Clinical characteristics in lymphangioleiomyomatosis-related pulmonary hypertension: an observation on 50 patients / 医学前沿
Frontiers of Medicine
; (4): 259-266, 2019.
Article
ي En
| WPRIM
| ID: wpr-772706
المكتبة المسؤولة:
WPRO
ABSTRACT
Lymphangioleiomyomatosis (LAM) is a rare diffuse cystic lung disease. Knowledge on LAM-related pulmonary hypertension (PH) is limited. This study aimed to analyze the clinical characteristics of LAM with elevated pulmonary artery pressure (PAP) and evaluate the potential efficacy of sirolimus. The study involved 50 LAM patients who underwent echocardiography. According to the tricuspid regurgitation velocity (TRV), these patients were divided into the TRV ⩽ 2.8 m/s group and TRV > 2.8 m/s group. Both groups comprised 25 females with an average age of 38.6 ± 8.1 and 41.5 ± 8.9 years. In the TRV > 2.8 m/s group, the estimated systolic PAP (SPAP) was significantly elevated (52.08 ± 12.45 mmHg vs. 30.24 ± 5.25 mmHg, P < 0.01). Linear analysis showed that SPAP was correlated with forced expiratory volume in 1 s (FEV), diffusing capacity of the lungs for carbon monoxide, alveolar arterial oxygen gradient (PO), and 6 min walking distance (r =-0.392, -0.351, 0.450, and -0.591, respectively; P < 0.05), in which PO was a risk factor for SPAP elevation (β = 0.064, OR = 1.066, P < 0.05). Moreover, in 10 patients who received sirolimus therapy, SPAP decreased from 57.0 12.6 mmHg to 35.2 ± 11.1 mmHg. The study showed that LAM patients with PH exhibit poor pulmonary function and hypoxemia and may benefit from sirolimus treatment.
Key words
النص الكامل:
1
الفهرس:
WPRIM
الموضوع الرئيسي:
Oxygen
/
Respiratory Function Tests
/
Therapeutics
/
Blood
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Carbon Monoxide
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Echocardiography
/
Logistic Models
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Multivariate Analysis
/
Lymphangioleiomyomatosis
/
Sirolimus
نوع الدراسة:
Prognostic_studies
/
Risk_factors_studies
المحددات:
Adult
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Female
/
Humans
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Male
اللغة:
En
مجلة:
Frontiers of Medicine
السنة:
2019
نوع:
Article