Screening and management of pulmonary arterial hypertension in systemic sclerosis
Systemic sclerosis-associated pulmonary hypertension (SSc-PH) and pulmonary arterial (PA) hypertension (SSc-PAH) are well-recognized manifestations. SSc-PH is a hemodynamic observation, and it is important to identify underlying etiology. SSc patients commonly have mixed etiology for SSc-PH due to i...
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SAGE Publishing
2017-01-01
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| Series: | Indian Journal of Rheumatology |
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| Online Access: | http://www.indianjrheumatol.com/article.asp?issn=0973-3698;year=2017;volume=12;issue=6;spage=204;epage=210;aulast=Nagaraja |
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| author | Vivek Nagaraja Dinesh Khanna |
| author_facet | Vivek Nagaraja Dinesh Khanna |
| author_sort | Vivek Nagaraja |
| collection | DOAJ |
| description | Systemic sclerosis-associated pulmonary hypertension (SSc-PH) and pulmonary arterial (PA) hypertension (SSc-PAH) are well-recognized manifestations. SSc-PH is a hemodynamic observation, and it is important to identify underlying etiology. SSc patients commonly have mixed etiology for SSc-PH due to interstitial lung disease, PAH, and left heart disease. SSc-PAH is associated with high morbidity and mortality. Early detection of PAH through routine screening improves survival in patients with SSc. Right heart catheterization is mandatory to diagnose PAH. SSc-PAH patients should be managed by a multidisciplinary team comprising of rheumatologist, pulmonologist, cardiologist, and physiotherapist. Various pharmacotherapy options to treat SSc-PAH are derived from the idiopathic PAH management. Upfront or sequential combination therapy of PAH-specific drugs seems to confer a clinical benefit compared to monotherapy. Cardiopulmonary rehabilitation should be considered as a part of the management plan. Lung transplantation is a consideration in patients who are not responding to pharmacotherapy. Although the long-term prognosis of SSc-PAH has been historically poor, the landscape is gradually changing with early detection and institution of treatment. |
| format | Article |
| id | doaj-art-c9a4868fd68c414f89834bca09794f5e |
| institution | Kabale University |
| issn | 0973-3698 0973-3701 |
| language | English |
| publishDate | 2017-01-01 |
| publisher | SAGE Publishing |
| record_format | Article |
| series | Indian Journal of Rheumatology |
| spelling | doaj-art-c9a4868fd68c414f89834bca09794f5e2025-01-02T04:57:33ZengSAGE PublishingIndian Journal of Rheumatology0973-36980973-37012017-01-01126204210Screening and management of pulmonary arterial hypertension in systemic sclerosisVivek NagarajaDinesh KhannaSystemic sclerosis-associated pulmonary hypertension (SSc-PH) and pulmonary arterial (PA) hypertension (SSc-PAH) are well-recognized manifestations. SSc-PH is a hemodynamic observation, and it is important to identify underlying etiology. SSc patients commonly have mixed etiology for SSc-PH due to interstitial lung disease, PAH, and left heart disease. SSc-PAH is associated with high morbidity and mortality. Early detection of PAH through routine screening improves survival in patients with SSc. Right heart catheterization is mandatory to diagnose PAH. SSc-PAH patients should be managed by a multidisciplinary team comprising of rheumatologist, pulmonologist, cardiologist, and physiotherapist. Various pharmacotherapy options to treat SSc-PAH are derived from the idiopathic PAH management. Upfront or sequential combination therapy of PAH-specific drugs seems to confer a clinical benefit compared to monotherapy. Cardiopulmonary rehabilitation should be considered as a part of the management plan. Lung transplantation is a consideration in patients who are not responding to pharmacotherapy. Although the long-term prognosis of SSc-PAH has been historically poor, the landscape is gradually changing with early detection and institution of treatment.http://www.indianjrheumatol.com/article.asp?issn=0973-3698;year=2017;volume=12;issue=6;spage=204;epage=210;aulast=NagarajaPulmonary arterial hypertensionpulmonary hypertensionsystemic sclerosis |
| spellingShingle | Vivek Nagaraja Dinesh Khanna Screening and management of pulmonary arterial hypertension in systemic sclerosis Indian Journal of Rheumatology Pulmonary arterial hypertension pulmonary hypertension systemic sclerosis |
| title | Screening and management of pulmonary arterial hypertension in systemic sclerosis |
| title_full | Screening and management of pulmonary arterial hypertension in systemic sclerosis |
| title_fullStr | Screening and management of pulmonary arterial hypertension in systemic sclerosis |
| title_full_unstemmed | Screening and management of pulmonary arterial hypertension in systemic sclerosis |
| title_short | Screening and management of pulmonary arterial hypertension in systemic sclerosis |
| title_sort | screening and management of pulmonary arterial hypertension in systemic sclerosis |
| topic | Pulmonary arterial hypertension pulmonary hypertension systemic sclerosis |
| url | http://www.indianjrheumatol.com/article.asp?issn=0973-3698;year=2017;volume=12;issue=6;spage=204;epage=210;aulast=Nagaraja |
| work_keys_str_mv | AT viveknagaraja screeningandmanagementofpulmonaryarterialhypertensioninsystemicsclerosis AT dineshkhanna screeningandmanagementofpulmonaryarterialhypertensioninsystemicsclerosis |