EUSTAR Study on Early SSc and Organ Manifestations Presented at Systemic Sclerosis World Congress

A EUSTAR study on organ manifestations in the early course of systemic sclerosis (SSc) was presented today, Feb. 19, by Dr. V.K. Jaeger at the 4^th Systemic Sclerosis World Congress, now underway in Lisbon, Portugal. The presentation was titled “Incidences and Predictors of Organ Manifestations in the Early Course of Systemic Sclerosis: A Longitudinal EUSTAR Study.” Scleroderma News Today is covering the event.

Systemic sclerosis is a rare, heterogeneous autoimmune disease characterized by fibrosis and micro-vascular obstruction in the skin and internal organs, typically organs of the digestive tract, kidneys, lungs, and heart. Patients with the condition can also experience Raynaud’s phenomenon (RP) – a reversible vasculopathy characterized by excessive reduced blood flow as a response to cold or emotional stress, usually in the fingers and toes.

Patients with early SSc – defined as those who had a visit within the first year since their first RP episode – were studied in this research. Researchers analyzed outcome measures as a function of time after RP onset, and possible predictors of incident organ manifestations.

The research team used data from the large, international EUSTAR (European scleroderma trials and research) cohort of data collected between 2004 and 2014, and analyzed the incidence and predictors of pulmonary, cardiac, gastrointestinal (GI), and renal involvement in the early course of systemic sclerosis.

Of the 695 SSc patients evaluated with a baseline visit within a year after RP onset, the incident non-RP manifestations were:

• Skin sclerosis (75 percent);
• GI symptoms (71 percent);
• Impaired diffusing capacity for carbon monoxide of less than 80 percent predicted (65 percent);
• Digital ulcers (34 percent);
• Cardiac involvement (32 percent);
• Impaired forced vital capacity (FVC) of  less than 80 percent predicted (31 percent);
• Increased resting systolic pulmonary artery pressure estimated by echocardiography (PAPsys) greater than 40 mmHg (14 percent);
• Renal crisis (3 percent).

The researchers found that:

1. Older age and anti-topoisomerase positivity were predictors of incident cardiac manifestations;
2. Male gender and older age were predictors of incident PAPsys  greater than 40 mmHg;
3. Diffuse skin involvement was a significant predictor of incident FVC impairment;
4. Diastolic dysfunction saw the highest incidence rates, closely followed by conduction blocks and pericardial effusion;
5. Renal crisis was predicted by male gender, anti-RNA-polymerase-III positivity and older age.

“In every organ system, half of all organ manifestations that evolved during the 10-year observation period became evident within the first two years after RP onset,” Jaeger said in the presentation.

The findings might have implications in the design of new diagnostic tests and therapeutic strategies for systemic sclerosis patients, and may also allow physicians to better manage and counsel systemic sclerosis patients at an early stage.