Poor prognosis with systemic sclerosis, pulmonary hypertension, and interstitial lung disease

Survival is reduced significantly in patients who have systemic sclerosis (SSc) with pulmonary hypertension (PH) combined with advanced interstitial lung disease (ILD) compared with patients with SSc who have isolated pulmonary arterial hypertension (PAH). The prognosis for patients in the former subset is extremely poor.

Mathai and coworkers reviewed data from 59 patients with PAH or ILD-associated PH (n = 20) confirmed by right heart catheterization. Results of pulmonary function tests and high-resolution CT of the chest were recorded. Variables assessed as risk factors included pulmonary hemodynamic features, pulmonary function, diagnosis, SSc disease type, and comorbidities.

Outcomes were worse for persons with ILD-associated PH than with PAH. The 1-, 2-, and 3-year survival rates were 82%, 46%, and 39% in persons with PH versus 87%, 79%, and 64% in those with PAH, respectively. The risk of mortality increased 5-fold for those with ILD-associated PH compared with others. Worsening hemodynamic factors and increased pulmonary vascular resistance contributed to increased mortality, suggesting that earlier treatment may benefit this patient group.

The authors noted that more studies are needed to determine which strategies, if any, might improve the prognosis for these patients.