RHEUMATOLOGY
How to assess systemic lupus
erythematosus in clinical care
Physicians involved in the care of SLE patients 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
Micaela Fredi MD
Angela Tincani MD
Rheumatology and
Clinical Immunology
Unit and Clinical and
Experimental Science
Department, ASST
Spedali Civili and
University of Brescia,
Brescia, Italy
Systemic lupus erythematosus (SLE) is
a multisystemic autoimmune disease, the
spectrum of which covers a wide array of clinical
and laboratory manifestations. The disease has
considerable clinical and immunological
heterogeneity; no two patients with SLE are
exactly alike. The aetiology of SLE is thought to be
multifactorial, with multiple genetic, epigenetic,
hormonal, and immunopathological pathways
being involved. SLE is characterised by the
production of autoantibodies which leads to
immune complex deposition, inflammation, and
eventually, permanent organ damage. The course
of SLE is largely unpredictable and characterised
by periods of remission and disease exacerbation
that could lead to progressive organ damage and
dysfunction. It most commonly presents in
women with a peak incidence between the ages
of 15 and 40. 1 However, SLE can affect all age
groups, from infants to geriatric patients.
Compared with the age- and sex-matched general
population, SLE is associated with at least
a five-fold increase in mortality. 2
Accurate clinical assessment of the disease is
desirable because SLE has a complex phenotype,
a cumulative damage and a variable disease
course with new organ system involvement
even many years after diagnosis.
For all these reasons, the availability of
measures for diagnosing, monitoring disease
activity, and assessing tissue damage are all
important and necessary in SLE management.
Assessment of SLE 3 can be divided into several
components:
1 diagnosis;
2 monitoring;
3 comorbidities;
4 family planning.
Diagnosis
All patients suspected of having SLE should be
referred to a rheumatologist or to a SLE specialist
to confirm diagnosis and be involved in ongoing
care. When considering a patient with a possible
diagnosis of SLE, a detailed clinical history and
examination is required in order to identify
relevant clinical features. An early diagnosis of
SLE could be challenging for many reasons: the
absence of pathognomonic findings, the
heterogeneity at onset and the time required for
its full development. This peculiar disease
pattern could explain the long delay reported
between the onset of the first symptoms and the
3
HHE 2019 | hospitalhealthcare.com
final diagnosis. In the last decades, however, the
delay between clinical onset and diagnosis has
been reduced, from more than 2 years in patients
diagnosed during the eighties to nine months in
those diagnosed in the last years. 4 The Systemic
Lupus International Collaborating Clinics (SLICC)
set of classification criteria (Table 1) 5 and the new
American College of Rheumatology and European
League Against Rheumatism (ACR/EULAR ) criteria
(Table 2) 6 may be helpful also considering the
diagnosis; however, they do not cover all the
possible clinical manifestations of SLE.
In conclusion, final diagnosis of SLE is
a combination of clinical features and the
presence of at least one relevant immunological
abnormality and it still requires the meticulous
clinical judgment of qualified physicians.
Screening questions should be employed to
detect possible SLE manifestations in all systems
of the body, particularly manifestations included
in the classification criteria and others that are
common in lupus patients, for example, fatigue,
photosensitivity, skin lesions, arthralgias, alopecia
and Raynaud’s phenomenon. Constitutional
symptoms such as fatigue, fever, unintentional
weight loss and lymphoadenopathy are common
presenting symptoms of SLE. These symptoms,
however, are not specific and other causes should
be excluded, such as infection, malignancy,
endocrinopathy, other connective tissue diseases,
depression and fibromyalgia. In addition,
environmental triggers such as exposure to
ultraviolet radiation, infection, or the use of
certain medications, should be identified, if
possible. At the moment of the diagnosis,
a complete clinical and immunological evaluation
should be performed, as reported in Table 3.
Possible kidney or neurological involvement
should always be ruled out, because involvement
of these organs is considered as a severe SLE
manifestation.
Monitoring
No data are available in the literature to suggest
an optimal frequency of clinical and laboratory
assessment in patients with SLE. Table 3
summarises the assessment and monitoring of
patients with SLE. 3
The frequency of the follow-up visits should be
based on the activity and severity of the disease,
its complications and evolution. In patients with
inactive disease, without organ damage and
comorbidities, the EULAR recommends that