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Associated pulmonary arterial hypertension

MedGen UID:
473476
•Concept ID:
C1701938
•
Disease or Syndrome
Synonym: Associated Pulmonary Arterial Hypertension
SNOMED CT: Associated pulmonary arterial hypertension (697902002)

Definition

Pulmonary hypertension that results from another disorder (e.g., connective tissue disorder). [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAssociated pulmonary arterial hypertension

Professional guidelines

PubMed

Safety and Efficacy of B-Cell Depletion with Rituximab for the Treatment of Systemic Sclerosis-associated Pulmonary Arterial Hypertension: A Multicenter, Double-Blind, Randomized, Placebo-controlled Trial.
Zamanian RT, Badesch D, Chung L, Domsic RT, Medsger T, Pinckney A, Keyes-Elstein L, D'Aveta C, Spychala M, White RJ, Hassoun PM, Torres F, Sweatt AJ, Molitor JA, Khanna D, Maecker H, Welch B, Goldmuntz E, Nicolls MR
Am J Respir Crit Care Med 2021 Jul 15;204(2):209-221. doi: 10.1164/rccm.202009-3481OC. PMID: 33651671Free PMC Article
Updated treatment algorithm of pulmonary arterial hypertension.
Galiè N, Corris PA, Frost A, Girgis RE, Granton J, Jing ZC, Klepetko W, McGoon MD, McLaughlin VV, Preston IR, Rubin LJ, Sandoval J, Seeger W, Keogh A
J Am Coll Cardiol 2013 Dec 24;62(25 Suppl):D60-72. doi: 10.1016/j.jacc.2013.10.031. PMID: 24355643
An update on the evaluation and management of pulmonary hypertension in scleroderma.
Coghlan JG, Schreiber B
Curr Rheumatol Rep 2012 Feb;14(1):1-10. doi: 10.1007/s11926-011-0226-2. PMID: 22215445

Recent clinical studies

Etiology

The DNA Damage Response and HIV-Associated Pulmonary Arterial Hypertension.
Simenauer A, Nozik-Grayck E, Cota-Gomez A
Int J Mol Sci 2020 May 7;21(9) doi: 10.3390/ijms21093305. PMID: 32392789Free PMC Article
Methamphetamine and the risk of pulmonary arterial hypertension.
Ramirez RL 3rd, Perez VJ, Zamanian RT
Curr Opin Pulm Med 2018 Sep;24(5):416-424. doi: 10.1097/MCP.0000000000000513. PMID: 30036313Free PMC Article
Drug-associated pulmonary arterial hypertension.
McGee M, Whitehead N, Martin J, Collins N
Clin Toxicol (Phila) 2018 Sep;56(9):801-809. Epub 2018 Mar 6 doi: 10.1080/15563650.2018.1447119. PMID: 29508628
Features and Outcomes of Methamphetamine-associated Pulmonary Arterial Hypertension.
Zamanian RT, Hedlin H, Greuenwald P, Wilson DM, Segal JI, Jorden M, Kudelko K, Liu J, Hsi A, Rupp A, Sweatt AJ, Tuder R, Berry GJ, Rabinovitch M, Doyle RL, de Jesus Perez V, Kawut SM
Am J Respir Crit Care Med 2018 Mar 15;197(6):788-800. doi: 10.1164/rccm.201705-0943OC. PMID: 28934596Free PMC Article
Systemic sclerosis-associated pulmonary arterial hypertension.
Chaisson NF, Hassoun PM
Chest 2013 Oct;144(4):1346-1356. doi: 10.1378/chest.12-2396. PMID: 24081346Free PMC Article

