Catastrophic antiphospholipid syndrome is a rare multisystem autoimmune condition characterised by rapid development of widespread thrombotic disease and subsequent multi-organ failure. It is the most severe complication of antiphospholipid syndrome, carrying significant morbidity and mortality. We report a patient with post-partum catastrophic antiphospholipid syndrome with cardiac, hepatic, renal and cutaneous manifestations. The diagnostic challenges in establishing a definitive diagnosis in catastrophic antiphospholipid syndrome is discussed, along with the difficulties in managing these patients in the intensive care unit.
Background
Recent literature emanating from the United Kingdom and United States has reported decreasing mortality rates in patients with decompensated cirrhosis and organ failures presenting to the intensive care unit (ICU).
Aim
To determine if there were comparable outcomes in a single‐centre non‐transplant unit in Australia.
Methods
A retrospective observational study was conducted in a tertiary, non‐liver transplant unit in Sydney, Australia. Admission data and mortality outcomes were collected from patients with cirrhosis non‐electively admitted to ICU between 2013 and 2017. Liver‐specific and general intensive care scoring tools were also assessed for their discriminative ability to predict short‐term prognostic outcomes.
Results
Sixty‐three patients were admitted with decompensated liver disease who fulfilled the inclusion criteria. The overall hospital mortality was 37% (95% CI: 0.26–0.49). There was no difference in survival based on aetiology of liver disease (P = 0.96) but a significant difference was found based on the presenting diagnosis, with greater survival among patients diagnosed with hepatic encephalopathy on ICU admission (P = 0.02). There was 4% mortality in patients with no organ failure and 52% mortality in those with ≥3 organs in failure (P < 0.001). The ICU prognostic Sequential Organ Failure Assessment score was the better discriminative tool in predicting short‐term outcomes when compared to liver prognostic scores.
Conclusion
The outcomes of this single‐centre Australian study align with current overseas literature. These results reinforce and expand on limited local evidence, corroborating the former universal prognostic pessimism towards cirrhotic patients with organ failure as unwarranted.
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