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EP.013
Catastrophic Antiphospholipid Syndrome: A Medical Complex Case of Multi-Organ Dysfunction following a Severe PET and HELLP Syndrome diagnosis at 21 weeks gestation

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Catastrophic Antiphospholipid Syndrome: A Medical Complex Case of Multi-Organ Dysfunction following a Severe PET and HELLP Syndrome diagnosis at 21 weeks gestation

Background

Antiphospholipid Syndrome (APS) is an acquired autoimmune disorder characterised by thrombosis (venous/arterial/small vessel) in the context of persistent antiphospholipid autoantibodies (aPL); namely anti-cardiolipin (α-CL), lupus anti-coagulant (LA) and anti-β2 glycoprotein-1 (anti-β2 GP-1). Among the many clinical manifestations of this condition are those presenting in the context of pregnancy. These include; recurrent fetal loss (first trimester and late), pre-eclampsia, HELLP (Haemolysis, Elevated Liver enzymes, Low Platelets) syndrome, eclampsia, and intra-uterine growth restriction1,2.  APS can be classed as primary or secondary, with the latter presenting alongside other autoimmune conditions such as systemic lupus erythematosus (SLE). ‘Catastrophic’ Antiphospholipid Syndrome (CAPS) is a rare accelerated form of APS in which multi-organ failure ensues over a period of days-to-weeks secondary to small vessel occlusion at multiple sites. It carries a mortality rate of approximately 50%3. Triggers include infection and surgery. Diagnosis can be challenging, but essentially requires a known history of APS or aPL, the involvement of 3 or more organs, and the histologic confirmation of microthrombosis4.  High rates of HELLP syndrome and placental infarctions have been reported in cases of CAPS during pregnancy and the pueperium. Additional features include renal (renal thrombotic microangiopathy (TMA)), respiratory (e.g. acute respiratory distress syndrome (ARDS), respiratory failure, pulmonary embolism), and central nervous system (infarcts, haemorrhage, encephalopathy) pathology5. Management of CAPS includes anticoagulation, steroid administration, plasma exchange, intravenous immunoglobulins and immunosuppression.

Case Report

39 year-old G5P0 with known APS diagnosis following 4 early miscarriages (<8 weeks gestation) and positive anti-β2 GP-1 and LA. Aspirin dose was increased from 75mg to 150mg after uterine artery dopplers showed bilateral increased resistance and notching on the left side. Prophylactic Fondaparinux 2.5mg commenced at 6/40 (systemic reactions to enoxaparin and dalteparin).

Presented in May 2018 at 21+4/40 unable to tolerate oral intake, vomiting, diarrhoea and epigastric pain.

•Hb 136, Plts 159, WBC 12.1, ALT 126, lactate 1.6, CRP 24
•Temp 38.1; cultures taken and commenced on Tazocin.
•FBC repeated 1 hour later Hb 127, Plt 133, WBC 12.1
•PCR 280
•BP within normal limits

Following day (21+5/40) feeling short of breath & transferred to delivery suite HDU:

•Hb 123, Plt 53, WBC 10.1, ALT 456, PT 11.3, APPT 30.2, fib 4.5
•Further blood requested – LDH 692, haptoglobin <0.08 (0.3-2.00), Reticulocytes 133 (20-80)
•Blood film: “Thrombocytopenia. No platelet clumps or red cell fragments. Mild polychromasia and red cell anisopoikilocytosis with occasional tear drop poikilocytes. Neutrophils show toxic granulation. No blasts or primitive cells seen.”
•Developed an oxygen requirement
•Became hypertensive resistant to oral anti-hypertensives, BP >160/90.
•Commenced on IV labetalol infusion and MgSO4-
•Urinary catheter and fluid restriction implemented
•Normal chest x-ray, but given 20mg IV furosemide as felt clinically evidence of pulmonary oedema
•Reviewed by ITU team and given 100mg IV Hydrocortisone
•Diagnosis: Severe Pre-eclampsia → HELLP Syndrome
•Same day decision for termination of pregnancy with hysterotomy

Post-operative period:

•CTPA immediately post-op: No pulmonary embolism, mild bilateral pleural effusions associated with mild basal atelectatic change.
•Weaned off IV anti-hypertensives and commenced Amlodipine – BP stable.
•Ongoing pyrexias >38oC; trialled multiple IV antibiotics on microbiology advice. All cultures (blood/urine/faeces/swabs) negative.
•Ongoing vomiting and loose stool – Abdominal x-ray demonstrated ileus.
•CT Abdomen and Pelvis 8 days post-op: Post-surgical appearances with no significant collection seen.
•Central line sited (very difficult peripheral venous access) and dietetic input.
•Ultrasound liver: unremarkable
•Echocardiogram: Good bi-ventricular systolic function with no vegetations seen.
•Day 17 post-op: PICC line & TPN commenced.
•Day 20 post-op: 3 witnessed seizures, intubated and admitted to ITU. CT Head including venogram (figure 5) initially reported as normal, later addendum added re: PRES.
•Lumbar puncture: Normal.
•Hypophosphataemic (0.24) and Hypokalaemic (3.0) - ?refeeding syndrome.
•OGD – Reflux oesophagitis
•Antibiotics stopped and white-cell scan arranged (figure 6), when attempting cannulation for scan had an episode of possible loss of consciousness. In view of history, neurology recommended MRI brain (figures 3 & 4).
•Fondaparinux withheld in context of SAH, within 48 hours had developed chest pain and shortness of breath; CTPA (Figure 7) confirmed pulmonary embolism (PE).
•CT Head Angiogram: No evidence of an intracranial aneurysm or arteriovenous malformation.
•Recommenced Fondaparinux after discussion with neurology and MDT meeting.
•Discharged after 10 weeks as an inpatient.

Readmission:

•Nausea, pyrexia, loose stool. Diagnosed with C.difficile infection and acute kidney injury (figure 2) which worsened with nephrotic range proteinuria. Renal biopsy demonstrated thrombotic micro-angiopathy (TMA) affecting small arterioles, pronounced acute tubular necrosis (ATN), interstitial inflammation, and ischaemic & congested appearance to glomeruli.
•Avoided the need for renal replacement therapy. Discharged home on warfarin, lamotrigine, amlodipine and continues under the care of nephrology (CKD Stage 3), neurology and haematology and has been clearly advised to avoid future pregnancy.
 
Discussion
 

This case presented multiple challenges and highlighted the importance of multidisciplinary team working. It also demonstrates the difficulties faced in diagnosing CAPS in the context of pregnancy; from initial presentation with symptoms that could fit with viral gastroenteritis, to a rapidly evolving early-onset pre-eclampsia and subsequent HELLP syndrome, followed by failure of the CNS with evidence of PRES and renal failure. The co-existence of PE and SAH also presented a dilemma regarding management with anti-coagulation. Placental examination revealed scattered small infarcts, as is often seen in CAPS. Although the patient developed C.Difficile infection late in the course of her illness, we cannot attribute her preceding and persistent pyrexias to this source; fevers have been reported as part of a post-partum syndrome in the context of APS2  and we wonder whether her temperatures could have been secondary to a systemic inflammatory-type response rather than an infection.
As with many medical conditions in pregnancy, pre-pregnancy counselling and initiation of prompt and appropriate risk-reducing treatments is key. CAPS only affects <1% of patients with APS, and so the importance of learning from each case as a medical community is paramount to further our understanding of this devastating condition.

 

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