Unusual liver-related heart injury.

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.

The arthropathy of systemic sclerosis: a 12 month prospective clinical and imaging study.

OBJECTIVE: To assess the clinical and radiological features of systemic sclerosis (SSc) joint involvement in a prospective cross-sectional study. DESIGN AND PATIENTS: Seventy-six consecutive patients with SSc divided into clinical and serological subsets were investigated. Clinical and radiological assessments of the hands and feet were carried out. Three radiological patterns of inflammatory, degenerative and fibrotic changes were predefined. The Health Assessment Questionnaire (HAQ) disability index (DI) and individual components of the HAQ-DI were also evaluated. RESULTS: The highest impairments on the HAQ-DI (median 0.44; range 0-2.87) were detected in subdimensions such as hygiene, grip and activity components. Clinically articular involvement, arthralgia and finger contractures were seen more frequently than arthritis, and a significantly higher prevalence of finger flexion was found in patients with diffuse cutaneous SSc (P=0.03) compared with the other SSc subtypes. Radiologically, distal interphalangeal joint space narrowing and flexion deformity indicating periarticular fibrosis were frequently detected. Juxta-articular osteoporosis, joint space narrowing and flexion contractures of the fingers were seen significantly more frequently in the hands. A significantly higher frequency of fibrotic pattern were found in the hands whereas a degenerative pattern was more frequent in the feet (P<0.05). Finally, significant correlations were detected between flexion contractures and a radiological fibrotic pattern (P<0.001), and the severity scores of peripheral vascular impairment (P=0.026) and skin (P=0.007). CONCLUSION: 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.