ACR Meeting Abstracts

ACR Meeting Abstracts

  • Home
  • Meetings Archive
    • ACR Convergence 2021
    • ACR Convergence 2020
    • 2020 ACR/ARP PRSYM
    • 2019 ACR/ARP Annual Meeting
    • 2018 ACR/ARHP Annual Meeting
    • 2017 ACR/ARHP Annual Meeting
    • 2017 ACR/ARHP PRSYM
    • 2016-2009 Meetings
    • Download Abstracts
  • Keyword Index
  • Advanced Search
  • Your Favorites
    • Favorites
    • Login
    • View and print all favorites
    • Clear all your favorites
  • Meeting Resource Center

Abstract Number: 231

Pulmonary Hypertension in the Antisynthetase Syndrome

Helena Andersson1, T. Mogens Aalokken2, Torhild Garen3, Oyvind Molberg4 and Jan Tore Gran1, 1Rheumatology, Oslo University Hospital, Oslo, Norway, 2Department of Radiology, Oslo University Hospital Rikshospitalet, Oslo, Norway, 3Department of Rheumatology, Oslo University Hospital Rikshospitalet, Oslo, Norway, 4Department of Rheumatology, Oslo University Hospital, Rikshospitalet, Oslo, Norway

Meeting: 2012 ACR/ARHP Annual Meeting

Keywords: Myositis and pulmonary complications

  • Tweet
  • Email
  • Print
Session Information

Session Title: Muscle Biology, Myositis and Myopathies: Clinical and Therapuetic Aspects of Idiopathic Inflammatory Myopathies

Session Type: Abstract Submissions (ACR)

Background/Purpose:

Methods:

Objectives: To describe the frequency and clinical characteristics of pulmonary hypertension (PH) in a cohort of Antisynthetase Syndrome (ASS) patients.

Methods: Patients from a single referral center diagnosed between 1994-2010 with a positive serologic test (immuno-blot) of antisynthetase antibodies, interstitial lung disease (ILD) and/or myositis were defined as ASS (N=90). All data were retrospectively collected from medical reports. A diameter of the Pulmonal artery >29 mm, measured by CT-scans, was defined as pathological. PH was suspected with an estimated pulmonary arterial pressure (PAP) of >45mmHg at transthoracal eccocardiography (TTE), and defined as a mean PAP >25 mmHg measured by right heart catheterization (RHC). The ratio between forced vital capacity and diffusion capacity of the lungs (FVC/DLCO) was considered pathological, indicating PH, at values > 1.6. All examinations were done within 3 months for each patient.

Fisher´s exact test was used to evaluate statistical significance (p<0.05)

Results: The cohort consisted of 75 anti-Jo1 positive, seven PL-7 positive and eight PL-12 positive patients with mean age at ASS diagnosis of 48 years (range 12-82 yrs) and median disease duration of seven years (range 0.25-34 yrs). Fourteen patients were referred to RHC due to clinical suspicion of PH. Ten out of 14 were diagnosed with PH, with a median PAP of 38 mmHg (range 26-55). Eight out of 10 patients had a pre-capillary type of PH, defined as pulmonal capillary wedge pressure (PCWP) < 15mmHg. Over all frequency of PH was 11 % (10/90). Twelve of 14 patients had available TTE-exams, all but one indicating PH with estimated PAP of >45mmHg. The Pulmonal artery was pathological enlarged in 9/10 patients with PH, median diameter 40 mm (range 33-45). Although not statistic significant (p<0.07), the result indicates a correlation between enlarged pulmonary artery and PH. Seven of nine patients with PH had pathological FVC/DLCO ratios. All 10 PH patients had manifest ILD at time of PH diagnosis. 2/10 patients with PH had co-existing rheumatic disease, both systemic sclerosis. The diagnosis of PH was made 36-336 months after ASS onset. Five of 10 patients with PH died during the observation-period.

     Clinical characteristics of ASS patients with performed RHC

PATIENT

SEX/AGE

ANTIBODY

SS-A

FVC/DLCO

DLCO

DIA A PULM

ECCO/ mmHg

RHC/ mmHg

PCWP/

mmHg

       DEATH

PH

 

 

 

 

 

 

 

 

 

 

1

M 61

Jo-1

+

2,12

17

45

60

33

2

+

2

F 63

Jo-1

 

NA

NA

36

55

35

14

 

3

M 44

Jo-1

+

1,52

38

42

40

26

9

 

4

F 43

Jo-1

+

1,91

45

42

70

44

7

 

5

F 54

Jo-1

+

3

15

33

70

38

10

+

6

F 18

Jo-1

+

2,5

12

40

NA

50

8

+

7

M 33

Jo-1

+

2,23

26

38

60

38

16

 

8

F 21

Jo-1

 

1,62

17

NA

76

55

18

+

9

M 43

Jo-1

+

1,8

41

41

60

26

7

 

10

F 55

Jo-1

+

0,82

56

33

98

50

15

+

No PH

 

 

 

 

 

 

 

 

 

 

11

M 60

Jo-1

+

1,87

56

28

60

23

11

 

12

F 12

PL-12

+

1,17

47

33

45

18

2

 

13

F 19

Jo-1

 

1,45

33

27

NA

15

4

 

14

F 31

PL-12

+

2,09

31

33

70

24

6

 

Conclusion:

In this cohort of 90 ASS patients and with a median disease duration of seven years, a PH frequency of 11 % diagnosed by RHC was observed. The study indicated a correlation between enlarged Pulmonal artery and PH. In spite of small series, the results indicate PH as an important complication of ASS and should be given further notice.


Disclosure:

H. Andersson,
None;

T. M. Aalokken,
None;

T. Garen,
None;

O. Molberg,
None;

J. T. Gran,
None.

  • Tweet
  • Email
  • Print

« Back to 2012 ACR/ARHP Annual Meeting

ACR Meeting Abstracts - https://acrabstracts.org/abstract/pulmonary-hypertension-in-the-antisynthetase-syndrome/

Advanced Search

Your Favorites

You can save and print a list of your favorite abstracts during your browser session by clicking the “Favorite” button at the bottom of any abstract. View your favorites »

ACR Pediatric Rheumatology Symposium 2020

© COPYRIGHT 2022 AMERICAN COLLEGE OF RHEUMATOLOGY

Wiley

  • Home
  • Meetings Archive
  • Advanced Search
  • Meeting Resource Center
  • Online Journal
  • Privacy Policy
  • Permissions Policies