Scleroderma patients with combined pulmonary hypertension and interstitial lung disease

Objective. Most studies differentiate scleroderma associated pulmonary hypertension and interstitial lung disease (ILD) as 2 separate pathological processes, concentrating on one or the other; however, many patients have both conditions. We studied the demographics, clinical features, and prognosis of individuals with both vascular and interstitial lung disease. Methods. A retrospective cross-sectional study of 619 patients with scleroderma who had echocardiograph and pulmonary function testing performed within 6 months of one another. Echocardiography determined the presence of pulmonary hypertension, and pulmonary function testing documented restrictive ventilatory defect (RVD) as a marker of ILD. Results. Among the study group, 139 (22.5%) patients had isolated RVD; 119 (19.2%) isolated pulmonary hypertension; and 112 (18.1%) patients had combined RVD and pulmonary hypertension. The individuals with combined RVD and pulmonary hypertension resembled patients with isolated RVD in that they had a high prevalence of diffuse skin involvement and antitopoisomerase positivity, but they were older at diagnosis and at disease onset (p < 0.01). Among those with mild RVD, 39.2% had pulmonary hypertension compared to those with severe RVD, in whom 51.4% had pulmonary hypertension. Compared to those without pulmonary disease, the mortality risk ratio for patients with isolated pulmonary hypertension, combined RVD and pulmonary hypertension, and isolated RVD was 2.9, 2.4, and 1.61, respectively. Conclusion. Patients with combined scleroderma lung disease features are more likely to have diffuse disease, represent older patients, and have a prognosis similar to individuals with isolated pulmonary hypertension, and may represent a distinct subpopulation of scleroderma.