P038 Catastrophic antiphospholipid syndrome: a unique patient perspective
Authors: Webster Jemma E. S 
Published: April 2022

Background/Aims

Catastrophic antiphospholipid syndrome (CAPS) is a life-threatening manifestation of APS affecting 1:100 APS patients with a mortality of up to 50%. The rarity, severity, and rapid onset of the condition present significant challenges for clinicians, patients, and their families.

Methods

This is the case of a 59-year-old female who presented to A&E with severe anxiety and foreign body sensation in the left ear, following recent investigations for thrombocytopenia and suspected transient ischaemic attack. She rapidly deteriorated, becoming obtunded with a stage 3 acute kidney injury. Bloods revealed platelets 22, anticardiolipin IgG 398.9 and B2-glycoprotein 984.3. She was ANA positive (1:1280), Anti-Ro positive but ds-DNA negative. Within 48h she was intubated and ventilated, receiving renal replacement therapy, plasma exchange and methylprednisolone. Initial differentials included CAPS +/- undiagnosed systemic lupus erythematosus and thrombotic thrombocytopenic purpura. She was ultimately diagnosed with CAPS, complicated by Libman-Sacks endocarditis with severe aortic and mitral regurgitation, acute renal failure and multiple intracranial infarcts. She was given rituximab, IV immunoglobulins and enoxaparin titrated to platelet count.

She suffered no new organ damage following the initial insult. However, she remained unresponsive as sedation was weaned and her potential for neurological recovery remained uncertain. Multiple palliative discussions took place with her family. However, after 6 weeks of intubation on ITU she began to respond, going on to make a surprising recovery.

Results

Four months on she is at her local acute stroke unit for neurorehabilitation. Her APS is managed with hydroxychloroquine and warfarin (target INR of 3-4). She remains quadriparetic, with several cognitive processing difficulties. She displays emotional lability and extreme anxiety at times and struggles with rational thinking. Dealing with the trauma of this experience remains a daily struggle. She and her husband have provided a written account of their experiences before, during and after her stay on intensive care. They give an honest and thought-provoking perspective on the personal impact of CAPS and the difficulties in dealing with prognostic uncertainty in rare, life-threatening conditions. Her husband reflects on the palliative discussion process and the ways in which this experience might have been made easier for him by our medical teams. In his own words “telling the boys that their mum was not coming home was the worst day of my life”. Though her recovery represents a significant success for all those involved in her care, their lives have been irreparably altered by the course of her illness.

Conclusion

This case provides a novel perspective on the diagnosis and management of CAPS. It also provides a unique opportunity for clinicians to reflect on how best to communicate with patients and their families in cases with significant prognostic uncertainty, via the eloquent perspectives of the patient and her husband.

Disclosure

J.E.S. Webster: None.