Survival in SLE-Related Pulmonary Arterial Hypertension Linked to Autoimmune Antibodies

Pulmonary Arterial Hypertension CT Scan
Pulmonary Arterial Hypertension CT Scan
Anti-U1 ribonucleoprotein antibodies may increase chances of survival in patients with pulmonary arterial hypertension as a complication of systemic lupus erythematosus.

Hydroxychloroquine and anti-U1 RNP (ribonucleoprotein) antibodies may play a protective role in overall survival in patients with systemic lupus erythematosus (SLE) and pulmonary arterial hypertension (PAH), according to results from a retrospective study published in Chest.1

The researchers identified 51 patients with SLE-PAH from the French Pulmonary Hypertension Registry. All patients included in the analysis had high anti-Sjögren’s-syndrome-related antigen A (SSA) and anti-Sjögren’s syndrome type B (SSB) antibodies. There was a median of 4.9 years between SLE diagnosis and PAH diagnosis.

At 6 months after PAH diagnosis, 49% of patients were prescribed cyclophosphamide and 39.2% of patients were prescribed corticosteroids. The 3- and 5-year survival rates were 89.4% (95% CI, 76.2%-96.5%) and 83.9% (95% CI, 68.8%-92.1%), respectively.

A trend toward better survival was observed in patients who received hydroxychloroquine either at the time of or 6 months after PAH diagnosis compared with patients who did not receive the medication (hazard ratio [HR],0.31; 95% CI, 0.09-1.11; P =.07).

At 10 years, 11 patients died; 9 died from cardiopulmonary complications 2 died from infection. In patients with anti-U1-RNP antibodies, survival was significantly better compared with patients without anti-UL-RNP antibodies (P =.04).

According to the investigators, the main limitations of this study were its retrospective and observational design and its small sample size. In addition, some data regarding auto-antibody testing may have been overlooked as a result of the study’s retrospective nature; therefore, the researchers urged exercising caution when determining the effects of therapies and risk factors for death.

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Despite these limitations, the researchers noted that “the data regarding survival are robust (51 patients over a 10-year period) and were not affected by the study design.”

Disclosures: Several researchers disclosed financial relationships with various pharmaceutical companies, including Actelion, Bayer HealthCare, GlaxoSmithKline, Merck, Novartis, Pfizer, and United Therapeutics, among others.


Hachulla E, Jais X, Cinquetti G, et al. Pulmonary arterial hypertension associated with systemic lupus: results from the French Pulmonary Hypertension Registry [published online August 26, 2017]. Chest. doi:10.1016/j.chest.2017.08.014