Renal dysfunction, malignant neoplasms, atherosclerotic cardiovascular diseases, and sarcopenia as key outcomes observed in a three-year follow-up study using the Werner Syndrome Registry.

Werner syndrome is an adult-onset progeria syndrome that results in various complications. This study aimed to clarify the profile and secular variation of the disease. Fifty-one patients were enrolled and registered in the Werner Syndrome Registry. Their data were collected annually following registration. A cross-sectional analysis at registration and a longitudinal analysis between the baseline and each subsequent year was performed. Pearson's chi-squared and Wilcoxon signed-rank tests were used. Malignant neoplasms were observed from the fifth decade of life (mean onset: 49.7 years) and were observed in approximately 30% of patients during the 3-year survey period. Regarding renal function, the mean estimated glomerular filtration rate calculated from serum creatinine (eGFRcre) and eGFRcys, which were calculated from cystatin C in the first year, were 98.3 and 83.2 mL/min/1.73 m2, respectively, and differed depending on the index used. In longitudinal analysis, the average eGFRcre for the first and fourth years was 74.8 and 63.4 mL/min/1.73 m2, showing a rapid decline. Secular changes in Werner syndrome in multiple patients were identified. The prevalence of malignant neoplasms is high, and renal function may decline rapidly. It is, therefore, necessary to carry out active and detailed examinations and pay attention to the type and dose of the drugs used.

A case of breakthrough Candida parapsilosis fungemia during micafungin therapy for a Candida glabrata bloodstream infection.

We describe a case of breakthrough Candida parapsilosis fungemia in an 80-year-old woman with pyoderma gangrenosum and rheumatoid arthritis. C. parapsilosis was detected in blood culture while the patient was treated with micafungin for a Candida glabrata bloodstream infection. The breakthrough infection was successfully treated with liposomal amphotericin B.

Significant attenuation of macrovascular involvement by bosentan in a patient with diffuse cutaneous systemic sclerosis with multiple digital ulcers and gangrene.

Systemic sclerosis (SSc) is characterized by vascular injuries, and bosentan has recently been proved to be efficacious for the prevention of new digital ulcers in SSc. We herein report a case of SSc in a patient with refractory digital ulcers and gangrene treated with bosentan. Stenosis of the ulnar artery, evaluated by magnetic resonance angiography, was attenuated by the bosentan treatment, suggesting that bosentan exerts a reverse remodeling effect against the pathological organic changes of arteries in SSc.

Disseminated cutaneous and visceral Kaposi's sarcoma in a patient with rheumatoid arthritis receiving corticosteroids and tacrolimus.

Kaposi's sarcoma (KS) is a vascular lesion of low-grade malignant potential caused by the complex interactions between geographic, genetic, environmental, and immunological factors. We recently experienced a rare case of KS associated with rheumatoid arthritis in a patient receiving corticosteroids and tacrolimus; the KS demonstrated unusually aggressive clinical behavior. We herein report the details of the clinical course and discuss the possible contribution of corticosteroids and tacrolimus to the development of aggressive KS in the present case.