Session Information
Session Title: Systemic Sclerosis, Fibrosing Syndromes, and Raynaud’s - Clinical Aspects and Therapeutics II
Session Type: Abstract Submissions (ACR)
Background/Purpose:
Pulmonary hypertension (PH) is a leading cause of morbidity and mortality in patients with connective tissue diseases (CTD). Patients with anti-U1-RNP antibodies belong to a heterogeneous group, including systemic sclerosis (SSc), systemic lupus erythematosus (SLE) or mixed connective tissue disease (MCTD). This study aimed to (i) describe the prevalence, clinical and hemodynamic characteristics of PH in U1-RNP positive patients and (ii) analyse survival and compare prognosis between the different CTDs associated with these antibodies.
Methods: In this National Pulmonary Hypertension database, more than 2000 patients had a right heart catheterisation (RHC) performed between 1998 and 2012. One hundred and twelve patients were tested anti-U1-RNP positive. Twenty-two patients did not have PH (mean Pulmonary Arterial Pressure [mPAP] < 25 mmHg). In the 90 patients with PH, 10 had post-capillary PH (pulmonary capillary wedge pressure [PCWP] > 15 mmHg). Eighty had pre-capillary PH (PCWP ≤ 15 mmHg). Six patients were excluded because of incomplete RHC data. Thirty-seven had PH due to lung disease (PH-ILD) and 37 had pulmonary arterial hypertension (PAH). Anti-U1-RNP positive patients with PH were compared with unselected U1-RNP negative patients also having PH: 387 SSc and 21 SLE. For binary variables, comparison between 3 PH groups was performed using Monte-Carlo method with Fischer test for comparison one by one. ANOVA was used to compare continuous variables with a normal distribution. Kruskal-Wallis test was used for non-parametric comparisons. Kaplan-Meier method with log-rank test was used for survival analysis. All statistical analyses were performed using SPSS for Microsoft Windows®.
Results: The proportion of patients with SSc, SLE or MCTD was not significantly different between the 3 groups of PH. Anti-Sm antibodies were only seen in PH-ILD (p=0.038). Six-minute walking distance and WHO FC were significantly better in PAH vs. PH-ILD (p=0.003 and p=0.007 respectively). Mean PAP was similar in 3 groups. In U1-RNP negative patients, mPAP was significantly higher in SLE than in SSc patients (p=0.003). In PAH patients, SLE patients had higher mPAP than others groups (p=0.023). When comparing U1-RNP positive vs. negative in SSc or SLE patients, no difference was observed in hemodynamic values. Among PH-ILD patients, U1-RNP negative SSc had lower DLCO than U1-RNP positive (p=0.001). MCTD patients had lower CI (p=0.007) and higher PVR (p=0.05) than U1-RNP negative SSc patients. SLE and MCTD had a better survival than SSc (p=0.005 and p=0.037, respectively). PH-SSc patients carrying the U1-RNP antibody tended to have a better survival than U1-RNP negative (p=0.065). This trend was found in PAH patients (p=0.068) but not in PH-ILD patients (p=0.225).
Conclusion: These data confirm that patients with anti-U1-RNP antibodies are heterogeneous. To our knowledge, this is the largest cohort of patients with PH and MCTD. It provides hemodynamic data for all patients at PH diagnosis. In this group, patients with SSc have the worse prognosis. Interestingly, survival of patients fulfilling MCTD classification criteria is intermediate between SSc and SLE.
Disclosure:
V. Sobanski,
None;
B. Lynch,
None;
B. E. Schreiber,
None;
S. I. Nihtyanova,
None;
J. Harvey,
None;
C. Handler,
None;
C. P. Denton,
None;
J. G. Coghlan,
None.
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