by the disease also has to be assessed. Since 1996,
a damage index proposed by Systemic Lupus
International Collaborating Clinics has been used
– the SLICC/ACR Damage Index (SLICC/ACR DI). 11
This index includes persistent damage related to
SLE per se (present for more than six months),
specific comorbidities associated to the
autoimmune disease, and features that are often
due to toxicity related to treatments. Higher
damage index scores early in disease have been
associated with a poor prognosis and with
increased mortality. 12 Thus, the SLICC/ACR DI
complements other measures of disease activity
described above, and it is an important outcome
measure. It is usually completed (or updated)
yearly.
Lifestyle guidance
Finally, lifestyle modifications should be
discussed. All SLE patients should stop smoking,
not just because of the effect that smoking has
on the activity of the disease and quality of life, 13
but also because of its causal association with
the increase in risk of cardiovascular disease,
infection and cancer. Regular physical exercise
in people with stable SLE with low to moderate
disease activity should be recommended in order
to avoid weight gain expecially in those patients
that are taking corticosteroids.
Adequate photoprotection should be advised
in all SLE patients, irrespective of the presence
of skin manifestations. 14 Broad spectrum
photoprotectors with high solar photoprotection
index should be applied in adequate quantity
before exposure and frequently reapplied and
the use of hat and long-sleeved clothing should
be advised.
TABLE 3
Summary of recommended assessment and
frequency
Assessment Frequency
Each clinic visit
Clinical history and complete physical
examination; vital signs, blood
pressure, weight
Blood count, ESR, CRP, creatinine,
Each clinic visit
liver function, urine analysis,
SLE serology: anti-dsDNA, C3, C4
Creatinine clearance,
As clinically indicated if active problem
24-hour urine protein
Extractable nuclear antigen
At baseline, pre-pregnancy counselling
Anticardiolipin, anti-b2 glycoprotein I
At baseline, annually and
antibodies, lupus anticoagulant,
pre-pregnancy counseling
PT, PTT
Immunoglobulin, vitamin D levels
Baseline and annually or as clinically
indicated if active problem
Assessment of cardiovascular risk:
At baseline and annually
that is, lipid profile, BMI, smoking
Each clinic visit
Disease activity index
(PGA, SLEDAI and/or BILAG)
Annually
SLICC/ACR damage index
Echocardiogram, chest X-ray,
Baseline and as clinically indicated
abdominal ultrasound
factors, age and the risk of bleeding.
Concerning the secondary prevention, for both
unprovoked venous thromboses and arterial
thrombosis, indefinite anticoagulation is
recommended. 17
The use of novel oral anticoagulants for
secondary prevention should be avoided,
especially in patients with triple aPL positivity. 18
It is well known that SLE patients present an
increase in risk of cardiac events 19 and coronary
disease compared with the rest of the population.
The risk that a young female SLE patient develops
cardiovascular events was reported 30–50-times
higher than that of the same age healthy control.
This excess of risk is not entirely explained by
classical risk factors, but rather, other factors
related to the actual disease may also be involved,
such as chronic systemic inflammation or
treatment with glucocorticoids. 19,20
Traditional risk algorithms (for example,
Framingham) show only marginal differences
between SLE and controls, and thus
underestimate cardiovascular disease risk of SLE
patients. A new risk score to predict the
development of cardiovascular disease has been
reported (QRISK3) 21 where SLE is considered as
a risk factor. The use of QRISK3 was able to
capture significantly more patients with SLE
with an elevated 10-year risk of developing
cardiovascular disease compared with
Framingham. 22 The European Soiety for
Cardiology includes SLE in the population group
with increased risk of suffering cardiovascular
disease, 23 for whom it recommends a target LDL
level lower than 2.5mmol/l (96mg/dl).
Comorbidities
Patients with SLE are at increased risk of
comorbidities such as infection, thrombotic
events, premature cardiovascular and peripheral
vascular disease and osteoporosis. These aspects
should be assessed at diagnosis and regularly
during follow-up. The frequency of the proposed
assessment is reported in Table 3.
Antiphospholipid antibodies, thrombosis and
cardiovascular risk
Antiphospholipid antibodies (aPL) are considered
a risk factor of thrombosis, both in the presence
and in the absence of a concomitant autoimmune
disease such as SLE. 15
It is very important to remember that all the
three test, namely lupus anticoagulant (LA),
anticardiolipin (aCL) and anti-β2glycoprotein
I antibodies (anti-β2-GPI) should be determined
at baseline and regularly during the follow-up
because only their complete evaluation allow to
assess the risk profile. It has been demonstrated
that the combinations of aPL generally increase
the risk of thrombosis, and the triple positivity
is associated with the greatest likelihood of
thromboembolic events. 16
The decision for the use of primary prophylaxis
in aPL carriers remains challenging: patients at
‘high risk’ (that is, triple positivity, high titres of
autoantibodies) should be treated with low-dose
aspirin, whereas in patients considered at ‘low
risk’, the decision should be made taking into
consideration also the classical cardiovascular risk
Infection
Patients with SLE are at high risk for infections
because of the underlying immune aberrations
and the prolonged use of immunosuppressive
treatments. 24,25 Protection against infections
should focus both on primary prevention, as
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