• perform a risk assessment: other than the
Ideally, all patients with SLE who wish to
conceive should have preconception counselling;
in fact, planned pregnancies have demonstrated
better maternal and foetal outcomes. Systemic
activity of SLE must be assessed, with careful
attention to renal involvement. A recent flare is
a risk factor for recurrence during pregnancy;
therefore a pregnancy should be planned after
at least six consecutive months of stable disease.
During the preconceptional visit, the SLE
specialist should:
common epidemiological risk factors that are
relevant to any pregnancy, the physician should
revise previous obstetric history, assess activity
(SLEDAI-2K and/or BILAG) and irreversible damage
(SLICC-SDI DI);
• complete laboratory testing, with particular
attention to aPL status, presence of anti-Ro/SSA
or LA/SSB antibodies
• review therapies, as immunosuppression,
antihypertensives and anticoagulation.
At the same time, patients need to be evaluated
by an experienced obstetrician who will perform
the investigation required for all the women who
wish to get pregnant.
Once pregnancy is confirmed, monthly visits
are usually indicated (or even more frequently)
if the disease is not controlled.
Special monitoring is dedicated to women with
Ro/SSA and/or La/SSB antibody positivity; these
patients should be informed about the risk of
neonatal lupus and congenital heart block, a
severe complication that occurs in 0.7–2% 31,32
of women with these autoantibodies.
Conclusions
Physicians involved in the care of patients
affected by SLE need easy to use, replicable and
practical evaluation tools. These can help in all
disease phases, from diagnosis through to the
occurrence of flares, comorbidities, or even
pregnancy. SLE shows a variable and complex
phenotype; therefore a precise assessment is
needed to administer the treatment able to
target the index manifestation. Only by following
this strategy will we be able to control the
disease, reaching remission or low disease
activity, 26 which are essential in improving
the morbidity, mortality and quality of life
in these patients.
References
1 Wahren-Herlenius M et
al. Immunopathogenic
mechanisms of systemic
autoimmune disease. Lancet
2013;382:819–31.
2 Mok CC et al. Life expectancy,
standardized mortality ratios,
and causes of death in six
rheumatic diseases in Hong
Kong, China. Arthritis Rheum
2011;63:1182–9.
3 Mosca M et al. European
League Against Rheumatism
recommendations for
monitoring patients with
systemic lupus erythematosus
in clinical practice and in
observational studies. Ann
Rheum Dis 2010;69:1269–74.
4 Doria A et al. SLE diagnosis and
treatment: when early is early.
Autoimmun Rev 2010;10:55–60.
5 Petri M, et al. Derivation and
validation of the Systemic Lupus
International Collaborating
Clinics classification criteria for
systemic lupus erythematosus.
Arthritis Rheum 2012;64:
2677–86.
6 Aringer M et al. 2019
European League Against
Rheumatism/American College
of Rheumatology classification
criteria for systemic lupus
erythematosus.Ann Rheum Dis
2019;78(9):1151–9.
7 Mosca M et al. Development
of quality indicators to evaluate
the monitoring of SLE patients
in routine clinical practice.
Autoimmun Rev 2011;10:383–8.
8 Griffiths B et al. Assessment
of patients with systemic lupus
erythematosus and the use of
lupus disease activity indices.
Best Pract Res Clin Rheumatol
2005; 19:685–708.
9 Gladman DD et al. Systemic
Lupus Erythematosus Disease
Activity Index 2000. J Rheumatol
2002;29:288–91.
10 Hay EM et al. The BILAG
index: a reliable and valid
instrument for measuring clinical
disease activity in SLE. Q J Med
1993;86:447–58.
11 Gladmand D et al. The
development and initial
validation of the Systemic Lupus
International Collaborating
Clinics/American College of
Rheumatology damage index for
systemic lupus erythematosus.
Arthritis Rheum 1996;39:363–9.
12 Rahman P et al. Early damage
as measured by the SLICC/ACR
damage index is a predictor
of mortality in systemic
lupus erythematosus. Lupus
2001;10(2):93–6.
