Catastrophic antiphospholipid syndrome: management challenges and lessons learnt in the third world set-up: Case report

Citation:
EK G, GO O. "Catastrophic antiphospholipid syndrome: management challenges and lessons learnt in the third world set-up: Case report." Afr J Rheumatol . 2015;2(3):67-72.

Abstract:

Background: Antiphospholipid
Syndrome (APS) is a disorder that
manifests clinically as recurrent venous
or arterial thrombosis and/or foetal loss.
Catastrophic Antiphospholipid Syndrome
(CAPS) is a very severe variant of the
classic APS. It is characterized by clinical
evidence of multiple organ involvement
developing over a very short period
of time, histopathological evidence of
multiple small vessel occlusions and
laboratory confirmation of the presence
of antiphospholipid antibodies, usually
in high titre. Although patients with
catastrophic APS represent less than 1%
of all patients with APS, this is usually a
life-threatening condition. The majority
of patients with catastrophic APS end
up in intensive care units with multiorgan
failure. Making the diagnosis is
challenging and can be missed. Unless the
condition is considered in the differential
diagnosis by attending physicians, it
may be completely missed, resulting in
a disastrous outcome. Catastrophic APS
develops rapidly and can result in death of
up to 30-50% of cases.
Case presentation: A nineteen year old
nulliparous lady diagnosed with Systemic
Lupus Erythematosus (SLE) four months
prior to admission with no prior history of
thrombo-embolic events presented at the
accident and emergency department with
one day history of fevers and convulsions.
This was associated with history of
progressively worsening memory loss and
confusion associated with incoordination
of hands. She also reported to have had
a productive cough of 3 months which
was episodic. The patient was admitted
and developed multiple organ failure
from lungs, heart and the kidney during
treatment in hospital attributed to this
disease. She succumbed during treatment

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