HARMONY Registry

Systemic sclerosis (SSc) is a complex autoimmune disease affecting the skin, the gastrointestinal tract, the lungs, the kidneys and the heart. The disease’s main pathogenic features are the vasculopathia, the inflammation and the progressive fibrosis. Alterations of the respiratory system can be found in more than 90% of SSc patients. SSc is a rare disorder; hence the evidence based information on the disease is limited. 

The SSc related lung disease (ILD) – including pulmonary fibrosis, decreased spirometry parameters- are poor prognostic signs of the disease, most of the patients die due to the cardiopulmonary manifestation of the disease. The disease is characterized by overproduction of collagen tissue of many organs including the lungs. The pathogenesis of the overproduced collagen is not clearly understood, it is supposed that immunologic events, aspiration of stomach content, or smoking can trigger the inflammatory and fibrotic response.

Hyperplasia of the Type II pneumocytes can be observed in ILD, the cells produce a large amount of cytokines including Krebs von den Lungen (KL-6). This is a mucin-like protein, its concentration correlates with the severity of the ILD. The Type II pneumocytes also produce surfactant protein A and D (SP-A and SP-D). These biomarkers concentration show negative correlation with the results of the spirometry tests. It was previously proven that baseline KL-6 concentration is predictive for the latter prognosis. 

Our study aims to evaluate the prevalence, incidence and clinical features of ILD, its possible associations with accompanying autoimmune diseases. During the prospective data collection we aim to determine risk factors indicative for disease course or therapeutic response. As a long term plan we might analyze survival. We also aim to examine biomarkers with the help of the collected serum and plasma samples. 

Hopefully based on the abovementioned data we might gain new information giving a hand to further studies.