Diagnosis

Biomarkers of haemodynamic severity of systemic sclerosis-associated pulmonary arterial hypertension by serum proteome analysis.
Sanges S, Rice L, Tu L, Valenzi E, Cracowski JL, Montani D, Mantero JC, Ternynck C, Marot G, Bujor AM, Hachulla E, Launay D, Humbert M, Guignabert C, Lafyatis R
Ann Rheum Dis 2023 Mar;82(3):365-373. Epub 2022 Dec 5 doi: 10.1136/ard-2022-223237. PMID: 36600187Free PMC Article
Safety and Efficacy of B-Cell Depletion with Rituximab for the Treatment of Systemic Sclerosis-associated Pulmonary Arterial Hypertension: A Multicenter, Double-Blind, Randomized, Placebo-controlled Trial.
Zamanian RT, Badesch D, Chung L, Domsic RT, Medsger T, Pinckney A, Keyes-Elstein L, D'Aveta C, Spychala M, White RJ, Hassoun PM, Torres F, Sweatt AJ, Molitor JA, Khanna D, Maecker H, Welch B, Goldmuntz E, Nicolls MR
Am J Respir Crit Care Med 2021 Jul 15;204(2):209-221. doi: 10.1164/rccm.202009-3481OC. PMID: 33651671Free PMC Article
Pulmonary Hypertension in HIV.
Basyal B, Jarrett H, Barnett CF
Can J Cardiol 2019 Mar;35(3):288-298. doi: 10.1016/j.cjca.2019.01.005. PMID: 30825951
Drug-associated pulmonary arterial hypertension.
McGee M, Whitehead N, Martin J, Collins N
Clin Toxicol (Phila) 2018 Sep;56(9):801-809. Epub 2018 Mar 6 doi: 10.1080/15563650.2018.1447119. PMID: 29508628
Pulmonary arterial hypertension in schistosomiasis.
Gavilanes F, Fernandes CJ, Souza R
Curr Opin Pulm Med 2016 Sep;22(5):408-14. doi: 10.1097/MCP.0000000000000300. PMID: 27428795

Therapy

Safety and Efficacy of B-Cell Depletion with Rituximab for the Treatment of Systemic Sclerosis-associated Pulmonary Arterial Hypertension: A Multicenter, Double-Blind, Randomized, Placebo-controlled Trial.
Zamanian RT, Badesch D, Chung L, Domsic RT, Medsger T, Pinckney A, Keyes-Elstein L, D'Aveta C, Spychala M, White RJ, Hassoun PM, Torres F, Sweatt AJ, Molitor JA, Khanna D, Maecker H, Welch B, Goldmuntz E, Nicolls MR
Am J Respir Crit Care Med 2021 Jul 15;204(2):209-221. doi: 10.1164/rccm.202009-3481OC. PMID: 33651671Free PMC Article
Drug-associated pulmonary arterial hypertension.
McGee M, Whitehead N, Martin J, Collins N
Clin Toxicol (Phila) 2018 Sep;56(9):801-809. Epub 2018 Mar 6 doi: 10.1080/15563650.2018.1447119. PMID: 29508628
Features and Outcomes of Methamphetamine-associated Pulmonary Arterial Hypertension.
Zamanian RT, Hedlin H, Greuenwald P, Wilson DM, Segal JI, Jorden M, Kudelko K, Liu J, Hsi A, Rupp A, Sweatt AJ, Tuder R, Berry GJ, Rabinovitch M, Doyle RL, de Jesus Perez V, Kawut SM
Am J Respir Crit Care Med 2018 Mar 15;197(6):788-800. doi: 10.1164/rccm.201705-0943OC. PMID: 28934596Free PMC Article
Human immunodeficiency virus-associated pulmonary arterial hypertension.
Barnett CF, Hsue PY
Clin Chest Med 2013 Jun;34(2):283-92. Epub 2013 Apr 8 doi: 10.1016/j.ccm.2013.01.009. PMID: 23702177Free PMC Article
Drugs and toxins-associated pulmonary arterial hypertension: lessons learned and challenges ahead.
de Jesus Perez V, Kudelko K, Snook S, Zamanian RT
Int J Clin Pract Suppl 2011 Jan;(169):8-10. doi: 10.1111/j.1742-1241.2010.02606.x. PMID: 21176010

Prognosis

Safety and Efficacy of B-Cell Depletion with Rituximab for the Treatment of Systemic Sclerosis-associated Pulmonary Arterial Hypertension: A Multicenter, Double-Blind, Randomized, Placebo-controlled Trial.
Zamanian RT, Badesch D, Chung L, Domsic RT, Medsger T, Pinckney A, Keyes-Elstein L, D'Aveta C, Spychala M, White RJ, Hassoun PM, Torres F, Sweatt AJ, Molitor JA, Khanna D, Maecker H, Welch B, Goldmuntz E, Nicolls MR
Am J Respir Crit Care Med 2021 Jul 15;204(2):209-221. doi: 10.1164/rccm.202009-3481OC. PMID: 33651671Free PMC Article
Pulmonary Hypertension in HIV.
Basyal B, Jarrett H, Barnett CF
Can J Cardiol 2019 Mar;35(3):288-298. doi: 10.1016/j.cjca.2019.01.005. PMID: 30825951
Methamphetamine and the risk of pulmonary arterial hypertension.
Ramirez RL 3rd, Perez VJ, Zamanian RT
Curr Opin Pulm Med 2018 Sep;24(5):416-424. doi: 10.1097/MCP.0000000000000513. PMID: 30036313Free PMC Article
HIV-associated pulmonary hypertension.
Jarrett H, Barnett C
Curr Opin HIV AIDS 2017 Nov;12(6):566-571. doi: 10.1097/COH.0000000000000418. PMID: 28902721
Systemic sclerosis-associated pulmonary arterial hypertension.
Chaisson NF, Hassoun PM
Chest 2013 Oct;144(4):1346-1356. doi: 10.1378/chest.12-2396. PMID: 24081346Free PMC Article

