Fat embolism syndrome: an autopsy-proven case involving a patient on dialysis and systemic scleroderma.

A 66-year-old woman receiving continuous ambulatory peritoneal dialysis developed acute respiratory distress 12 hours after a fall. Blood gas analysis revealed hypoxia (PaO2 67.7 torr) and metabolic acidosis with an increased anion gap, consistent with lactic acidosis (lactate, 86.5 mg/dL; normal range, 4.0-16.0). Magnetic resonance imaging showed a lumbar vertebral body fracture. On the fourth hospital day, the patient died of multiorgan failure and disseminated intravascular coagulation. Postmortem studies revealed fat emboli in the systemic circulation, ie, fat embolism syndrome. Diagnosing fat embolism syndrome can be difficult in patients on dialysis or in those with collagen vascular or pulmonary diseases.

Retrospective analysis of factors predicting end-stage renal failure or death in patients with microscopic polyangiitis with mainly renal involvement.

BACKGROUND: The aim of this study was to identify risk factors for end-stage renal failure (ESRF) or death in Japanese patients with microscopic polyangiitis (MPA) with renal involvement. METHODS: From 54 consecutive patients with systemic vasculitis based on Watt's algorithm, we retrospectively analyzed 39 MPA patients with renal involvement, including 19 (48.7 %) with renal-limited vasculitis. RESULTS: Thirty-three of 39 patients (84.6 %) demonstrated rapidly progressive glomerulonephritis, and 13 (33.3 %) developed ESRF; 8 of 13 required dialysis within 1 week. Thirteen (33.3 %) died during follow-up of more than 12 months, and 7 died during the first 6 months, mainly because of opportunistic infections. Overall survival at 6 and 12 months was 79.5 and 71.1 %, respectively. Serum creatinine levels did not differ significantly between survivors and non-survivors (P = 0.092). The mean Birmingham Vasculitis Activity Score, version 3 (BVAS v.3), was 16.2 ± 6.5, with a renal subscore of over 12 points in 82.1 %, and BVAS v.3 was marginally higher in non-survivors than survivors (P = 0.045). An age- and sex-adjusted Cox proportional hazards analysis demonstrated that neither the serum creatinine level (P = 0.277) nor BVAS v.3 (P = 0.188) at initial diagnosis was a risk factor for overall survival. The baseline serum creatinine cutoff value for discriminating between ESRF and non-ESRF was 4.6 mg/dl, with a sensitivity and specificity of 92.3 and 84.6 %, respectively. CONCLUSIONS: Survival rates do not relate to ESRF in MPA patients with mainly renal involvement. Although patients with ESRF required regular hemodialysis, longer survival can be achieved.