NEW YORK (Reuters Health) - The prognosis for patients with systemic sclerosis complicated by pulmonary hypertension is "grim," particularly among African Americans and those with interstitial lung disease, physicians at Johns Hopkins University, Baltimore, report in the February issue of Arthritis & Rheumatism.
Based on patients enrolled in their Pulmonary Hypertension Program, Dr. Reda E. Girgis and investigators suggest that "early diagnosis and treatment may improve outcomes, since worsening hemodynamic factors were associated with reduced survival."
Their study included 59 patients who started therapy after their condition was confirmed by right heart catheterization. Mean age was 60 years, 88% were female, and 20% were African American.
During a mean follow-up of 4.4 years, 30 patients died and 3 underwent lung transplantation.
Survival at 3 years was 39% in the 20 patients with interstitial lung disease-associated pulmonary hypertension, and 64% among the 39 who had isolated pulmonary arterial hypertension. In multivariate analysis, those with interstitial lung disease were 5 times more likely to die during follow-up than the rest of the cohort.
In the interstitial lung disease group, African American race and diffusing capacity for carbon monoxide were also associated with reduced survival.
"Such patients, in the absence of other significant comorbidity, should be considered early for lung transplantation," the investigators advise.
They add, "Given the dramatically worse long-term outcomes in patients with systemic sclerosis and pulmonary arterial hypertension, studies focusing on this group of patients are warranted."
Arthritis Rheum 2009;60:569-577.