Lupus and Organ Damage – Top 10 Series

1. What is lupus?

Systemic lupus erythematosus (SLE), often referred to as simply “lupus” is a chronic, systemic autoimmune disease that affects many organ systems – most commonly the skin, joints, and kidneys. Due to persistent, lupus-specific autoantibodies (antibodies that attack healthy self-proteins) in the blood that cause tissue damage, the clinical manifestations of lupus are diverse. Common symptoms include fatigue, joint pain and swelling, fever, skin rash (especially “butterfly rash” on the face), and sensitivity to light.

2. What is “organ damage?”

During uncontrolled acute or chronic lupus activity, organs may be damaged, most commonly those in the cardiovascular, neuropsychiatric, renal, and musculoskeletal systems. Depending on the affected organ, an acute problem can result in permanent tissue injury (“damage”), e.g., heart failure, stroke with loss of function, or chronic kidney failure. Organ damage can also result from medications. For example, corticosteroids can cause cataracts, osteoporosis, or avascular necrosis (the death of bone tissue due to a lack of blood supply).

3. Is organ damage reversible?

Organ damage is not reversible. Based on the Systemic Lupus International Collaborating Clinics (SLICC)/American College of Rheumatology (ACR) Damage Index, organ damage must be present for at least six months to be accepted as “damage.” Once it has persisted for at least six months, it is considered to be permanent, which may have a significant negative effect on physical functioning.

4. How can we assess organ damage in lupus patients?

The SLICC/ACR Damage Index, which captures 41 different clinical problems in 12 organ systems, is an instrument specifically developed to measure the “damage” caused by SLE. The total index score ranges from 0 to 49 (the higher the number, the higher the damage score). Organ systems included in the damage index are: ocular, neuropsychiatric, renal, pulmonary, cardiovascular, peripheral vascular, gastrointestinal, musculoskeletal, and skin.

5. What are the clinical problems included as organ damage, as defined by  SLICC/ACR?

• Cataracts, retinal change, optic atrophy (a cause of visual loss)
• Seizures, cognitive impairment (memory deficit, poor concentration, difficulty with calculation, difficulty in spoken or written communications)
• Stroke, cranial or peripheral neuropathy (inflammation of the nerves in brain or spinal cord), transverse myelitis (inflammation of the spinal cord)
• Protein in the urine, end-stage renal disease, decreased kidney function
• Pulmonary hypertension, pulmonary fibrosis (scarring in the lungs), pulmonary infarction (dead tissue in the lung), pleural fibrosis (tissue covering the lungs becomes thick and stiff), shrinking lung
• Coronary bypass, heart attack, cardiomyopathy (heart muscle becomes enlarged, thick, or rigid), heart valve disease , pericarditis (inflammation of the sac which surrounds the heart)
• Minor or significant tissue loss, venous thrombosis (blood clot), claudication (pain and cramping in the lower leg while walking or exercising, caused by blocked arteries)
• Infarction or resection of gastrointestinal tract (dead tissue or the surgical removal of the digestive tract), stricture (a narrowing or tightening of the gastrointestinal tract), mesenteric insufficiency (a condition that restricts blood flow to the intestines), chronic peritonitis (long-lasting inflammation of the abdomen's lining), chronic pancreatitis (long-lasting inflammation of the pancreas)
• Muscle atrophy or weakness, tendon rupture, deforming or erosive arthritis, avascular necrosis, osteomyelitis (inflammation of the bone caused by an infecting organism), osteoporosis with fracture
• Scarring alopecia (hair loss), skin ulceration
• Premature gonadal failure (loss of function of the ovary or testis before the normal age)
• Diabetes
• Cancer

6. What is the risk of organ damage in lupus?

Based on different studies, 10%-30%, 20%-40%, and 30%-50% of SLE patients have demonstrated organ damage at one, five, and 10 years, respectively. In early disease, higher damage index scores are associated with a poor prognosis. However, the early damage scores are usually quite low for most patients, and approximately 90% SLE patients have minimal or no damage in the first year.

