How Severe is Your Lupus? Use a Lupus Severity Index

Our goal in treating systemic lupus erythematosus (SLE) is to get it into remission and on no, or very little, steroids (such as prednisone). This means getting rid of all inflammation using medications as well as ultraviolet light protection, vitamin D, and the other measures as discussed in my “Lupus Secrets.”

It is a good idea to get a sense of how severe your SLE is using a lupus severity index. Some patients resist additional treatment when we recommend it. They think that their lupus is not bad enough to warrant additional drugs or higher doses of the medicines they are taking.

On the other hand, you may feel that  your lupus is more active than your rheumatologist thinks. Some rheumatologists may underestimate the severity of someone’s lupus. You could help your rheumatologist by calculating the severity yourself, present the results to him or her to see what they think about therapy.

So, how can you measure how active your lupus disease activity is? … EASY!

The Systemic Lupus Erythematosus Disease Activity Index (SLEDAI for short) is a research tool that is used to measure lupus disease activity in research. Yet, it is also helpful for doctors to understand how active someone’s SLE is. Information from this lupus severity index can help make better decisions regarding treatment.

Here is an online calculator that makes it incredibly easy to calculate your score:

https://qxmd.com/calculate/calculator_335/sledai-2k

To use the SLEDAI (table 29.1), get a copy of the last note, and lab results from your rheumatologist.

Go to the online calculator and click “yes” or “no” for each question. It is essential to make sure that you completely fulfill the definition. For example, you may have severe arthritis from lupus in 2 joints. That does not count. You need 3 joints to be affected. You must click an answer for all 24 possibilities, then click on “Go to results” to see your final score.

IMPORTANT CAUTIONS WHEN DOING YOUR SLEDAI:

• Many to most patients will over score themselves. Please pay attention to these important recommendations. The SLEDAI is only for lupus inflammatory symptoms and NOT for type 2 symptoms (diffuse pain, fatigue, lupus fog, etc.)
• Only include those items active in the past 10 days
• Do NOT click yes for headaches. True lupus disease headaches are so rare, I’ve only seen 2 in my career. Lupus experts are recommending it even be removed from the lupus severity index
• Do NOT count visual disturbance. 99% of visual disturbances are not due to lupus inflammation. Only your ophthalmologist would know. You’d have to have had seen your eye doc in the past 10 days and they would have had to tell you that you have active inflammatory lupus retinopathy, maculopathy, or active optic neuritis (optic nerve damage, retina damage do not count)
• Do NOT mark CVA unless you had a new stroke in the past 10 days and it was 100% due to lupus (not from hardening of the arteries)
• Do NOT mark arthritis unless your rheumatologist tells you that you have active inflammatory arthritis of 3 or more joints (joint pain and other arthritis causes do not count)
• Do NOT click psychosis or organic brain syndrome unless your rheumatologist says these are active. Lupus fog and memory problems does not count.
• Do NOT mark alopecia unless a lupus dermatologist says you have active discoid lupus in your scalp causing hair loss or nonscarring alopecia from lupus. 90% of the time, hair loss in lupus is due to other causes (stress, telogen effluvium, androgenic alopecia, CCCA, traction alopecia, alopecia areata, etc) and these do not count
• Do NOT count mouth sores due to drugs such as methotrexate
• The labs have to be exactly as bad as they state in the definitions

Good luck on your scoring!

If your SLEDAI is 6 or higher, then you should discuss other treatment options with your rheumatologist. 

Type 2 Symptoms

VERY IMPORTANT: This calculator does not take into account what we call “Type 2 symptoms” of lupus. This looks at type one symptoms. Those are lupus problems that we can improve with medicines that calm down the immune system.

Type 2 symptoms are by far the hardest part of lupus to treat. These are the overall body pain, severe fatigue, trouble with memory, “lupus fog,” and sleep problems. I wish we had better treatments for these. This is a big unmet need in the treatment of our SLE patients. I’ll do a post later that discusses Type 1 vs Type 2 symptoms.

This method (as well as others) of measuring disease activity is not perfect. For example, suppose you have class IV lupus nephritis (kidney inflammation), your urine protein level (proteinuria) is 3 grams per 24 hours, your blood pressure is 190/110 (very high), your kidney function is decreased, and all other labs are normal. Your SLEDAI score is 4 (mild). However, all rheumatologists would agree that you have high disease activity (severe lupus) and are at increased risk for kidney failure if not treated aggressively.

Another example would be having a faint butterfly (malar) rash for 2 points and 3 slightly tender joints in the middle of the fingers with minimal discomfort (4 points). This person would be considered to have moderate disease activity in a research study using the SLEDAI score. However, it could be argued that this person is better categorized as having low disease activity.

And how can a rheumatologist make the mistake of thinking someone has mild SLE that does not need additional treatment, yet she has worse disease than he thinks?

Suppose a week ago, you had your typical monthly lupus flare. You were fatigued. And you had arthritis pains in multiple joints that kept you from doing all usual activities during your menstrual cycle. As usual, the flare resolved after a few days, and when you see your rheumatologist, you tell him that you feel great except for those few days. Physical exam shows 3 mildly tender joints, some small sores on the roof of your mouth that you don’t notice, and your labs show a slightly elevated anti-dsDNA and a mildly low C4 complement level.

Many rheumatologists would consider you as having “mild” lupus. You had a “mild” flare that only lasted a few days, and the physical exam only shows a few tender joints and mouth sores that you do not even notice. However, suppose you measure the SLEDAI score. In this case, you have a total of 10 points (arthritis, mucosal ulcers, increased DNA, and low complement). You clearly have moderate disease activity, bordering on HIGH disease activity.

Research studies show that even mild flares increase ongoing organ damage over time. You clearly need better therapy to control your SLE. This is a reason why we think it is a good idea for rheumatologists to measure the SLEDAI on their SLE patients. A lupus severity index can help them get a better understanding of how active their SLE is. ​​

I always recommend: Be proactive in your health care. Knowledge is power