Session Information
Session Type: ACR Poster Session A
Session Time: 9:00AM-11:00AM
Background/Purpose:
Pulmonary hypertension (PH) is a feared complication of the anti-synthetase syndrome (ASS) and has previously been reported to occur in 8% of the patients [1]. Prognosis is currently poor, but may be improved by earlier referral to diagnostic right heart catheterization (RHC) and therapeutic interventions. Data on non-invasive screening approaches for PH in ASS are limited. Here, the aim was to test the utility of four different and complementary PH screening approaches.
Methods:
In September 2011, 68/70 living ASS patients from the Oslo University Hospital ASS cohort were included in a cross-sectional study where pulmonary function tests (PFT), Echocardiography (Echo) and HRCT of the lungs were performed. In addition, medical reports from six dead patients with confirmed PH by RHC were analyzed. Arbitrary cut-off values for PH suspicion were defined as follows; (1) DLCO < 50 % of expected value, (2) a ratio between FVC% and DLCO% >1.6, (3) Arteria pulmonalis (A Pulm) diameter ≥ 30 mm on lung CT, (4) estimated mean pulmonary arterial pressure (mPAP) ≥ 45 mmHg by Echo. HRCT and Echo analysts were blinded to the participant’s clinical status. Chi-Square test was used to evaluate statistical significance, p<0.05.
Results:
Median disease duration for the 68 patients was 71 months (6-362) and 97 % of the patients had been diagnosed with interstitial lung disease. Median DLCO% was 62% (range12-87), median FVC /DLCO ratio was 1.33 (0.53-3.03) and the median estimated mPAP and pulmonary artery diameters were 23 mmHg (0-98) and 29 mm (20-45), respectively. Altogether, 16/74 patients had performed RHC, confirming PH in 11/16 patients.
Values below the arbitrary PH suspicion cut-offs were frequent, most frequent was increased diameter of A Pulm, seen in 30/72 (42%) patients. Values suspicious of PH in DLCO, FVC/DLCO ratio, and estimated mPAP were seen in 34%, 28% and 17%, respectively. The correlations between the four variables and confirmed/not confirmed PH are seen in Table 1; Significant correlations were seen between all four variables and confirmed PH, most with the diameter of A Pulm.
Table 1;Correlations between different parameters and Pulmonary Artery Pressure
|
Parameters |
Not confirmed PH
|
Confirmed PH |
p-value |
|
A Pulm < 30 mm, n/N(%) |
42/62 (68) |
0/10 (0) |
<0.001 |
|
A Pulm > 30 mm, n/N(%) |
20/62 (32) |
10/10 (100) |
|
|
mPAP <45mmHg,n/N(%) |
58/62 (94) |
2/10 (20) |
<0.001 |
|
mPAP >45mmHg,n/N(%) |
4/62 (6) |
8/10 (80) |
|
|
DLCO ≥ 50%, n/N (%) |
45/60 (75) |
1/10 (10) |
<0.001 |
|
DLCO ≤ 50%, n/N (%) |
15/60 (25) |
9/10 (90) |
|
|
FVC/DLCO <1.6 n/N (%) |
46/60 (77) |
4/10 (40) |
<0.027 |
|
FVC/DLCO >1.6 n/N(%) |
14/60 (23) |
6/10 (60) |
Conclusion:
Although a small number of patients with confirmed PH, this study shows significant correlation between all tested screening-tools for PH and confirmed PH. All patients (N=10) with confirmed PH had a diameter of A Pulm > 30 mm, and 8/10 had a mPAP of > 45mmHg, indicating these two methods as possible screeningtest for PH. Further studies on PH and it’s predictors are most warranted.
To cite this abstract in AMA style:
Andersson H, Sjaastad I, Schwartz T, Garen T, Aalokken TM, Molberg . Screening for Pulmonary Hypertension in the Anti-Synthetase Syndrome; Utility of Four Different Screening Approaches [abstract]. Arthritis Rheumatol. 2015; 67 (suppl 10). https://acrabstracts.org/abstract/screening-for-pulmonary-hypertension-in-the-anti-synthetase-syndrome-utility-of-four-different-screening-approaches/. Accessed .« Back to ACR/ARHP Annual Meeting 2015
ACR Meeting Abstracts - https://acrabstracts.org/abstract/screening-for-pulmonary-hypertension-in-the-anti-synthetase-syndrome-utility-of-four-different-screening-approaches/