دورية أكاديمية
أعرض في EDS

Sarcoidosis-associated pulmonary hypertension due to pulmonary arteries stenosis - a case report.

التفاصيل البيبلوغرافية
العنوان: Sarcoidosis-associated pulmonary hypertension due to pulmonary arteries stenosis - a case report.
المؤلفون: Sobiecka M; 1 st Department of Lung Diseases, National Tuberculosis and Lung Diseases Research Institute, Plocka 26, Warsaw, 01-138, Poland. m.sobiecka@igichp.edu.pl., Siemion-Szczesniak I; 1 st Department of Lung Diseases, National Tuberculosis and Lung Diseases Research Institute, Plocka 26, Warsaw, 01-138, Poland., Burakowska B; Department of Radiology, National Tuberculosis and Lung Diseases Research Institute, Plocka 26, Warsaw, 01-138, Poland., Kurzyna M; Department of Pulmonary Circulation, Thromboembolic Diseases and Cardiology European Health Center Otwock, Medical Centre for Postgraduate Education, Otwock, Poland., Dybowska M; 1 st Department of Lung Diseases, National Tuberculosis and Lung Diseases Research Institute, Plocka 26, Warsaw, 01-138, Poland., Tomkowski W; 1 st Department of Lung Diseases, National Tuberculosis and Lung Diseases Research Institute, Plocka 26, Warsaw, 01-138, Poland., Szturmowicz M; 1 st Department of Lung Diseases, National Tuberculosis and Lung Diseases Research Institute, Plocka 26, Warsaw, 01-138, Poland.
المصدر: BMC pulmonary medicine [BMC Pulm Med] 2024 Jul 16; Vol. 24 (1), pp. 346. Date of Electronic Publication: 2024 Jul 16.
نوع المنشور: Case Reports; Journal Article
اللغة: English
بيانات الدورية: Publisher: BioMed Central Country of Publication: England NLM ID: 100968563 Publication Model: Electronic Cited Medium: Internet ISSN: 1471-2466 (Electronic) Linking ISSN: 14712466 NLM ISO Abbreviation: BMC Pulm Med Subsets: MEDLINE
أسماء مطبوعة: Original Publication: London : BioMed Central, [2001]-
مواضيع طبية MeSH: Hypertension, Pulmonary*/etiology , Hypertension, Pulmonary*/diagnosis , Stenosis, Pulmonary Artery*/etiology , Stenosis, Pulmonary Artery*/diagnostic imaging, Humans ; Female ; Aged ; Pulmonary Artery/diagnostic imaging ; Pulmonary Artery/pathology ; Computed Tomography Angiography ; Sarcoidosis/complications ; Sarcoidosis/diagnosis ; Angioplasty, Balloon ; Sarcoidosis, Pulmonary/complications ; Sarcoidosis, Pulmonary/diagnosis
مستخلص: Background: Sarcoidosis-associated pulmonary hypertension (SAPH) is listed in Group 5 of the clinical classification of pulmonary hypertension, due to its complex and multifactorial pathophysiology. The most common cause of SAPH development is advanced lung fibrosis with the associated destruction of the vascular bed, and/or alveolar hypoxia. However, a substantial proportion of SAPH patients (up to 30%) do not have significant fibrosis on chest imaging. In such cases, the development of pulmonary hypertension may be due to the lesions directly affecting the pulmonary vasculature, such as granulomatous angiitis, pulmonary veno-occlusive disease, chronic thromboembolism or external compression of vessels by enlarged lymph nodes. Based on the case of a 69-year-old female who developed SAPH due to pulmonary arteries stenosis, diagnostic difficulties and therapeutic management are discussed.
Case Presentation: The patient, non-smoking female, diagnosed with stage II sarcoidosis twelve years earlier, presented with progressive dyspnoea on exertion, dry cough, minor haemoptysis and increasing oedema of the lower limbs. Computed tomography pulmonary angiography (CTPA) showed complete occlusion of the right upper lobe artery and narrowing of the left lower lobe artery, with post-stenotic dilatation of the arteries of the basal segments. The vascular pathology was caused by adjacent, enlarged lymph nodes with calcifications and fibrotic tissue surrounding the vessels. Pulmonary artery thrombi were not found. The patient was treated with systemic corticosteroid therapy and subsequently with balloon pulmonary angioplasty. Partial improvement in clinical status and hemodynamic parameters has been achieved.
Conclusions: An appropriate screening strategy is required for early detection of pulmonary hypertension in sarcoidosis patients. Once SAPH diagnosis is confirmed, it is crucial to determine the appropriate phenotype of pulmonary hypertension and provide the most effective treatment plan. Although determining SAPH phenotype is challenging, one should remember about the possibility of pulmonary arteries occlusion.
(© 2024. The Author(s).)
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فهرسة مساهمة: Keywords: Balloon pulmonary angioplasty; Corticosteroids; Pulmonary hypertension; SAPH; Sarcoidosis; Treatment
تواريخ الأحداث: Date Created: 20240716 Date Completed: 20240717 Latest Revision: 20240719
رمز التحديث: 20240720
مُعرف محوري في PubMed: PMC11251360
DOI: 10.1186/s12890-024-03152-0
PMID: 39014431
قاعدة البيانات: MEDLINE
الوصف
تدمد:1471-2466
DOI:10.1186/s12890-024-03152-0