7. What factors increase the risk of organ damage in lupus?

• Older age at diagnosis
• Male sex
• Race (e.g., African Americans)
• Disease activity
• Certain organ involvement (e.g., central nervous system, kidney)
• Longer disease duration
• Antiphospholipid antibody positivity
• Lower socioeconomic status
• Long duration of steroid use

8. What is the impact of antiphospholipid antibodies on the risk of organ damage in lupus?

In lupus patients with persistent moderate- or high-level antiphospholipid antibodies (aPL) – independent of a diagnosis for antiphospholipid syndrome – the risk of organ damage is approximately 2-3 times higher, compared to those without aPL.

9. Is it possible to prevent organ damage in lupus?

Early diagnosis and aggressive treatment of lupus are important to prevent organ damage. Treatment should include a multidisciplinary approach, close monitoring and suppression of disease activity, prevention of flares, and use of antimalarial medications from an early stage. Also critical is the optimal management of additional risk factors, e.g., cardiovascular disease, smoking, and steroid use.

10. What is the best strategy to manage organ damage?

Please speak with your physician to learn whether you have any organ damage and, if so, its severity. Depending on the type and severity of organ involvement, the management strategy may differ (e.g., no intervention for mild cataracts, medications for blood clots, surgery for tendon rupture).

Posted: 8/17/2016

Authors

Related articles

Other resources

References:

1. Bruce IN, O’Keeffe AG, Farewell V, et al. Factors associated with damage accrual in patients with systemic lupus erythematosus: results from the Systemic Lupus International Collaborating Clinics (SLICC) Inception Cohort. Ann Rheum Dis. 2015;74:1706-13.
2. Duarte Couto M, Ines L, Liang MH. Epidemiology of systemic lupus erythematosus. In: Systemic lupus erythematosus, 5th Edition. Lahita RG, Tsokos G, Buyon J, Koike T, editors. Philadelphia: Elsevier; 2011; pp. 673–96.
3. Eder L, Urowitz MB, Gladman DD. Damage in lupus patients – what have we learned so far? Lupus 2013;22 :1225-31.
4. Erkan D, Criscione-Schreiber LG, Dall’era M, et al. Is there an association between persistently high positive antiphospholipid antibody profiles and organ damage accrual in lupus patients? ACR abstract, 2014.
5. Gladman D, Ginzler E, Goldsmith C, 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.
6. Karlson EW, Daltroy LH, Lew RA, et al. The relationship of socioeconomic status, race, and modifiable risk factors to outcomes in patients with systemic lupus erythematosus. Arthritis Rheum. 1997;40:47–56.
7. Lam GKW, Petri M. Assessment of systemic lupus erythematosus. Clin Exp Rheumatol. 2005;23:120-32.
8. Lopez R, Davidson JE, Beeby MD, et al. Lupus disease activity and the risk of subsequent organ damage and mortality in a large lupus cohort. Rheumatology (Oxford) 2012;51:491-8.
9. Rahman P, Gladman DD, Urowitz MB, et al. Early damage as measured by the SLICC/ACR damage index is a predictor of mortality in systemic lupus erythematosus. Lupus 2001;10:93-6.
10. Ruiz-Irastorza G, Egurbide MV, Martinez-Berriotxoa A, et al. Antiphospholipid antibodies predict early damage in patients with systemic lupus erythematosus. Lupus 2004;13:900-5.
11. Sutton EJ, Davidson JE, Bruce IN. The systemic lupus international collaborating clinics (SLICC) damage index: a systematic literature review. Semin Arthritis Rheum. 2013;43:352-61.
12. Stoll T, Stucki G, Gordon C, et al. A damage index for lupus patients – where are we now? Lupus 1997;6:219-22.
13. Taraborelli LL, Lazzaroni MG, Martinazzi N, et al. Antiphospholipid antibodies and the risk of damage accrual in systemic lupus erythematosus. Ann Rheum Dis. 2015;74:574.
14. Unlu O, Zuily S, Erkan D. The clinical significance of antiphospholipid antibodies in systemic lupus erythematosus. Eur J Rheumatol. 2016;3:75-84.
15. Urowitz MB, Gladman DD, Ibanez D, et al. Evolution of disease burden over five years in a multicenter inception systemic lupus erythematosus cohort. Arthritis Care Res. 2012 Jan;64:132-7.
16. Zonana-Nacach A, Barr SG, Magder LS, et al. Damage in systemic lupus erythematosus and its association with corticosteroids. Arthritis Rheum. 2000;43:1801-8.