Clinical prediction guides

Intravenous prostanoids in systemic sclerosis-associated pulmonary arterial hypertension: a single-centre experience.
Vlachou M, Fayed H, Dawson A, Reddecliffe S, Stevenson A, Thomson RJ, Schreiber BE, Coghlan JG
Rheumatology (Oxford) 2022 Mar 2;61(3):1106-1114. doi: 10.1093/rheumatology/keab478. PMID: 34142120
Safety and Efficacy of B-Cell Depletion with Rituximab for the Treatment of Systemic Sclerosis-associated Pulmonary Arterial Hypertension: A Multicenter, Double-Blind, Randomized, Placebo-controlled Trial.
Zamanian RT, Badesch D, Chung L, Domsic RT, Medsger T, Pinckney A, Keyes-Elstein L, D'Aveta C, Spychala M, White RJ, Hassoun PM, Torres F, Sweatt AJ, Molitor JA, Khanna D, Maecker H, Welch B, Goldmuntz E, Nicolls MR
Am J Respir Crit Care Med 2021 Jul 15;204(2):209-221. doi: 10.1164/rccm.202009-3481OC. PMID: 33651671Free PMC Article
Drug-associated pulmonary arterial hypertension.
McGee M, Whitehead N, Martin J, Collins N
Clin Toxicol (Phila) 2018 Sep;56(9):801-809. Epub 2018 Mar 6 doi: 10.1080/15563650.2018.1447119. PMID: 29508628
An Update on Systemic Sclerosis-Associated Pulmonary Arterial Hypertension: a Review of the Current Literature.
Sundaram SM, Chung L
Curr Rheumatol Rep 2018 Feb 27;20(2):10. doi: 10.1007/s11926-018-0709-5. PMID: 29488016
Challenges and opportunities in treating inflammation associated with pulmonary hypertension.
Voelkel NF, Tamosiuniene R, Nicolls MR
Expert Rev Cardiovasc Ther 2016 Aug;14(8):939-51. Epub 2016 May 4 doi: 10.1080/14779072.2016.1180976. PMID: 27096622Free PMC Article

Recent systematic reviews

Schistosomiasis-associated pulmonary arterial hypertension: a systematic review.
Knafl D, Gerges C, King CH, Humbert M, Bustinduy AL
Eur Respir Rev 2020 Mar 31;29(155) Epub 2020 Feb 5 doi: 10.1183/16000617.0089-2019. PMID: 32024722Free PMC Article
Drug-associated pulmonary arterial hypertension.
McGee M, Whitehead N, Martin J, Collins N
Clin Toxicol (Phila) 2018 Sep;56(9):801-809. Epub 2018 Mar 6 doi: 10.1080/15563650.2018.1447119. PMID: 29508628
Serological biomarkers as risk factors of SLE-associated pulmonary arterial hypertension: a systematic review and meta-analysis.
Wang J, Qian J, Wang Y, Zhao J, Wang Q, Tian Z, Li M, Zeng X
Lupus 2017 Nov;26(13):1390-1400. Epub 2017 Apr 14 doi: 10.1177/0961203317702255. PMID: 28409522
Survival and prognostic factors of systemic lupus erythematosus-associated pulmonary arterial hypertension: A PRISMA-compliant systematic review and meta-analysis.
Qian J, Wang Y, Huang C, Yang X, Zhao J, Wang Q, Tian Z, Li M, Zeng X
Autoimmun Rev 2016 Mar;15(3):250-7. Epub 2015 Nov 27 doi: 10.1016/j.autrev.2015.11.012. PMID: 26640159
Screening and diagnostic modalities for connective tissue disease-associated pulmonary arterial hypertension: a systematic review.
Gladue H, Altorok N, Townsend W, McLaughlin V, Khanna D
Semin Arthritis Rheum 2014 Feb;43(4):536-41. Epub 2013 Sep 5 doi: 10.1016/j.semarthrit.2013.08.002. PMID: 24012044Free PMC Article

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