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Mean pulmonary arterial pressure as a prognostic indicator in connective tissue disease associated with interstitial lung disease: a retrospective cohort study.

Takahashi K, Taniguchi H, Ando M, Sakamoto K, Kondoh Y, Watanabe N, Kimura T, Kataoka K, Suzuki A, Ito S, Hasegawa Y - BMC Pulm Med (2016)

Bottom Line: We did not observe a significant difference in MPAP among various CTDs.A univariate Cox proportional hazard model showed that MPAP has a significant impact on survival, while the type of CTD did not contribute to survival in our cohort.A higher MPAP at the initial evaluation was a significant independent predictor of survival in CTD-ILD.

View Article: PubMed Central - PubMed

Affiliation: Department of Respiratory Medicine, Nagoya University Graduate School of Medicine, Nagoya, Aichi, Japan.

ABSTRACT

Background: Pulmonary hypertension (PH) can develop in connective tissue disease associated interstitial lung disease (CTD-ILD), and contributes to increased morbidity and mortality. However, except for systemic sclerosis and mixed connective tissue disease, the impact of mean pulmonary arterial pressure (MPAP) on survival in CTD-ILD has not been sufficiently elucidated. We hypothesized that pulmonary arterial pressure may be a prognostic factor in CTD-ILDs regardless of the kind of CTD.

Methods: We evaluated the survival impact of MPAP, which is measured using right heart catheterization, on survival of patients with CTD-ILD with various CTD backgrounds. We retrospectively analyzed data of consecutive CTD-ILD patients undergoing a pulmonary function test and right-heart-catheterization at the initial evaluation.

Results: We studied 74 patients (33 men and 41 women, mean age 62.8 ± 9.6, 24 with rheumatoid arthritis, 14 with systemic sclerosis, 14 with polymyositis/dermatomyositis, 11 with primary Sjögren's syndrome, and 11 with other diagnoses). Six patients exhibited pulmonary hypertension (MPAP ≥ 25 mmHg), and 16 (21.6 %) had mild elevation of MPAP (≥20 mmHg). The mean MPAP was 17.2 ± 5.5 mmHg. We did not observe a significant difference in MPAP among various CTDs. A univariate Cox proportional hazard model showed that MPAP has a significant impact on survival, while the type of CTD did not contribute to survival in our cohort. A multivariate Cox proportional hazard model showed MPAP (HR = 1.087; 95 % CI 1.008-1.172; p = 0.030) to be the sole independent determinant of survival.

Conclusions: Mild elevation of MPAP is relatively common in CTD-ILD patients with various CTD backgrounds. A higher MPAP at the initial evaluation was a significant independent predictor of survival in CTD-ILD. MPAP evaluation provides additional information on disease status and will help physicians predict mortality in CTD-ILD.

No MeSH data available.


Related in: MedlinePlus

Criteria for screening and inclusion of patients in the study. CTD-ILD: connective tissue disease associated interstitial lung disease; PH: pulmonary hypertension; LTOT: long term oxygenation therapy; PCWP: pulmonary capillary wedge pressure; RHC: right heart catheterization
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Fig1: Criteria for screening and inclusion of patients in the study. CTD-ILD: connective tissue disease associated interstitial lung disease; PH: pulmonary hypertension; LTOT: long term oxygenation therapy; PCWP: pulmonary capillary wedge pressure; RHC: right heart catheterization

Mentions: In this study, we retrospectively analyzed our ILD patient database, which registers the results of systematic evaluations undergone at the time of their initial consultation with our department at Tosei General Hospital (Aichi, Japan). Of 619 records of patients with ILD examined between July 2007 and June 2012, 102 were diagnosed with CTD-ILD. Twenty-eight patients were excluded from the study for the following reasons: (1) they had been treated with PH targeted therapy, (2) evaluation was done using supplemental oxygen, (3) they were suffering from an unstable disease, such as acute exacerbation, infection, or heart failure, or (4) RHC was not performed within 3 months of initial evaluation or RHC data was missing. Finally, we reviewed the charts of 74 stable CTD-ILD patients who underwent RHC for evaluation during this period (Fig. 1).Fig. 1


Mean pulmonary arterial pressure as a prognostic indicator in connective tissue disease associated with interstitial lung disease: a retrospective cohort study.

