708 Saturday, 14 june 2014 Scientific Abstracts in cPAH (72±18%) versus iPAH 78±16% or PoPH 77±20%; p<0.001). A decline in FEV1 was associated with known markers of disease severity (elevated right atrial pressure, low cardiac index, reduced 6 minute walk distance). A decline in FEV1 was associated with worse survival in cPAH but not other forms of PAH. For every 10% decline in FEV1, 5-year all-cause mortality increased 1. 27 fold (95% C.I 1.07-1.49; p=0.008). The association of FEV1 with mortality persisted after adjusting for age, sex, DLCO, pulmonary pressure and cardiac output. Conclusions: In scleroderma-related PAH, spirometry provides prognostic infor- mation over conventional parameters including pulmonary hemodynamics and diffusing capacity. Whether modest reductions in FEV1 may represent sub-clinical interstitial inflammation or fibrosis, pulmonary venous involvement or concomitant airway involvement warrants further testing. Disclosure of Interest: None declared DOI: 10.1136/annrheumdis-2014-eular.5749 SAT0320 COEXISTENCE OF LEFT HEART DISEASES IS COMMON AMONG PATIENTS WITH CONNECTIVE TISSUE DISEASES-ASSOCIATED PULMONARY ARTERIAL HYPERTENSION K. Tsuchida 1 , H. Yamada 1 ,Y. Yamasaki 1 , S. Ooka 1 , K. Suzuki 2 , Y. Akashi 2 , S. Ozaki 1 . 1 Rheumatology and Allergology; 2 Cardiology, St. Marianna University School of Medicine, Kawasaki, Japan Background: The cause of connective tissue diseases-associated pulmonary hypertension (CTD-PH) includes left heart disease and interstitial lung disease (ILD) as well as pulmonary arterial hypertension (PAH). Careful differential diagnosis of them will be required, because all of these treatment strategies are different (1,2). However, the possibility cannot be denied that left heart diseases are complicated in PAH in CTD patients and have some impact on their PAH. Objectives: To investigate whether left heart diseases can be complicated in CTD patients having PAH. Methods: A total of 396 CTD patients having either dyspnea or lower carbon monoxide diffusing capacity (DL CO ) was performed Doppler echocardiography before and after exercise with the Master’s double two-step. Right heart catheterization (RHC) was recommended in 120 patients (30%) who had >45 mmHg of tricuspid regurgitation pressure gradient (TRPG) just after exercise Doppler echocardiography or who had unexplained dyspnea and <40% of DL CO . Out of the 49 patients whose RHC data was available, 13 (27%) had ≥25 mmHg of mean pulmonary arterial pressure (mPAP) by RHC. Of them, 11 patients had less than 15 mmHg of pulmonary capillary wedge pressure (PCWP). We investigated whether left heart disease were seen in the 11 patients with PAH. Involvement of Left heart diseases was assessed using Doppler echocardiography, thallium scintigraphy, cardiac MRI, and biomarkers. Results: The 11 patients with PAH consisted of 8 with systemic sclerosis, each one with mixed connective tissue disease, systemic lupus erythematosus, and rheumatoid arthritis. Average age was 59.6. Average mPAP and PCWP was 30.7mmHg and 9.9 mmHg. Among 11 PAH patients, 6 (55%) showed <7 mmHg of diastolic pulmonary vascular pressure gradient (DPG). 5 patients (83%) out of them were suggested to have left heart diseases by increased left atrial volume index and E/E’ on echocardiography (n=5, 83%) and/or delayed contrast enhancement on cardiac MRI (n=1,17%). Furthermore, 3 patients (60%) among 5 PAH patients who had ≥7 mmHg of DPG also showed some signs of left heart disease. Conclusions: Coexistence of left heart diseases such as diastolic dysfunction was seen in CTD-PAH patients, even among patients with higher DPG. Left heart diseases should be evaluated in CTD patients with PAH particularly who showed lower DPG values, because of the risk for the deterioration of left heart disease under the treatment with pulmonary vasodilators. References: [1] Wilson SR et el. Pulmonary hypertension and right ventricular dysfunction in left heart disease. Prog Cardiovasc Dis. 2012 Sep-Oct; 55(2): 104-18. [2] Le Pavec J et el. Systemic sclerosis-related pulmonary hypertension as- sociated with interstitial lung disease. Arthritis Rheum, 2011 Aug; 63(8): 2456-64. Disclosure of Interest: None declared DOI: 10.1136/annrheumdis-2014-eular.4878 SAT0321 THE ABSENCE OF SSC PATTERN AT NFC CARRIES A 90% NEGATIVE PREDICTIVE VALUE FOR THE CLASSIFICATION OF VERY EARLY OR ESTABLISHED SSC IN AN UNSELECTED COHORT OF PATIENTS WITH RAYNAUD’S PHENOMENON; EXPERIENCE FROM A SINGLE TERTIARY CENTRE L.