Pulmonary Hypertension Often Occurs Early After SSc-ILD Diagnosis

Investigators examined the prevalence, characteristics, treatment, and outcomes for patients with pulmonary hypertension in a systemic-sclerosis-associated interstitial lung disease cohort.

A significant proportion of patients with systemic sclerosis-associated interstitial lung disease (SSc-ILD) develop pulmonary hypertension (PH) early in the course of their SSc disease, according to results of a prospective observational cohort study published in Arthritis & Rheumatology.

Participants with SSc-ILD, who were assessed using high-resolution computed tomography, were included in the study. All patients had been screened for PH using a standardized algorithm and, if indicated, also underwent right heart catheterization. A total of 93 participants ≥18 years were included in the study, of whom 76.3% were women and 65.6% had diffuse SSc. The mean participant age was 54.9 years and the mean duration of SSc was 7.9 years.

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Overall, 31.2% of the patients had right heart catheterization-proven PH. Of these, 24.1% had pulmonary arterial hypertension (PAH), 55.2% had World Health Organization (WHO) group III PH, 34.5% had WHO group III PH with pulmonary vascular resistance >3 Wood units, 48.3% had received a diagnosis of PH within 7 years of SSc onset, 82.8% received treatment with ILD therapy, and 82.8% were treated with PAH therapy.

Among all participants, the survival rate 3 years after a diagnosis of SSc-ILD was 97%. In addition, the survival rate among those with SSc-ILD and PH at 3 years after a diagnosis of PH was 91%.

The investigators concluded that in this cohort, a significant percentage of patients had coexisting PH, which frequently occurred early after diagnosis. They note that it is important for all patients with SSc-ILD to be evaluated for the presence of coexisting PH. Future prospective studies on the subject are warranted to confirm the findings from this analysis.


Young A, Vummidi D, Visovatti S, et al. Prevalence, treatment and outcomes of coexistent pulmonary hypertension and interstitial lung disease in systemic sclerosis [published online February 14, 2019]. Arthritis Rheumatol. doi: 10.1002/art.40862