CARDIOVASCULAR JOURNAL OF AFRICA • Vol 24, No 9/10, October/November 2013
e10
AFRICA
raised liver enzymes (alanine transaminase 61 units/ml, aspartate
transaminase 32 units/ml, alkaline phosphatase 129 units/ml)
and ESR (
>
150 mm/h). Her HIV antibody screening test, lipid
profile, fasting blood sugar, serum electrolytes and urinalysis
were normal.
Pregnancy was ruled out with a serum HCG test and pelvic
ultrasound. Echocardiography showed normal cardiac chamber
sizes, normal heart valves, no valvular stenosis or regurgitation,
no intracardiac clots, vegetations or mass; normal diastolic and
systolic function and normal pericardium.
The serum anticardiolipin antibodies were elevated (IgM
17.08 units/ml and IgG 100.68 units/ml: normal for both
<
10
units/ml). The anti-beta-2 glycoprotein IgM and IgG antibodies
were increased: 50 units/ml and 182 units/ml, respectively
(normal
<
20 units/ml). Anti-nuclear antigen (ANA) was positive
(1:1 280-speckled pattern). The anti-double-stranded DNA was
positive (OD ratio 1.201). Rheumatoid factor was negative.
From these results, a definitive diagnosis of antiphospholipid
syndrome secondary to systemic lupus erythematosis was
made and the patient’s further management was along that line.
The patient was managed with a daily dose of subcutaneous
enoxaparin 40 mg, tablet aspirin 75 mg, prednisolone 40 mg,
vitamin C 400 mg tds, tramadol 50 mg tds, ranitidine 150 mg
nocte. She was transfused with three units of blood on account
of anaemia.
She was reviewed by plastic surgeons and orthopaedic
surgeons who decided that amputation of the 10 toes (toe
disarticulation) was indicated due to the gangrene. She has been
stable since amputation but was subsequently lost to follow up.
Discussion
Very few cases of antiphospholipid syndrome (APLS) have
been reported from sub-Saharan Africa. Apart from a low case
incidence, other reasons could be a low index of suspicion, or a
lack of investigative tools, and poverty.
The patient’s illiteracy and poverty, made worse by the
absence of an adequate health insurance scheme, was contributory
to the inability of the authors to fully investigate and manage the
index case presented here. The patient opted for surgery outright
instead of allowing repeat assessment of the antiphospholipid
antibodies. Only one set of antiphospholipid antibodies was also
reported from western Nigeria, perhaps for similar reasons.
From Nigeria, one of the two cases reported was that
by Adelowo and Oguntona who documented five cases of
secondary APLS in which association with pregnancy was the
most common presentation; the rest being due to neurological
manifestations.
14
We realise that a single documentation of antiphospholipid
antibodies in this case report does not fully satisfy the criteria for
diagnosis of APLS, which requires that these antibodies should
be found in medium to high levels on at least two occasions,
at least 12 weeks apart. Studies among different ethnic groups
have indicated that IgG antibodies are indeed the most common
antibody associated with thrombosis in African-Americans.
15,16
In other words, the high level of IgG found in this index case,
together with the degree of toe gangrene morbidity, is strongly
supportive of the diagnosis of APLS.
Furthermore, the British committee for standards in
haematology has stated in the 2012 guideline that the diagnostic
criteria should be used with care. The final diagnosis of
APLS should depend on a thorough assessment of the clinical
history, consideration of alternative causes of thrombosis or
pregnancy morbidity, and review of the laboratory data in light
of knowledge of the limitations of the assays.
10
APLS with toe gangrene is also infrequently reported in
African-Americans. In a series of eight cases, the majority
was secondary to systemic lupus erythematosis and mixed
connective tissue disease and the most common presentation was
neurological.
17
It does appear that APLS associated with toe gangrene is rare
in Africans and African-Americans and that toe gangrene is one
of the most unlikely forms of presentation. Toe gangrene has
been reported as a presentation of APLS in Caucasians.
18
To the best of our knowledge, however, this is the first report
of such a presentation in Africans or African-Americans. Livedo
reticularis supports the diagnosis of APLS and signifies increased
risk of arterial thrombosis.
19
Fig. 2 shows vasculopathy in our index
patient. The occurrence of gangrene as a potential complication
in Africans emphasises the importance of considering APLS as a
differential diagnosis in patients with toe gangrene.
The renal impairment seen in the index case raises a question
as to whether there was associated catastrophic antiphospholipid
syndrome. However, venous or arterial thrombosis of the
large vessels is less common in patients with catastrophic
antiphospholipid syndrome, who tend to present with an acute
thrombotic microangiopathy affecting the small vessels of
multiple organs.
20
In catastrophic antiphospholipid syndrome (CAPLS), there
is clinical involvement of at least three different organ systems
over a period of days or weeks, with histopathological evidence
of multiple occlusions of the large or small vessels.
20
The kidney
is the organ most commonly affected (in 78% of patients: renal
thrombotic microangiopathy), followed by the lungs (66%: adult
respiratory distress syndrome), the central nervous system (56%;
cerebral microthrombi and micro-infarction), the heart (50%:
myocardial microthrombi), and the skin (50%).
Disseminated intravascular coagulation, which does not occur
in primary or secondary APLS, occurs in approximately 25%
of patients with CAPLS. The abnormal liver function tests in
our index patient may have been part of the clinical syndrome.
Most patients with renal involvement have hypertension,
often malignant, and approximately 25% require dialysis. The
mortality rate in CAPLS is 50%, and death is usually due to
multi-organ failure.
20
It is unlikely that our patient had CAPLS
given the clinical presentation.
It will be necessary to make extra efforts to trace this patient.
Mere amputation and healing of the wound will not cure her.
Without adequate follow up and treatment, she is likely to come
back with another thrombotic episode.
Conclusion
The essence of this case report is to raise the awareness
that, although antiphospholipid syndrome typically presents
in Africans in association with a pregnancy-related event or
a neuropathology, it should be considered as a differential
diagnosis in all African patients with unexplained vasculitis.
This is because early diagnosis and initiation of appropriate
therapy will reduce morbidity and mortality rates.
