They include progressive SSc (increasing skin score or declining pulmonary function), age at onset <40 years, male gender, antibodies against topoisomerase I or RNA polymerase III, elevated level of C-reactive protein (>10 mg/l), recurrent digital ulcers, tendon friction rubs, weight loss (>10% during the past three months), active myositis on MRI or elevated creatine kinase levels (>2 x ULN), early (during the first year) lung manifestations with DLCO <60% and/or FVC <70% and cardiac manifestations with elevated troponin levels and/or myocardial involvement [54, 56]. Here, CRP is linked to myositis disease.