13 Ghaussy NO et al. Cigarette
smoking and disease activity in
systemic lupus erythematosus.
J Rheumatol 2003;30:1215–21.
14 Vilá L et al. Association
of sunlight exposure and
photoprotection measures with
clinical outcome in systemic
lupus erythematosus. P R Health
Sci J 1999;18:89–94.
15 Miyakis S, et al. International
consensus statement on an
update of the classification
criteria for definite
antiphospholipid syndrome
(APS). J Thromb Haemost 2006;4:
295–306.
16 Chighizola CB et al. The
treatment of anti-phospholipid
syndrome: a comprehensive
clinical approach. J Autoimmun
2018;90:1–27.
17 Tektonidou MG et al.
EULAR recommendations
for the management of
antiphospholipid syndrome in
adults. Ann Rheum Dis 2019;
Epub ahead of print doi:10.1136/
annrheumdis-2019-215213.
18 Pengo V et al. Rivaroxaban
vs warfarin in high-risk
patients with antiphospholipid
syndrome. Blood
2018;132:1365–71.
19 Magder LS, Petri M. Incidence
of and risk factors for adverse
cardiovascular events among
patients with systemic lupus
erythematosus. Am J Epidemiol
2012; 176: 708-19.
20 Esdaile JM et al. Traditional
Framingham risk factors fail to
fully account for accelerated
atherosclerosis in systemic lupus
erythematosus. Arthritis Rheum
2001;44:2331–7.
21 Hippisley-Cox J et al.
Development and validation
of QRISK3 risk prediction
algorithms to estimate future
risk of cardiovascular disease:
prospective cohort study. BMJ
2017; 357:j2099
22 Edwards N et al. QRISK3
improves detection of
11
HHE 2019 | hospitalhealthcare.com
cardiovascular disease risk in
patients with systemic lupus
erythematosus. Lupus Sci Med
2018;5:e000272.
23 Perk J et al. European
Guidelines on cardiovascular
disease prevention in clinical
practice (version 2012): The Fifth
Joint Task Force of the European
Society of Cardiology and Other
Societies on Cardiovascular
Disease Prevention in Clinical
Practice. Atherosclerosis
2012;223:1–68.
24 Elkayam O et al. Update of
EULAR recommendations for
vaccination of patients with
autoimmune inflammatory
rheumatic diseases. Ann Rheum
Dis 2018;77.
25 Rúa-Figueroa I et al.
Incidence, associated factors
and clinical impact of severe
infections in a large, multicentric
cohort of patients with systemic
lupus erythematosus. Semin
Arthritis Rheum 2017;47:38–45.
26 Fanouriakis A et al.
2019 update of the EULAR
recommendations for the
management of systemic lupus
erythematosus. Ann Rheum Dis
2019;78(6):736–45.
27 Almehed K et al. Prevalence
and risk factors of osteoporosis
in female SLE patients-extended
report. Rheumatology (Oxford)
2007;46:1185–1190.
28 Grossman JM et al. American
College of Rheumatology
2010 recommendations for
the prevention and treatment
of glucocorticoid-induced
osteoporosis. Arthritis Care Res
(Hoboken) 2010;62:1515–26.
29 Andreoli L et al. EULAR
recommendations for women’s
health and the management
of family planning, assisted
reproduction, pregnancy and
menopause in patients with
systemic lupus erythematosus
and/or antiphospholipid
syndrome. Ann Rheum Dis
2017;76(3):476–85.
30 Götestam Skorpen C et al.
The EULAR points to consider
for use of antirheumatic drugs
before pregnancy, and during
pregnancy and lactation. Ann
Rheum Dis 2016 May;75(5):
795–810.
31 Brucato A et al. Risk
of congenital complete
heart block in newborns
of mothers with anti-Ro/
SSA antibodies detected by
counterimmunoelectrophoresis:
a prospective study of 100
women. Arthritis Rheum
2001;44:1832–5.
32 Fredi M et al. First report of
the Italian Registry on immune-
mediated congenital heart
block (Lu.Ne registry). Frontiers
Immunology 2019;6:11.