Patients with systemic sclerosis (SSc) and early-diagnosed pulmonary arterial hypertension (PAH) have a substantial rate of disease progression over a relatively short period of time and may require close follow-up, according to the results of the longitudinal stage of the DETECT study, published in Annals of the Rheumatic Diseases.

Patients with SSc-PAH who were enrolled in the DETECT study were followed for up to 3 years. Univariable logistic regression analysis was used to evaluate baseline parameter associations with disease progression. The median follow-up time was 12.6 months (range, 2.4-35.8 months).

Among the 57 patients with PAH for whom longitudinal data were available, PAH was progressive in 25 (43.9%) and stable in 32 (56.1%). 


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Disease progression was defined as the occurrence of ≥1 of the following events: worsening in World Health Organization Functional Class since enrollment, use of combination therapy for PAH, PAH-related hospitalization, and death from any cause. During follow-up, 24 of the 25 patients with progressive PAH and 28 of the 32 individuals with stable PAH received PAH treatment.

The following factors were associated with disease progression: male gender (odds ratio [OR], 4.085; 95% CI, 1.181-14.128; P =.026); high forced vital capacity %/carbon monoxide lung diffusion capacity (DLCO)% predicted ratio (OR, 3.608; 95% CI, 1.223-10.647; P =.020); high Borg dyspnea index (OR, 1.685; 95% CI, 1.092-2.598; P =.018); and low DLCO% predicted (P =.008).

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Based on the study findings, the investigators suggest that the presence of even mild PAH should be regarded as a possible high-risk complication of SSc.  

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Reference

Mihai C, Antic M, Dobrota R, et al. Factors associated with disease progression in early-diagnosed pulmonary arterial hypertension associated with systemic sclerosis: longitudinal data from the DETECT cohort [published October 23, 2017]. Ann Rheum Dis. doi:10.1136/annrheumdis-2017-211480