Just who is at risk for organ damage? What factors contribute to it? How does it impact patients and their lives? And, most importantly, what treatment strategies can slow its progress?
How many of your patients with SLE could be at risk for developing permanent organ damage?
Organ damage begins to accrue early, with as many as 50% of patients showing evidence of irreversible damage* within 5 years of SLE diagnosis7-9
|*||Based on Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI) measurements|
Percentage of patients with permanent organ damage7,8
Damage is defined as an irreversible tissue injury occurring after SLE diagnosis and lasting at least 6 months.9 SDI is the internationally agreed and validated measure of organ damage. A limitation of the SDI is that it may also capture damage that may not be related to SLE specifically.
Assessing Organ Damage via SDI9
While typically employed in clinical studies, the SDI may prove useful in assessing organ damage in your patients with SLE.
SDI measures permanent, irreversible damage in a lupus patient. Items should be present for at least 6 months, except manifestations such as myocardial infarction and stroke, which are recorded once they occur.
It is important to note that an increase of even one point in a patient’s SDI can correlate with serious health issues (eg, myocardial infarction, cerebrovascular accident, or cataracts).
|Damage is defined for
|Premature gonadal failure|
|Diabetes (regardless of treatment)|
Damage over time can only be stable or increase, theoretically, to a maximum of 47 points.
Which organs can be affected?
Several organ systems are commonly involved in SLE and should be carefully monitored for disease activity. Commonly involved organ systems include the following: central nervous; renal; gastrointestinal; mucous membranes; cardiovascular; mucocutaneous; hematologic; musculoskeletal; and, pulmonary.4
Severity of the disease can vary from mild to potentially fatal, due to detrimental organ damage.10
Renal damage is one of the most serious complications of SLE.16 Usually manifesting during the early stage of SLE, renal disease has consistently been shown to be a major cause of mortality in patients with SLE.16
- Urinalysis abnormalities or renal dysfunction exist in 35% of patients early in the course of the disease.17
- Up to 60% of adults with SLE develop renal abnormalities over the course of their disease.18
Patients with SLE are 5 times more likely to have a bone fracture than age-matched controls.19
This is primarily due to the prolonged use of steroids in their treatment and age at SLE diagnosis.
- The relative risk of osteoporotic fractures is increased 1.9-fold with a cumulative dose of 36.5 g of corticosteroids.20
Mucocutaneous involvement is almost universal in SLE. Manifestations include11:
- nasal and oral ulcers
Neuropsychiatric syndromes can affect many patients with SLE.12 They range from mild to severe. The most common manifestations include:
- cognitive dysfunction
SLE may affect almost any part of the eye and the visual pathway. If a patient has ophthalmic issues, he or she should be referred to an ophthalmologist. The following symptoms may be a sign of potential complications13:
- dry eyes
- loss of vision
Risks of coronary heart disease (CHD) and stroke are significantly higher for patients with SLE compared to general population.14,15
- Patients with SLE are at 2- to 10- times increased risk of CHD.
How can permanent organ damage affect your patients?
There is also a risk of organ damage accrual while the patient is completely unaware. For example, osteonecrosis may be asymptomatic in patients with SLE.24 Cardiovascular disease may also be subclinical in patients with SLE—prevalence of plaques in internal carotid arteries is 3 times higher, and endothelial dysfunction, an early marker of atherosclerosis, is 2 times as common compared to the control group.25,26
What factors contribute to organ damage?
Persistent disease activity is linked to increased organ damage, which, in turn, is predictive of increased damage and mortality.3
Currently, no consensus exists on how to assess patient outcomes and measure disease activity. Tools,27-29 including SELENA-SLEDAI, BILAG, and PGA, have been developed for use in clinical trials, but their use in real-world practice is limited.
The changeable nature of SLE may make it difficult to predict when disease activity—in the form of flares—will occur.2
Patterns of SLE Disease Activity
Disease activity in SLE may appear in different patterns—relapsing-remitting (RR), chronically active (CA), and long-quiescent.1,2
A study of the Hopkins Lupus Cohort found that while RR was the most common disease pattern observed, over a 3-year perspective, less than half of patients maintained their original pattern.30
The charts below are for illustrative purposes only and represent activity over any three-year period.
RR was the most prevalent pattern type.
80%-94% of patients with SLE had RR and/or CA patterns.30
(M-SLEDAI: 66% n=1244, CA 14.6% n = 275; PGA: RR 56.7% n= 1069, CA 37.8% n = 712)
Drug-related side effects can also result in the accrual of organ damage.3
- Chronic corticosteroid use is associated with cataracts, diabetes mellitus, atherosclerotic heart disease, osteoporosis and osteonecrosis, and fluid retention17
- Opportunistic infections and certain malignancies can develop, most often in patients receiving chronic immunosuppressive therapy31
- Long-term use of NSAIDs is associated with GI bleeding and kidney damage10
- There is evidence for multiple beneficial effects of antimalarials in SLE; however, patients should be monitored for potential retinal toxicities.32
How can organ damage be prevented or managed?
Minimize disease activity and flares
- Persistent disease activity can result in ongoing organ damage4
- Since damage predicts subsequent damage and death, prevention of the damage accrual should be a major therapeutic goal in SLE33
- Prevention of flares (especially severe flares) is a realistic target in SLE and should be a therapeutic goal33
EULAR treatment recommendations highlight the importance of controlling disease activity and flares while also limiting steroid dose.27
EULAR=European League Against Rheumatism
Graphical representation of a hypothetical disease course of a patient with SLE.
Reduce glucocorticoid use as much as possible
- Glucocorticoids are invaluable in the treatment of SLE; however, their chronic use has consistently shown to increase irreversible damage in patients with lupus, a major predictor of morbidity and mortality34
- In general, the risk of organ damage increases at 7.5 mg/day35
Glucocorticoids ≥7.5 mg/day
1.7x greater risk of
new organ damage
- Dosage should be reduced to the minimum needed to control disease, or eliminated if possible.33,34,36
Monitor for subclinical activity
- Regular monitoring should be undertaken to detect "silent variables"37
- At 3-4-month intervals for patients with mild or inactive disease.
DON'T LEAVE LUPUS TO SMOLDER
Can you do more to decrease disease activity and prevent flares with the lowest possible dose of glucocorticoids?3,27,33
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