Graham Hughes1 and Shirish Sangle2
(1)
The London Lupus Centre, London Bridge Hospital, London, UK
(2)
Louise Coote Lupus Unit, St Thomas’ Hospital, London, UK
Abstract
In the 1980s with the increasing referral pattern of APS patients to our unit, we began to see cases of acute, widespread thrombotic disease, always requiring the help of the intensive care unit and with a high fatality rate. There was no clear triggering factor, though in one of our cases, the acute collapse occurred in a patient with Hughes Syndrome, whose warfarin was stopped following a head injury.
19.1 Background
In the 1980s with the increasing referral pattern of APS patients to our unit, we began to see cases of acute, widespread thrombotic disease, always requiring the help of the intensive care unit and with a high fatality rate. There was no clear triggering factor, though in one of our cases, the acute collapse occurred in a patient with Hughes Syndrome, whose warfarin was stopped following a head injury.
In 1991, Greisman reported two patients with “acute catastrophic, widespread non-inflammatory visceral vascular occlusions associated with high titre antiphospholipid antibodies”.
In an editorial in the same issue of the journal, my colleague Nigel Harris described two further cases. Another of my research fellows, Ron Asherson, a year later, repeated the term “catastrophic” in a series of 20 of our cases.
Thanks in large part to the efforts of Ron Asherson and Richard Cervera, an international registry of this rare “catastrophic” APS has been initiated.
19.2 Clinical Features
Approximately one half of the patients had no prior thrombotic history. In those with previous thromboses, arterial thrombosis accounted for a minority (13%).
A prior history of miscarriage or of thrombocytopenia was rare. Precipitating factors were diverse, and included infection, recent fetal loss and surgery. However, in the majority, there was no clear trigger (Fig. 19.1).
Figure 19.1
Catastrophic APS (Khamashta, M.A., Hughes Syndrome: Antiphospholipid Syndrome, 2nd ed., 2006, reproduced with permission from Springer Science+Business Media B.V.)
The characteristic feature is of widespread thrombosis, including renal, cerebral, pulmonary involvement.
Clinical sequelae include adult respiratory stress syndrome, adrenal infarction (leading in some cases to Addison’s disease), and seizure, livedo and widespread skin necrosis.
Thrombocytopenia occurs in approximately one half of these patients and a picture of disseminated intravascular coagulation (DIC) is seen in some. In most reported series, some 50% of cases are fatal.
19.3 Treatment
As multi-organ failure occurs, treatment always involves intensive care therapy. At the present time, the combination of heparin, steroids, plasmapheresis and IVIG seemed to provide a marginally better outcome in this extreme form of APS.