Targeted treatment strategy for patients with severe pulmonary hypertension secondary to connective tissue diseases
Abstract Background Pulmonary arterial hypertension (PAH) associated with connective tissue diseases (CTD) (CTD‐PAH) remains a difficult challenge in clinical practice. We aimed to evaluate the effects of targeted vasodilators in patients with severe CTD‐PAH. Methods The data of 53 patients with sev...
| Main Authors: | , , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Wiley
2023-09-01
|
| Series: | Rheumatology & Autoimmunity |
| Subjects: | |
| Online Access: | https://doi.org/10.1002/rai2.12086 |
| _version_ | 1797672432383819776 |
|---|---|
| author | Bo Jiang Chun Wang Yansheng Jin Guanjun Guo Kangxing Zhou Xuebing Feng Genhong Yao |
| author_facet | Bo Jiang Chun Wang Yansheng Jin Guanjun Guo Kangxing Zhou Xuebing Feng Genhong Yao |
| author_sort | Bo Jiang |
| collection | DOAJ |
| description | Abstract Background Pulmonary arterial hypertension (PAH) associated with connective tissue diseases (CTD) (CTD‐PAH) remains a difficult challenge in clinical practice. We aimed to evaluate the effects of targeted vasodilators in patients with severe CTD‐PAH. Methods The data of 53 patients with severe CTD‐PAH hospitalized at the Department of Rheumatology and Immunology, The Affiliated Drum Tower Hospital of Nanjing University Medical School, were retrospectively reviewed. Patients were followed up for an average of 2 years to track their outcomes. The efficacy of treatment and the survival rate of patients with severe CTD‐PAH were determined. Results Among the causes of severe CTD‐PAH, systemic lupus erythematosus (SLE) was the most common (39.6%), and the age at onset in patients with SLE‐PAH was younger than that of patients with other CTD. Bosentan was more effective than sildenafil in reducing pulmonary artery pressure, improving cardiac function, and increasing survival time. Combination therapy with targeted vasodilators significantly improved the prognosis of patients with severe CTD‐PAH compared with monotherapy. Conclusions Patients with severe CTD‐PAH should be treated early with targeted vasodilators. In this study, bosentan was superior to sildenafil. Combined treatment might be an option for severe CTD‐PAH. |
| first_indexed | 2024-03-11T21:29:58Z |
| format | Article |
| id | doaj.art-7ba1aef5f42b4476b071169bc0472a33 |
| institution | Directory Open Access Journal |
| issn | 2767-1429 |
| language | English |
| last_indexed | 2024-03-11T21:29:58Z |
| publishDate | 2023-09-01 |
| publisher | Wiley |
| record_format | Article |
| series | Rheumatology & Autoimmunity |
| spelling | doaj.art-7ba1aef5f42b4476b071169bc0472a332023-09-27T10:22:43ZengWileyRheumatology & Autoimmunity2767-14292023-09-013315716510.1002/rai2.12086Targeted treatment strategy for patients with severe pulmonary hypertension secondary to connective tissue diseasesBo Jiang0Chun Wang1Yansheng Jin2Guanjun Guo3Kangxing Zhou4Xuebing Feng5Genhong Yao6Department of Rheumatology and Immunology The Affiliated Drum Tower Hospital of Nanjing University Medical School Nanjing Jiangsu ChinaDepartment of Rheumatology and Immunology The Affiliated Drum Tower Hospital of Nanjing University Medical School Nanjing Jiangsu ChinaDepartment of Rheumatology and Immunology Wuzhong People's Hospital Suzhou Jiangsu ChinaDepartment of Cardiac Ultrasound The Affiliated Drum Tower Hospital of Nanjing University Medical School Nanjing Jiangsu ChinaDepartment of Rheumatology and Immunology The Affiliated Drum Tower Hospital of Nanjing University Medical School Nanjing Jiangsu ChinaDepartment of Rheumatology and Immunology The Affiliated Drum Tower Hospital of Nanjing University Medical School Nanjing Jiangsu ChinaDepartment of