A recently published study has found that a protein the in blood, called CC16, can predict lung disease progression in patients with systemic sclerosis. High levels of the protein are linked to lung disease deterioration. The full article can be read here, at Scleroderma News.

Systemic sclerosis (SSc) is a type of scleroderma. Scleroderma is a condition that causes areas of skin to become thickened and hard, and in patients with SSc the blood circulation and internal organs may also be affected. The condition occurs as a result of the immune system becoming over-active and producing too much collagen in the connective tissue under the skin, and around blood vessels and internal organs, which causes scarring and thickening. SSc more commonly affects women than men, and it usually appears between the ages of 30 and 50. In some people, SSc affects organs such as the lungs, heart, or kidneys, which can lead to problems such as pulmonary hypertension (high lung blood pressure) and shortness of breath.

The recent study, published in The Journal of Rheumatology, examined whether the blood protein Clara cell 16 kDa (CC16) could be used to predict the worsening of lung disease in patients with SSc. CC16 is a type of protein that is released into the blood when the lungs are damaged. As a result, CC16 levels can be used as a biomarker for lung damage, and some patients with lung damage, such as those with pulmonary fibrosis, are known to have raised levels of CC16.

However, it was not known if CC16 could also be used as a predictor of lung function for patients with SSc. The researchers prospectively (over time) followed 106 patients with SSc over a four-year period. Approximately half of the patients involved in the study had interstitial lung disease, and a third had cutaneous scleroderma.

After adjusting for age, clinical and lung function measures, and duration of the disease, it was found that patients with high CC16 levels were 2.9 times more likely to have rapid disease worsening than those with low CC16 levels. The researchers concluded, “High baseline serum CC16 predicts lung disease worsening in patients with SSc.”

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