-effectiveness of bosentan in the United Kingdom for patients with pulmonary arterial hypertension of WHO functional class III. 38
19558373 2009

Variations for Pulmonary Arterial Hypertension Associated with Connective Tissue...

Expression for Pulmonary Arterial Hypertension Associated with Connective Tissue...

Search GEO for disease gene expression data for Pulmonary Arterial Hypertension Associated with Connective Tissue Disease.

Pathways for Pulmonary Arterial Hypertension Associated with Connective Tissue...

Pathways related to Pulmonary Arterial Hypertension Associated with Connective Tissue Disease according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1
Show member pathways
11.55 EDNRB EDNRA
2 10.88 EDNRB EDNRA
3 10.49 EDNRB EDNRA
4 10.1 EDNRB EDNRA

GO Terms for Pulmonary Arterial Hypertension Associated with Connective Tissue...

Cellular components related to Pulmonary Arterial Hypertension Associated with Connective Tissue Disease according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 integral component of plasma membrane GO:0005887 8.8 HLA-B EDNRB EDNRA

Biological processes related to Pulmonary Arterial Hypertension Associated with Connective Tissue Disease according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 positive regulation of cytosolic calcium ion concentration GO:0007204 9.32 EDNRB EDNRA
2 regulation of blood pressure GO:0008217 9.26 EDNRB EDNRA
3 vasoconstriction GO:0042310 9.16 EDNRB EDNRA
4 enteric nervous system development GO:0048484 8.96 EDNRB EDNRA
5 endothelin receptor signaling pathway GO:0086100 8.62 EDNRB EDNRA

Molecular functions related to Pulmonary Arterial Hypertension Associated with Connective Tissue Disease according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 endothelin receptor activity GO:0004962 8.62 EDNRB EDNRA

Sources for Pulmonary Arterial Hypertension Associated with Connective Tissue...

3 CDC
7 CNVD
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10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
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33 ICD10
34 ICD10 via Orphanet
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57 OMIM
58 OMIM via Orphanet
62 PubMed
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69 SNOMED-CT via HPO
70 TGDB
71 Tocris
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73 UMLS via Orphanet
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