Early Treatment of Systemic Sclerosis Symptoms Leads to Better Outcomes

Good early management of pulmonary hypertension in patients with systemic sclerosis leads to better outcomes and fewer complications of systemic sclerosis, researchers report.

Furthermore, increases in pulmonary vascular resistance and mean pulmonary artery pressure predict poor outcomes while six minute walking distance and lung diffusing capacity predict long-term survival in patients with pulmonary hypertension and systemic sclerosis, researchers from Stanford University recently reported at the 2016 American College of Rheumatology annual meeting.

Other studies have described various subsets of systemic sclerosis patients with pulmonary hypertension, specifically World Health Organization Group II patients. However, none have examined such a large prospective database in an effort to discover predictors of survival and clinical worsening long-term.

This prospective study examined data from the Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma Registry and included 167 patients with systemic sclerosis associated pulmonary hypertension. The subjects were classified with World Health Organization Group I pulmonary hypertension. Time to clinical worsening (TTCW), cause of death, and survival at 1, 3, 5, and 8-year intervals were collected.

The authors found that the median time to clinical worsening was 4.15 years (95% CI 3.07-5.94).  The fraction of patients experiencing clinical worsening at 1, 3, 5, and 8 years was 23%, 40%, 57%, and 70% respectively. Predictors of clinical worsening included high pulmonary vascular resistance (PVR) defined as greater than 4.76 woods units (95% CI 1.14-3.68), and mean pulmonary artery pressures above 34 mmHg (CI 1.16-3.79).

Survival at 1, 3, 5, and 8 years was 95%, 76%, 64%, and 50% respectively with the bulk of deaths in the short and intermediate term being attributed to pulmonary arterial hypertension (62% and 60%) and in the long term due to non-systemic sclerosis related causes.

Finally, strong predictors of long-term survival included a 6-minute walking distance greater than 165 meters (95% CI 0.16-0.88) and percentage of predicted lung diffusing capacity greater than 40% (95% CI 0.23-0.89).

Key Points

• The Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma Registry is a large prospective database.
• Patients with systemic sclerosis and world health organization group 1 pulmonary hypertension have survival rates at 1, 3, 5, and 8 years of 95%, 76%, 64%, and 50% respectively.
• Causes of death in the short and intermediate time frames are pulmonary arterial hypertension and over the long-term, non-systemic sclerosis causes.
• Clinical worsening can be predicted when high pulmonary vascular resistance (>4.76 woods units) or high mean pulmonary artery pressures (>34mmHg) are present.
• 6-minute walking distances greater than 165m and lung diffusing capacity greater than 40% of predicted predict long term survival in patients with systemic sclerosis and pulmonary hypertension.

References:

“Predictors of Long-Term Outcomes in Systemic Sclerosis Associated Pulmonary Arterial Hypertension from the Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma Registry,” Kathleen Kolstad. Nov. 16, 2016. 2016 American College of Rheumatology annual meeting. Abstract 3246.