Arthritis & Rheumatism, Volume 62,
November 2010 Abstract Supplement

Abstracts of the American College of
Rheumatology/Association of Rheumatology Health Professionals
Annual Scientific Meeting
Atlanta, Georgia November 6-11, 2010.

Immunosuppression in the Treatment of Systemic Lupus Erythematosus Associated Pulmonary Arterial Hypertension: Improvement in Functional and Exercise Capacity.

Ashouri2,  Judith, Sung2,  Yon, Zamanian2,  Roham, Chung1,  Lorinda

Stanford Univ Medical Center, Palo Alto, CA
Stanford University School of Medicine


Accumulating evidence suggests that autoimmunity and inflammation are important in the pathogenesis of systemic lupus erythematosus associated pulmonary arterial hypertension (SLE-APAH). Several case reports and series have described improvement in dyspnea, exercise capacity, and hemodynamics with aggressive immunosuppression (IMM), both alone and in combination with PAH-specific therapies. We aimed to assess the effects of IMM in combination with PAH-specific therapies on the clinical outcomes of SLE-APAH patients followed at our center.


We identified 13 patients from Stanford's PAH database diagnosed with PAH by right heart catheterization between 1999–2009. All patients fulfilled ACR criteria for a diagnosis of SLE based on medical record review, and patients who fulfilled ACR criteria for another connective tissue disease were excluded. All patients received PAH-specific therapies according to standard of care. Patients who underwent aggressive IMM for a minimum of 8 months between the baseline and follow-up evaluations were compared with those receiving PAH-specific therapies alone. Cumulative probability of freedom from worsening New York Heart Association functional class (FC) was calculated using the Kaplan-Meier estimator, and compared using the log-rank test. Follow-up time was calculated from the time of the baseline evaluation. The mean changes in clinical parameters were compared between the IMM and non-immunosuppression (non-IMM) groups using Student's t-test.


All patients were female, 69% Asian, 15% white, with a mean age of 38±9 years and mean SLE disease duration at PAH diagnosis of 5.6±6.1 years. 46% had a history of renal disease, 46% had Raynaud's, and 38% a history of serositis. 100% were ANA+, 85% anti-dsDNA+, 71% anti-RNP+, 40% anti-Smith+, and 29% antiphospholipid antibody+. 4 (30%) patients were treated with aggressive IMM (3 with high dose steroids and mycophenolate mofetil, 1 with hematopoietic stem cell transplant) during a mean follow-up time of 14.8±4.3 months. At baseline, there were no significant differences in demographics, SLE clinical features or autoantibodies in patients treated with or without IMM, except for increased prevalence of oral ulcers in the IMM group (23% vs. 8%, p=.02). Baseline PAH disease duration (9±15 vs. 35±42 months), hemodynamics (mean right atrial pressure 11.3±6.1 vs. 7.5±4.9 mmHg, mean pulmonary arterial pressure 46±12.7 vs. 51.3±16.1 mmHg, cardiac index 2.6±0.7 vs. 2.3±0.6, peripheral vascular resistance 10.1±2 vs. 12.4±6.1 WU), FC (3.3±0.5 vs. 2.7±0.9), 6 minute walk distance (6MWD, 276±168 vs. 397±177 m), and PAH-specific therapies did not differ significantly between the IMM and non-IMM groups. At follow-up, hemodynamics and PAH-specific therapies did not differ between the groups. No patients in the IMM group had worsened FC compared with 22% and 45% at 1- and 3-years in the non-IMM group (p=.2). Mean improvements in FC (-2±1 vs. 0±1, p=.01) and 6MWD (+206±46 vs. +52±200 m, p=.054) were higher in the IMM group.


Immunosuppression in addition to PAH-specific therapies may improve functional and exercise capacity in patients with SLE-APAH.

To cite this abstract, please use the following information:
Ashouri, Judith, Sung, Yon, Zamanian, Roham, Chung, Lorinda; Immunosuppression in the Treatment of Systemic Lupus Erythematosus Associated Pulmonary Arterial Hypertension: Improvement in Functional and Exercise Capacity. [abstract]. Arthritis Rheum 2010;62 Suppl 10 :1157
DOI: 10.1002/art.28923

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