Monitoring
Lifelong monitoring is required for systemic
lupus erythematosus patients. The frequency of visits depends on the disease
activity, severity and extent, response to treatment, type of treatment, and monitoring
of toxicity. For
active systemic lupus erythematosus disease, monitoring can be done every 1-3
months after diagnosis or flare while for low or no disease activity or with
stable treatment course, monitoring can be done every 6-12 months. Patients with
severe systemic lupus erythematosus, complications, comorbidities and drug
toxicity will require more frequent follow-up.
The most important tool
in the management of systemic lupus erythematosus is a careful, frequent
clinical and laboratory evaluation to detect disease flares, drug side effects and appearance of infections,
and to tailor management based on patient response.
Monitoring during clinic visits should include history-taking, physical
examination, and laboratory tests (eg CBC, platelet count, creatinine
measurement, urinalysis, renal and liver function tests, ESR, CRP, immunology
or serology, electrocardiography, and imaging). Disease activity is assessed
regularly, particularly when there is change in clinical status or systemic
lupus erythematosus-directed medications, with disease damage assessment done
at least manually. It is therefore essential to do active surveillance of
opportunistic infections and adjust current therapy (eg corticosteroids and immunosuppressants)
according to the severity of organ involvement during systemic lupus
erythematosus flares. Gradual tapering of treatment is considered (withdraw
glucocorticoids first) in patients with sustained remission. Monitor for
glucocorticoid withdrawal syndrome and adrenal insufficiency during
corticosteroid tapering as these may present with non-specific symptoms that
can mimic systemic lupus erythematosus disease activity.
After
achieving a low disease activity or remission, 20-25% of patients with systemic
lupus erythematosus will flare within 1-2 years and 40-66% within 5-10 years.
The risk factors for systemic lupus erythematosus flare include age of systemic
lupus erythematosus onset ≤25 years old, male gender,
persistent clinical disease activity, major organ involvement (eg cytopenias,
nephritis, neuropsychiatric, vasculitis); lab findings of low serum C3/C4, high
anti-dsDNA antibodies; and treatment concerns (eg poor compliance,
discontinuation or has never been on Hydroxychloroquine, rapid tapering or
withdrawal of maintenance immunosuppressive treatment).
The results of laboratory
tests that may precede a disease flare include a decrease in serum complement
levels, an increase in anti-dsDNA, an increase in ESR, a decrease in hemoglobin
level, leukocyte, or platelet counts, an increase in creatine phosphokinase
(CPK) levels, and the appearance of microscopic hematuria or proteinuria.
Physicians may be aided by
at least one of the following several global or body system-specific indices
developed for long-term observation of systemic
lupus erythematosus: SLEDAI, BILAG, PGA, and ECLAM.
Lupus
Nephritis
Monitoring of lupus nephritis includes blood pressure
checks and
laboratory tests such as urinalysis, protein/creatinine ratio, serum
creatinine, C3/C4, and anti-dsDNA levels. Repeat kidney biopsy
should be considered to assess treatment response, evaluate worsening of
kidney-specific laboratory tests, or guide contemplated withdrawal of
immunosuppressive therapy. Non-immune therapies such as
renin-angiotensin-aldosterone blockers, sodium-glucose co-transporter 2 (SGLT2)
inhibitors, statins, and/or bone-protective agents to preserve kidney function
and reduce cardiovascular and bone risks should be given as appropriate.
Pregnancy may be planned after achieving stable control of lupus nephritis and
extrarenal disease manifestations, initiation of pregnancy-compatible
medications, and regular multidisciplinary assessments. All methods of kidney
replacement therapy may be used when indicated, and kidney transplantation
should be considered in patients with clinically inactive extrarenal disease of
at least 6 months.
Please see Lupus Nephritis disease
management chart for further information.
Comorbidities of Systemic Lupus
Erythematosus
All individuals with systemic lupus erythematosus
should be screened, monitored, and managed for comorbid conditions associated
with systemic lupus erythematosus and its treatment (including cardiovascular
disease, infection, malignancy, bone and joint damage, reproductive health
complications, and presence of antiphospholipid antibodies).
Cardiovascular
Disease
Regularly
evaluate patients with systemic lupus erythematosus for cardiovascular risk,
recognizing systemic lupus erythematosus as a risk-enhancing factor and
assessing both traditional and disease-related risk factors to guide management
(eg persistently active disease, increased disease duration, chronic use of
corticosteroids, persistent proteinuria and/or GFR of <60 mL/min, and medium
or high titers of antiphospholipid). Vascular events may be reduced with a
blood pressure maintained at <140/90 mmHg in patients with systemic lupus
erythematosus. Patients may be given low-dose Aspirin and/or lipid-lowering
agents based on their individual cardiovascular risk profile. For acute and/or
worsening lupus myocarditis, treatment with glucocorticoid and IV
Cyclophosphamide, Mycophenolic acid analogs, anti-CD20 therapy and/or IVIg may
be considered. For non-bacterial (Libman Sacks) endocarditis, immunosuppressive
therapy and/or anticoagulation may be considered.
Infectious
Diseases
Evaluate
patients with systemic lupus erythematosus for general and disease-related risk
factors for infection (eg advanced age, frailty, renal disease, diabetes
mellitus, use of corticosteroid, and immunosuppressive therapy). Early
diagnosis and treatment of infection or sepsis and general preventive measures
(eg immunizations) are recommended. Screen for HBV, hepatitis C virus (HCV),
tuberculosis (TB), and HIV prior to immunosuppressive and/or glucocorticoid
therapy. Infection prophylaxis for Pneumocystis jirovecii may be
considered for all people on high-dose glucocorticoids.
Malignancies
Malignancies may include
non-Hodgkin's lymphoma, leukemia, thyroid, lung, stomach, liver, bladder,
cervical, and vulval cancer. Perform cancer screening and manage according to
the established guidelines for the specific condition.
Antiphospholipid
Syndrome
Antiphospholipid syndrome
is an autoimmune overlap condition that may occur in association with systemic
lupus erythematosus. Antiplatelet agents as primary prophylaxis (eg low-dose
Aspirin) may be given to patients with systemic lupus erythematosus with a
high-risk antiphospholipid profile, especially if with other atherosclerotic or
thrombophilic factors, after considering potential for bleeding. Management for
secondary prevention (thrombosis, pregnancy loss or complication) should be the
same as for primary antiphospholipid syndrome. Vitamin K antagonists should be
given long-term after the first arterial or unprovoked venous thrombotic event
in patients with systemic lupus erythematosus associated with thrombotic
antiphospholipid syndrome.
Complications
Toxicity
Patients on long-term
glucocorticoids should be monitored for electrolyte, glucose, and lipid levels
to identify metabolic conditions. Bone densitometry may be requested to
identify osteoporosis and monitor response to treatment. Patients on Hydroxychloroquine should have
ophthalmological screening (eg visual fields examination and/or spectral
domain-optical coherence tomography) performed at baseline, after 5 years, and
then annually in those with no risk factors for retinal toxicity. For those
with known toxic retinopathy risk factors (eg long-term and/or high-dose use of
Hydroxychloroquine, advanced age, history of retinal and macular disease,
concomitant Tamoxifen), ophthalmologic screening should be performed at
baseline and then annually. Patients on immunosuppressants
should be monitored for hematologic, liver and renal toxicity, and the
occurrence of infection.