Rheumatology and Immunology The Affiliated Drum Tower Hospital of Nanjing University Medical School Nanjing Jiangsu ChinaAbstract Background Pulmonary arterial hypertension (PAH) associated with connective tissue diseases (CTD) (CTD‐PAH) remains a difficult challenge in clinical practice. We aimed to evaluate the effects of targeted vasodilators in patients with severe CTD‐PAH. Methods The data of 53 patients with severe CTD‐PAH hospitalized at the Department of Rheumatology and Immunology, The Affiliated Drum Tower Hospital of Nanjing University Medical School, were retrospectively reviewed. Patients were followed up for an average of 2 years to track their outcomes. The efficacy of treatment and the survival rate of patients with severe CTD‐PAH were determined. Results Among the causes of severe CTD‐PAH, systemic lupus erythematosus (SLE) was the most common (39.6%), and the age at onset in patients with SLE‐PAH was younger than that of patients with other CTD. Bosentan was more effective than sildenafil in reducing pulmonary artery pressure, improving cardiac function, and increasing survival time. Combination therapy with targeted vasodilators significantly improved the prognosis of patients with severe CTD‐PAH compared with monotherapy. Conclusions Patients with severe CTD‐PAH should be treated early with targeted vasodilators. In this study, bosentan was superior to sildenafil. Combined treatment might be an option for severe CTD‐PAH.https://doi.org/10.1002/rai2.12086connective tissue diseasepulmonary arterial hypertensionsystemic lupus erythematosustargeted vasodilator |
| spellingShingle | Bo Jiang Chun Wang Yansheng Jin Guanjun Guo Kangxing Zhou Xuebing Feng Genhong Yao Targeted treatment strategy for patients with severe pulmonary hypertension secondary to connective tissue diseases Rheumatology & Autoimmunity connective tissue disease pulmonary arterial hypertension systemic lupus erythematosus targeted vasodilator |
| title | Targeted treatment strategy for patients with severe pulmonary hypertension secondary to connective tissue diseases |
| title_full | Targeted treatment strategy for patients with severe pulmonary hypertension secondary to connective tissue diseases |
| title_fullStr | Targeted treatment strategy for patients with severe pulmonary hypertension secondary to connective tissue diseases |
| title_full_unstemmed | Targeted treatment strategy for patients with severe pulmonary hypertension secondary to connective tissue diseases |
| title_short | Targeted treatment strategy for patients with severe pulmonary hypertension secondary to connective tissue diseases |
| title_sort | targeted treatment strategy for patients with severe pulmonary hypertension secondary to connective tissue diseases |
| topic | connective tissue disease pulmonary arterial hypertension systemic lupus erythematosus targeted vasodilator |
| url | https://doi.org/10.1002/rai2.12086 |
| work_keys_str_mv | AT bojiang targetedtreatmentstrategyforpatientswithseverepulmonaryhypertensionsecondarytoconnectivetissuediseases AT chunwang targetedtreatmentstrategyforpatientswithseverepulmonaryhypertensionsecondarytoconnectivetissuediseases AT yanshengjin targetedtreatmentstrategyforpatientswithseverepulmonaryhypertensionsecondarytoconnectivetissuediseases AT guanjunguo targetedtreatmentstrategyforpatientswithseverepulmonaryhypertensionsecondarytoconnectivetissuediseases AT kangxingzhou targetedtreatmentstrategyforpatientswithseverepulmonaryhypertensionsecondarytoconnectivetissuediseases AT xuebingfeng targetedtreatmentstrategyforpatientswithseverepulmonaryhypertensionsecondarytoconnectivetissuediseases AT genhongyao targetedtreatmentstrategyforpatientswithseverepulmonaryhypertensionsecondarytoconnectivetissuediseases |