-A. Bissell 1,2 , G. Abignano 1,2 , F. Del Galdo 1,2 , M.H. Buch 1,2 . 1 Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds; 2 NIHR Leeds Musculoskeletal Biomedical Research Unit, Leeds Teaching Hospitals NHS Trust, Leeds, United Kingdom Background: Nail-fold capillaroscopy (NFC) patterns of vasculopathy have been well described in systemic sclerosis (SSc) but few studies have reported their predictive value in an unselected cohort of patients with Raynaud’s Phenomenon (RP). Objectives: To describe the association and predictive value of NFC patterns with a clinical diagnosis of SSc. Methods: Patients (pts) referred to a tertiary Scleroderma clinic to aid in the diagnosis and management of RP were evaluated by light/video-NFC, performed by a fully trained physician. Data collected included clinical diagnosis, antibody (Ab) status, NFC findings and fulfilment of the VEDOSS or 2013 ACR/EULAR criteria for SSc. Primary RP (pRP) was defined as RP with no features of CTD/Ab. NFC patterns were determined: normal, non-specific, “early”, “active” or “late” SSc patterns as described. Results: 347 pts were referred to the NFC service between Jan 2009 and Oct 2013; mean (SD) age was 47 (15.2) yrs, 83% were female, 26% smokers (Table 1). On clinical review, 54 (15%) did not have RP, 69 (20%) had primary RP, 52 had SSc (56% ACA+ve, 8% Scl70+ve; 67% lcSSc, 10% dcSSc, 11.5% undefined, 11.5% overlap; at referral, 29% met VEDOSS criteria, 60% met 2013 ACR/EULAR criteria), and 172 (39.5%) had secondary RP (sRP). NFC SSc pattern was detected in 80 (23%) pts: 43 with “early”, 31 with “active” and 6 with “late” pattern. 37 of the 52 patients with SSc had SSc pattern at NFC. Among the other patients with SSc pattern, 30 had sRP, 9 pRP and 4 no RP. Non-specific findings were detected in 8/52 patients with SSc, 62/172 patients with sRP, 33/69 patients with pRP and 21/54 patients with no RP. Of those without a clinical diagnosis of scleroderma (n=295), 41 (14%) met VEDOSS criteria following NFC and 4 (1.3%) the 2013 ACR/EULAR criteria for SSc. Of the 41 pts meeting VEDOSS, 28 (68%) were found to have a SSc NFC pattern. Considering the entire unselected cohort, the detection of SSc pattern on NFC had a sensitivity of 71%, specificity 95%, positive predictive value 84% and negative predictive value 90% for identifying pts who met the VEDOSS or 2013 ACR/EULAR criteria. Table 1. NFC patterns seen in pts referred to the Leeds NFC service Clinical n (%) NFC pattern SSc criteria met post diagnosis (n) NFC (n (%)) Normal Non- Early Active Late VEDOSS 2013 specific ACR/EULAR No RP 54 (15.6) 29 21 4 0 0 5 (9.3) 1 (1.9) pRP 69 (19.9) 27 33 9 0 0 0 (0) 0 (0) sRP 172 (39.5) 80 62 17 12 2 36 (21) 3 (2) Subgroup of sRP with features suspicious of SSc 98 (28.2) 49 36 9 3 1 15 (15.3) 3 (3.1) SSc 52 (15) 7 8 13 20 4 12 (23.1) 37 (71.2) Total 347 143 124 43 31 6 53 (15.3) 41 (11.8) Conclusions: Our specialised NFC service improved the detection of SSc in an unselected cohort of pts with RP or suspected CTD. Pts meeting the new ACR/EULAR criteria are more likely to have a SSC NFC pattern than those meeting VEDOSS criteria. The absence of SSc NFC pattern in pts with RP or suspected CTD is very valuable in the exclusion of SSc. Disclosure of Interest: None declared DOI: 10.1136/annrheumdis-2014-eular.1577 SAT0322 WITHDRAWN