Scleroderma renal crisis accompanied by new-onset pulmonary arterial hypertension: an acute systemic endothelial injury? Case report and literature.

Systemic Sclerosis (SSc) is a multisystem connective tissue disease characterized by fibrosis of the skin and internal organs and extensive vasculopathy. We report a case of a 41 year-old white woman with a 10 year-history of limited scleroderma, who developed the rare combination of Scleroderma Renal Crisis (SRC) and Systemic Sclerosis related Pulmonary Arterial Hypertension (SScPAH) in the same time. Although the patient received the proposed antihypertensive treatment, the renal function did not recover, and she initiated on renal replacement therapy. SRC and SScPAH are two aspects of SSc vasculopathy characterized by endothelial dysfunction mediated by endothelin-1 and other vasoactive hormones. Further new studies with therapies directed towards the underlying mechanisms of SRC (i.e. endothelin-receptor antagonists), which are proven helpful in SScPAH, should take place to establish new therapeutic options and improve prognosis of these patients, for which our therapeutic armamentarium is currently poor.

Efficacy of omega-3 fatty acids, atorvastatin and orlistat in non-alcoholic fatty liver disease with dyslipidemia.

AIM: To evaluate the efficacy and safety of three hypolipidemic agents in patients with non-alcoholic fatty liver disease associated with hyperlipidemia. METHODS: Patients with dyslipidemia (Fredrickson type IIb), asymptomatic persistent transaminasemia lasting 24 weeks, and evidence of hepatic fat infiltration on ultrasonography and liver biopsy were studied. Those with predominant hypertriglyceridemia received omega-3 fatty acids (5 mL thrice daily) (Group A), those with predominant hypercholesterolemia received atorvastatin 20 mg/daily (Group B), and overweight patients received orlistat 120 mg thrice daily before meals (Group C). After 24 weeks of treatment, serum transaminase and lipid levels and liver ultrasonography were repeated. RESULTS: Serum transaminase levels decreased significantly (p< 0.001) in all groups but the decrease was more marked in Group C (AST 75 [16] to 31 [7] IU/L; ALT 120 [38] to 41 [10] IU/L) than in Group A (AST 70 [14] to 41 [6]; ALT 110 [20] to 68 [12]) or Group B (AST 68 [13] to 46 [9]; ALT 115 [22] to 76.6 [13]). After treatment, ultrasonography showed resolution of fatty liver in 35% of patients in Group A, 61% in Group B, and in 86% in Group C (p< 0.001, Group C vs. A). CONCLUSIONS: A decline in transaminase levels and normalization of ultrasonographic evidence of fatty liver were observed on treatment with omega-3 fatty acids in patients with hypertriglyceridemia, with atorvastatin in those with hypercholesterolemia, and orlistat in overweight patients with hyperlipidemia.