This cross-sectional prospective study confirms that an arthropathy is common in SSc patients and shows that it is a major determinant of disability. A classification of radiological alterations into three specific patterns is proposed.

A 72‐year‐old man with polycystic liver disease and unexplained shock was admitted to our Emergency Department. The presence of turgidity in the jugular veins and acute prerenal kidney failure led to a possible hypothesis of right ventricular heart failure. A massive hepatic cyst resulted in right atrial compression and, secondarily, a state of shock. Surgical decompression by drainage of the hepatic cyst resulted in rapid improvement in the patient's hemodynamics. We report the description of an extremely rare complication of polycystic liver disease.