Takahashi K, Taniguchi H, Ando M, Sakamoto K, Kondoh Y, Watanabe N, Kimura T, Kataoka K, Suzuki A, Ito S, Hasegawa Y - BMC Pulm Med (2016)

Criteria for screening and inclusion of patients in the study. CTD-ILD: connective tissue disease associated interstitial lung disease; PH: pulmonary hypertension; LTOT: long term oxygenation therapy; PCWP: pulmonary capillary wedge pressure; RHC: right heart catheterization
© Copyright Policy - OpenAccess
Related In: Results  -  Collection

License 1 - License 2
Show All Figures
getmorefigures.php?uid=PMC4837628&req=5

Fig1: Criteria for screening and inclusion of patients in the study. CTD-ILD: connective tissue disease associated interstitial lung disease; PH: pulmonary hypertension; LTOT: long term oxygenation therapy; PCWP: pulmonary capillary wedge pressure; RHC: right heart catheterization
Mentions: In this study, we retrospectively analyzed our ILD patient database, which registers the results of systematic evaluations undergone at the time of their initial consultation with our department at Tosei General Hospital (Aichi, Japan). Of 619 records of patients with ILD examined between July 2007 and June 2012, 102 were diagnosed with CTD-ILD. Twenty-eight patients were excluded from the study for the following reasons: (1) they had been treated with PH targeted therapy, (2) evaluation was done using supplemental oxygen, (3) they were suffering from an unstable disease, such as acute exacerbation, infection, or heart failure, or (4) RHC was not performed within 3 months of initial evaluation or RHC data was missing. Finally, we reviewed the charts of 74 stable CTD-ILD patients who underwent RHC for evaluation during this period (Fig. 1).Fig. 1

Bottom Line: We did not observe a significant difference in MPAP among various CTDs.A univariate Cox proportional hazard model showed that MPAP has a significant impact on survival, while the type of CTD did not contribute to survival in our cohort.A higher MPAP at the initial evaluation was a significant independent predictor of survival in CTD-ILD.

View Article: PubMed Central - PubMed

Affiliation: Department of Respiratory Medicine, Nagoya University Graduate School of Medicine, Nagoya, Aichi, Japan.

ABSTRACT

Background: Pulmonary hypertension (PH) can develop in connective tissue disease associated interstitial lung disease (CTD-ILD), and contributes to increased morbidity and mortality. However, except for systemic sclerosis and mixed connective tissue disease, the impact of mean pulmonary arterial pressure (MPAP) on survival in CTD-ILD has not been sufficiently elucidated. We hypothesized that pulmonary arterial pressure may be a prognostic factor in CTD-ILDs regardless of the kind of CTD.

Methods: We evaluated the survival impact of MPAP, which is measured using right heart catheterization, on survival of patients with CTD-ILD with various CTD backgrounds. We retrospectively analyzed data of consecutive CTD-ILD patients undergoing a pulmonary function test and right-heart-catheterization at the initial evaluation.

Results: We studied 74 patients (33 men and 41 women, mean age 62.8 ± 9.6, 24 with rheumatoid arthritis, 14 with systemic sclerosis, 14 with polymyositis/dermatomyositis, 11 with primary Sjögren's syndrome, and 11 with other diagnoses). Six patients exhibited pulmonary hypertension (MPAP ≥ 25 mmHg), and 16 (21.6 %) had mild elevation of MPAP (≥20 mmHg). The mean MPAP was 17.2 ± 5.5 mmHg. We did not observe a significant difference in MPAP among various CTDs. A univariate Cox proportional hazard model showed that MPAP has a significant impact on survival, while the type of CTD did not contribute to survival in our cohort. A multivariate Cox proportional hazard model showed MPAP (HR = 1.087; 95 % CI 1.008-1.172; p = 0.030) to be the sole independent determinant of survival.

Conclusions: Mild elevation of MPAP is relatively common in CTD-ILD patients with various CTD backgrounds. A higher MPAP at the initial evaluation was a significant independent predictor of survival in CTD-ILD. MPAP evaluation provides additional information on disease status and will help physicians predict mortality in CTD-ILD.

No MeSH data available.


Related in: MedlinePlus