What is Lupus?
Lupus (systemic lupus erythematosus or SLE) is a chronic autoimmune disease where your immune system mistakenly attacks your own healthy tissues, causing inflammation and damage to multiple parts of your body including your skin, joints, kidneys, heart, lungs, brain, and blood cells. 1, 2
Understanding What Happens in Your Body
Your immune system loses its ability to tell the difference between foreign invaders (like bacteria) and your own healthy tissue. 3
Your body produces abnormal antibodies (called autoantibodies) that form immune complexes, which deposit in various organs and trigger inflammation. 4
This inflammation can affect virtually any organ system, which is why lupus symptoms vary so much from person to person. 5, 6
Who Gets Lupus?
Approximately 90% of people with lupus are women, particularly women of childbearing age. 2
Certain racial groups are at higher risk: Latin American, African American, Native American, and Asian individuals tend to develop lupus earlier, experience more severe disease, and have worse outcomes. 1
The disease results from a combination of genetic predisposition, hormonal factors, and environmental triggers like UV light exposure and Epstein-Barr virus infection. 6
Common Symptoms You Might Experience
Joint pain and swelling (arthritis) affecting multiple joints 1, 2
Skin rashes, including the characteristic "butterfly rash" across your cheeks and nose 2, 4
Extreme fatigue that doesn't improve with rest 6
Fever without obvious infection 2
Sensitivity to sunlight that can trigger rashes or disease flares 1
Kidney problems (lupus nephritis), which affects about 40% of people with lupus and can cause protein in the urine 2
Blood abnormalities including low blood cell counts (anemia, low white blood cells, low platelets) 2, 4
Chest pain from inflammation of the lining around the heart or lungs 4
Neuropsychiatric symptoms including headaches, confusion, memory problems, mood changes, or seizures 1, 4
How Lupus is Treated
Foundation Treatment for Everyone
All patients with lupus should take hydroxychloroquine (Plaquenil) unless you have a specific reason you cannot take it—this medication is the cornerstone of lupus treatment and has been shown to reduce disease activity, prevent flares, and improve survival. 7, 8, 2
The dose should not exceed 5 mg/kg of your actual body weight. 7
You will need regular eye exams: at baseline, after 5 years, and then yearly to monitor for rare retinal toxicity. 7
You must protect your skin from the sun using sunscreen daily, as UV exposure can trigger lupus flares. 7
You should take calcium and vitamin D supplements if you're on long-term steroids to protect your bones. 7
Steroids (Glucocorticoids/Prednisone)
If needed for active disease, steroids should be used at the lowest possible dose and for the shortest time period. 1
For severe flares, you may receive high-dose intravenous methylprednisolone (IV steroids) in the hospital. 7
The goal is to reduce your steroid dose to less than 7.5 mg per day of prednisone (or equivalent) for long-term use, and ideally to stop them completely to prevent organ damage. 7
Additional Medications for Moderate to Severe Disease
If hydroxychloroquine and low-dose steroids don't control your disease, or if you have organ-threatening lupus, your doctor will add immunosuppressive medications: 7
Azathioprine for maintenance therapy, especially if you're planning pregnancy 7
Mycophenolate mofetil (CellCept) for kidney disease and other severe manifestations 7, 2
Cyclophosphamide (Cytoxan) for severe, organ-threatening disease, particularly kidney, heart, lung, or brain involvement 7, 2
Newer Biologic Therapies
If standard treatments don't work adequately, three newer medications have been FDA-approved specifically for lupus: 7, 9, 2
Belimumab (Benlysta) for active lupus and lupus kidney disease 9, 2
Anifrolumab for moderate-to-severe active lupus 7
Voclosporin specifically for lupus kidney disease 7
Treatment for Specific Organ Involvement
For lupus kidney disease (lupus nephritis):
- You will need a kidney biopsy to determine the type and severity of kidney inflammation. 7
- Initial treatment combines high-dose steroids with either mycophenolate mofetil or cyclophosphamide. 7
- After the initial treatment phase, you'll transition to maintenance therapy with mycophenolate mofetil or azathioprine. 7
For brain and nervous system involvement (neuropsychiatric lupus):
- If caused by inflammation: high-dose steroids and immunosuppressive drugs like cyclophosphamide 7
- If caused by blood clots: blood thinners (anticoagulation) 7
- Sometimes both mechanisms occur together and require combination treatment 7
For severe low platelet counts:
- Initial treatment with high-dose IV steroids 7
- Addition of immunosuppressive drugs (azathioprine, mycophenolate, or cyclosporine) 7
- IVIG (intravenous immunoglobulin) may be used in acute situations 7
- Rituximab or cyclophosphamide for cases that don't respond to standard treatment 7
What to Expect Long-Term
Lupus is a chronic disease with periods of increased activity (flares) and periods of remission. 3, 6
The goal of treatment is to achieve remission or very low disease activity with minimal symptoms and minimal need for steroids. 1, 7
You will need regular monitoring with blood tests (including anti-dsDNA antibodies, complement levels C3 and C4, complete blood count, kidney function, and urinalysis) at each visit. 7
People with lupus have a 5-fold increased risk of death compared to the general population, primarily from active disease, infections, and cardiovascular disease. 7
About 10% of people with lupus kidney disease develop end-stage kidney disease requiring dialysis after 10 years. 2
Important Complications to Monitor
Infections are a major risk because both the disease and the medications suppress your immune system. 9, 6
Cardiovascular disease occurs prematurely in lupus patients, so controlling blood pressure, cholesterol, and diabetes is critical. 7
Osteoporosis risk increases, especially with long-term steroid use. 1, 7
Mental health problems including depression and anxiety are common and should be addressed. 9
You should not receive live vaccines while on immunosuppressive medications. 9
Special Considerations for Pregnancy
If you're planning pregnancy, discuss this with your doctor before conceiving. 9
Safe medications during pregnancy include prednisone, azathioprine, hydroxychloroquine, and low-dose aspirin. 7
You must stop mycophenolate mofetil, cyclophosphamide, and methotrexate before pregnancy as they can harm the developing baby. 7
If you have antiphospholipid antibodies, you'll need blood thinners during pregnancy to prevent miscarriage. 7
Key Points to Remember
Take your hydroxychloroquine every day—non-adherence is associated with more flares and worse outcomes. 7
Protect yourself from the sun daily with sunscreen and protective clothing. 1, 7
Report any signs of infection immediately (fever, chills, painful urination, cough with mucus). 9
Work closely with both your rheumatologist and primary care doctor. 1
Attend all scheduled appointments and blood work monitoring. 7