Dominating Cause of Pulmonary Hypertension May Change Over Time—Diagnostic and Therapeutic Considerations in a Patient with Pulmonary Hypertension Due to Rheumatoid Arthritis with Lung Involvement
Chronic lung diseases are one of the most frequent causes of pulmonary hypertension (PH). The diagnostic challenge is to differentiate PH due to chronic lung disease from pulmonary arterial hypertension (PAH) with coexisting chronic lung disease. Moreover, the dominating cause of PH may change over...
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MDPI AG
2021-10-01
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| Series: | Diagnostics |
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| author | Monika Szturmowicz Monika Franczuk Małgorzata Ewa Jędrych Dorota Wyrostkiewicz Karina Oniszh Szymon Darocha Krzysztof Kasperowicz Marcin Kurzyna |
| author_facet | Monika Szturmowicz Monika Franczuk Małgorzata Ewa Jędrych Dorota Wyrostkiewicz Karina Oniszh Szymon Darocha Krzysztof Kasperowicz Marcin Kurzyna |
| author_sort | Monika Szturmowicz |
| collection | DOAJ |
| description | Chronic lung diseases are one of the most frequent causes of pulmonary hypertension (PH). The diagnostic challenge is to differentiate PH due to chronic lung disease from pulmonary arterial hypertension (PAH) with coexisting chronic lung disease. Moreover, the dominating cause of PH may change over time, requiring the implementation of new diagnostic procedures and new treatment modalities. We present a 68-year-old female, initially diagnosed with PH in the course of interstitial lung disease, with restrictive impairment of lung function. Therapy with immunosuppressive drugs resulted in significant clinical, radiological and functional improvement. However, five years later, arthritis symptoms developed, with PH worsening, despite stable lung disease. The patient was diagnosed with PAH in the course of rheumatoid arthritis. The introduction of sildenafil resulted in marked clinical and hemodynamic responses. Long-term survival (eleven years from PH onset and five years from PAH confirmation) has been achieved, and the patient remains in good functional condition. As the differential diagnosis of PH in patients with lung diseases is complex, the cooperation of pulmonologists and cardiologists is mandatory to obtain therapeutic success. |
| first_indexed | 2024-03-10T06:37:46Z |
| format | Article |
| id | doaj.art-9d73a4c4fa8547d489bc1cbca86cb35c |
| institution | Directory Open Access Journal |
| issn | 2075-4418 |
| language | English |
| last_indexed | 2024-03-10T06:37:46Z |
| publishDate | 2021-10-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Diagnostics |
| spelling | doaj.art-9d73a4c4fa8547d489bc1cbca86cb35c2023-11-22T17:59:00ZengMDPI AGDiagnostics2075-44182021-10-011110193110.3390/diagnostics11101931Dominating Cause of Pulmonary Hypertension May Change Over Time—Diagnostic and Therapeutic Considerations in a Patient with Pulmonary Hypertension Due to Rheumatoid Arthritis with Lung InvolvementMonika Szturmowicz0Monika Franczuk1Małgorzata Ewa Jędrych2Dorota Wyrostkiewicz3Karina Oniszh4Szymon Darocha5Krzysztof Kasperowicz6Marcin Kurzyna7Department of Lung Diseases, National Tuberculosis and Lung Diseases Research Institute, 01-138 Warsaw, PolandDepartment of Physiopathology, National Tuberculosis and Lung Diseases Research Institute, 01-138 Warsaw, PolandDepartment of Lung Diseases, National Tuberculosis and Lung Diseases Research Institute, 01-138 Warsaw, PolandDepartment of Lung Diseases, National Tuberculosis and Lung Diseases Research Institute, 01-138 Warsaw, PolandDepartment of Radiology, National Tuberculosis and Lung Diseases Research Institute, 01-138 Warsaw, PolandDepartment of Pulmonary Circulation and Thromboembolic Diseases, Medical Centre of Postgraduate Medication, European Health Centre Otwock, 05-400 Otwock, PolandDepartment of Pulmonary Circulation and Thromboembolic Diseases, Medical Centre of Postgraduate Medication, European Health Centre Otwock, 05-400 Otwock, PolandDepartment of Pulmonary Circulation and Thromboembolic Diseases, Medical Centre of Postgraduate Medication, European Health Centre Otwock, 05-400 Otwock, PolandChronic lung diseases are one of the most frequent causes of pulmonary hypertension (PH). The diagnostic challenge is to differentiate PH due to chronic lung disease from pulmonary arterial hypertension (PAH) with coexisting chronic lung disease. Moreover, the dominating cause of PH may change over time, requiring the implementation of new diagnostic procedures and new treatment modalities. We present a 68-year-old female, initially diagnosed with PH in the course of interstitial lung disease, with restrictive impairment of lung function. Therapy with immunosuppressive drugs resulted in significant clinical, radiological and functional improvement. However, five years later, arthritis symptoms developed, with PH worsening, despite stable lung disease. The patient was diagnosed with PAH in the course of rheumatoid arthritis. The introduction of sildenafil resulted in marked clinical and hemodynamic responses. Long-term survival (eleven years from PH onset and five years from PAH confirmation) has been achieved, and the patient remains in good functional condition. As the differential diagnosis of PH in patients with lung diseases is complex, the cooperation of pulmonologists and cardiologists is mandatory to obtain therapeutic success.https://www.mdpi.com/2075-4418/11/10/1931pulmonary arterial hypertensionrheumatoid arthritislung diseaseright heart catheterizationbody plethysmography |
| spellingShingle | Monika Szturmowicz Monika Franczuk Małgorzata Ewa Jędrych Dorota Wyrostkiewicz Karina Oniszh Szymon Darocha Krzysztof Kasperowicz Marcin Kurzyna Dominating Cause of Pulmonary Hypertension May Change Over Time—Diagnostic and Therapeutic Considerations in a Patient with Pulmonary Hypertension Due to Rheumatoid Arthritis with Lung Involvement Diagnostics pulmonary arterial hypertension rheumatoid arthritis lung disease right heart catheterization body plethysmography |
| title | Dominating Cause of Pulmonary Hypertension May Change Over Time—Diagnostic and Therapeutic Considerations in a Patient with Pulmonary Hypertension Due to Rheumatoid Arthritis with Lung Involvement |
| title_full | Dominating Cause of Pulmonary Hypertension May Change Over Time—Diagnostic and Therapeutic Considerations in a Patient with Pulmonary Hypertension Due to Rheumatoid Arthritis with Lung Involvement |
| title_fullStr | Dominating Cause of Pulmonary Hypertension May Change Over Time—Diagnostic and Therapeutic Considerations in a Patient with Pulmonary Hypertension Due to Rheumatoid Arthritis with Lung Involvement |
| title_full_unstemmed | Dominating Cause of Pulmonary Hypertension May Change Over Time—Diagnostic and Therapeutic Considerations in a Patient with Pulmonary Hypertension Due to Rheumatoid Arthritis with Lung Involvement |
| title_short | Dominating Cause of Pulmonary Hypertension May Change Over Time—Diagnostic and Therapeutic Considerations in a Patient with Pulmonary Hypertension Due to Rheumatoid Arthritis with Lung Involvement |
| title_sort | dominating cause of pulmonary hypertension may change over time diagnostic and therapeutic considerations in a patient with pulmonary hypertension due to rheumatoid arthritis with lung involvement |
| topic | pulmonary arterial hypertension rheumatoid arthritis lung disease right heart catheterization body plethysmography |
| url | https://www.mdpi.com/2075-4418/11/10/1931 |
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