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FRI0249 Pulmonary arterial hypertension in very early systemic sclerosis
  1. J. Sanchez1,
  2. S. Jordan1,1,
  3. J. Distler2,
  4. B. Maurer1,
  5. D. Huscher3,
  6. B. Michel1,
  7. R. Speich4,
  8. O. Distler1
  1. 1Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland
  2. 2Department of Internal Medicine 3, Friedrich-Alexander-University of Erlangen-Nuremberg, Erlangen
  3. 3German Rheumatism Research Centre, Berlin, Germany
  4. 4Department of Internal Medicine, Pulmonary Hypertension Unit, University Hospital Zurich, Zurich, Switzerland


Background Pulmonary arterial hypertension (PAH) is mainly considered a late complication of systemic sclerosis (SSc), but it occurs also in patients with short disease duration. We recently did not find evidence for PAH in a cross-sectional cohort of very early SSc patients with limited follow-up.

Objectives To analyse whether patients with very early SSc develop evidence of PAH in an extended longitudinal follow up study.

Methods The very early SSc cohort for this study consisted of (1) patients with Raynaud’s phenomenon (RP) and at least one of the following features: nailfold capillaroscopy with SSc pattern (SSc-NFC), puffy fingers (PF), SSc-specific auto-antibodies (SSc-Ab), and (2) patients without RP, but with SSc-Ab and SSc-NFC or PF. All very early SSc patients did not fulfil the ACR classification criteria. Screening tests for PAH were: echocardiography, lung function test, levels of brain natriuretic peptide precursor (pro-BNP) and, in cases of suspected PAH, right heart catheterisation (RHC). Lung function data were compared to patients with established SSc fulfilling ACR criteria and to healthy controls. Data were analysed using standard descriptive statistics and presented by mean and standard deviation or median and range.

Results Forty-five patients with very early SSc were recruited for the study. Extended longitudinal follow up data were available for 32/45 patients with a total follow up time of 912 person-months with a range of 6-96 months. In none of the patients, PAH was suspected and none had to undergo RHC during follow up.

Echocardiography: No difference from baseline and no evidence for PAH was found by echocardiography. 0/31 (0%) patients had systolic pulmonary arterial pressure (sPAP) >30 mmHg, 6/31 (19%) had diastolic dysfunction grade 1 with 3/6 developing it during follow up, 2/31 (6%) had dilated right atrium and 0/31 (0%) dilated right ventricle.

Lung function: Lung function data are summarized in Table 1. While healthy controls did not show DLCO<70% predicted, it was found in 18% of very early SSc patients at baseline, slightly progressed during follow up, and was much more prevalent in established SSc.

Table 1. DLCO <70% and FVC <70% predicted in healthy controls, early SSc baseline and follow up and in established SSc

Pro-BNP: Prevalence of increased pro-BNP was higher at follow up than at baseline, but pro-BNP was only slightly elevated, and the 3/25 patients with unexplained elevated pro-BNP levels did not had any other sign of PAH.

Conclusions We found no evidence that PAH develops in very early SSc patients during follow up. However, we observed an isolated reduction of DLCO in this very early SSc cohort, which occurs at earliest disease stages and slightly progresses at follow up. These patients might be at risk for later PAH development.

This study was partially supported by Actelion Switzerland

Disclosure of Interest J. Sanchez: None Declared, S. Jordan: None Declared, J. Distler: None Declared, B. Maurer: None Declared, D. Huscher: None Declared, B. Michel: None Declared, R. Speich: None Declared, O. Distler Grant/Research support from: Actelion, Pfizer, Ergonex and Sanofi, Consultant for: Actelion, Pfizer, Ergonex, BMS, Sanofi-Aventis, United BioSource Corporation, medac, 4D Science, Boehringer-Ingelheim, Active Biotech and Roche, Speakers Bureau: Actelion, Pfizer, Encysive and Ergonex.

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