Session Type: ACR Poster Session C
Session Time: 9:00AM-11:00AM
Background/Purpose: Patients with connective tissue diseases (CTDs) positive for anti-U1RNP antibody (U1) is characterized by overlapping feature of systemic sclerosis (SSc), systemic lupus erythematosus (SLE), and/or polymyositis/dermatomyositis (PM/DM). Anti-U1RNP antibody is reported to associate with development of pulmonary arterial hypertension (PAH), which is an important organ involvement that determines the prognosis of these patients. The aim of this study was to clarify whether the antibody profile or underlying disease component can determine the characteristic of PAH in this group of patients.
Methods: Thirty-two patients with U1(+)CTD-PAH were involved and 7 U1(-)SLE patients complicated with PAH were used as controls. Patients with SSc, SLE, PM/DM were fulfilled 2013 ACR/EULAR classification criteria, 2012 SLICC classification criteria, and Bohan and Peter’s criteria, respectively. Baseline clinical information retrospectively collected from records, the changes of parameters of right heart catheterization (RHC) within 12 months as a response to the initial treatment and survival rate were compared among patients with ovelap syndrome (OL), U1(+)SLE and U1(-) SLE.
Results: Of 32 patients with U1(+)CTD-PAH, underlying diseases were OL (58 %), SLE (29 %), Sjogren’s syndrome (9 %), and SSc (6 %). Interestingly, all patients with OL fulfilled criteria of SSc and SLE components. Age at diagnosis of PAH was tended to be higher in OL (44 ± 14 years) compared to U1(+)SLE (36 ± 14 years) or U1(-) SLE (35 ± 10 years), and frequency of having ILD was higher in OL compared to U1(+) or U1(-)SLE (72 % versus 11 %, P < 0.001 or 14 %, P < 0.03, respectively) . The change of RHC parameters from baseline such as mean pulmonary arterial pressure (-3.3 ± 8.4 versus -13.3 ± 10.0 mmHg, P < 0.04), pulmonary vascular resistance (0.2 ± 9.2 versus -5.5 ± 2.0 wood units, P < 0.05), or cardiac output (0.4 ± 1.6 versus 1.0 ± 2.3 L/min) were worse in patients with OL compared to those with U1(+)SLE. There was no difference in survival rate among patients with OL, U1(+)SLE, and U1(-)SLE. However, all 4 deceased cases with SLE-PAH, both U1(+) and U1(-), were observed within 13 months, but those with OL-PAH were distributed through the course and only 1 of 6 patients died within 13 months (P < 0.05).
Conclusion: Characteristic of PAH complicated with OL patients, who had clinical features of SSc and SLE, was different from those with SLE patients, irrespective of U1-positivity, suggesting that clinical characteristic of PAH complicated with CTD patients with anti-U1RNP antibody was determined by underlying disease components rather than autoantibody profile.
To cite this abstract in AMA style:Yasuoka H, Takei H, Shirai Y, Yamaoka K, Kuwana M, Takeuchi T. Characteristic of Pulmonary Arterial Hypertension in Patients with Anti-U1RNP Antibody-Positive-Connective Tissue Diseases Is Determined By the Underlying Disease Rather Than Autoantibody Profile [abstract]. Arthritis Rheumatol. 2017; 69 (suppl 10). http://acrabstracts.org/abstract/characteristic-of-pulmonary-arterial-hypertension-in-patients-with-anti-u1rnp-antibody-positive-connective-tissue-diseases-is-determined-by-the-underlying-disease-rather-than-autoantibody-profile/. Accessed February 21, 2018.
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