Systemic lupus erythematosus (SLE) is an autoimmune disease that presents in many ways. The disease is characterized by the production of autoantibodies that deposit onto tissue and fix complement on almost any organ which causes systemic inflammation. Typically it affects women (9:1) aged between 15 and 45 years and it tends to affect people of African-American, Asian, and Hispanic descent more so than Caucasians: 3 to 4 times higher.[1]
This is a very complex condition and we’re still trying to understand the underlying cause and trying to find effective treatments. Even diagnosis remains challenging at times. Today I’d like to go over 10 important warning signs of systemic lupus.
Criteria for the classification of SLE
The following are the criteria for the diagnosis of SLE. To fulfill criteria you need to have at least 4 criteria with at least one coming from the clinical section and one coming from the immunological section. An exception to the rule is if someone has a kidney biopsy that shows lupus nephritis in the presence of a positive ANA or other lupus-related autoantibody.
It’s important to note that there are exceptions to the rule. These criteria are meant to be used in research and we all know that real life sometimes doesn’t fit the mold! Also, as researchers make more discoveries, classification systems change. The Autoimmunity Blog has a great pdf showing how these criteria evolved with time.
Systemic Lupus International Collaborating Clinics Classification Criteria for SLE (2012)
| Criterion | Definition |
| Clinical Criteria | |
| 1. ACLE (acute cutaneous lupus erythematosus) | Malar rash, bullous lupus, TEN variant, maculopapular, photosensitive, subacute cutaneous lupus |
| 2. CCLE (chronic cutaneous lupus erythematosus) | Classic discoid, hypertrophic, lupus panniculitis/profundus, mucosal, lupus erythematosus tumidus, chilblains lupus, discoid/lichen panus overlap |
| 3. Alopecia | Non-scarring, diffuse hair thinning or visible broken hairs |
| 4. Oral ulcers | Oral (palate, buccal, tongue) or nasal ulceration |
| 5. Synovitis | Arthritis involving two or more peripheral joints, characterized by tenderness, swelling, or effusion and morning stiffness > 30 minutes |
| 6. Serositis | Pleuritis: convincing history of pleuritic chest pain for >1 days or pleural rub or evidence of pleural effusion, or Pericarditis documented by EKG or rub, or evidence of a pericardial effusion |
| 7. Renal disorder | Persistent protein ≥ 0.5 grams/day or red blood cell casts |
| 8. Neurological disorder | Seizures, psychosis, myelitis, mononeuritis multiplex, peripheral or cranial neuropathy, acute confusional state |
| 9. Hemolytic anemia | Direct Coombs positive |
| 10. Leukopenia | Leukopenia < 4000/mm³ at least once or lymphopenia < 1000/mm³ at least once |
| 11. Thrombocytopenia | Platelets < 100 000/ mm³ |
| Immunological Criteria | |
| 1. Positive ANA | Level above laboratory reference |
| 2. Anti-dsDNA | Level above laboratory reference range (or >2-fold ELISA reference range) |
| 3. Anti-Sm | Presence of antibody to Sm nuclear antigen |
| 4. Antiphospholipid antibody
– Positive lupus anticoagulant – False positive for rapid plasma regain – Medium titer or high titer anticardiolipin antibody level – Positive anti-β-glycoprotein I |
|
| 5. Low complements | Low C3, low C4, or low CH50 |
| 6. Direct Coombs test | In the absence of hemolytic anemia |
RheumDoctor’s simplified lupus criteria
As you can see these criteria are somewhat complicated and use A LOT of “medicalese”. Let’s try simplifying things! Basically, the criteria include clinical features and blood tests that show abnormal changes with the immune system. The clinical criteria do include some blood tests like white cells, blood cells, platelets, and urine, but these are NOT tests that specifically show problems with the immune system. They are abnormal BECAUSE the immune system is affecting them.
10 Important warning signs of systemic lupus erythematosus
- Rash
- Hair loss
- Oral and/or nose ulcers
- Autoimmune joint pain
- Chest pain
- Kidney problems
- Neurologic or psychiatry changes
- Anemia
- Frequent infections
- Easy bruising or bleeding
Other symptoms that are not included in the criteria include profound fatigue, fevers, unintentional weight loss, and Raynaud’s phenomenon.
Rash
The first thing that pops up in most people’s mind when they think about lupus is the famous “butterfly” rash, more specifically the malar rash. This type of rash is one of many ways the disease can inflame the skin. DermNet New Zealand has a great selection of examples. People with lupus also tend to get a rash when their skin is exposed to the sun (photosensitivity). Sometimes exposure to the sun can cause a systemic flare, e.g., cause joint pain, swelling, and fatigue.
Hair loss
Unless the hair loss is caused by a rash, e.g., discoid lupus, hair loss tends not to scar. It can happen in patches or simply be generalized.
Oral and/or nose ulcers
Almost everyone gets a canker sore once in a while. People with lupus often get multiple ulcers over and over again. They usually DON’T hurt.
Autoimmune joint pain
Autoimmune joint pain or inflammatory arthritis looks very similar to the joint pain that people have with rheumatoid arthritis. However, joint inflammation caused by lupus is NON-EROSIVE, meaning that people do not get permanent joint deformities. Like rheumatoid arthritis, joints get swollen, tender, and they tend to stay stiff for at least 30 minutes to an hour in the morning.
Chest pain
Lupus can cause pleuritic and pericarditis. Basically, the lining of the lungs (pleuritic) or the lining of the heart (pericarditis) get inflamed. This can cause sharp chest pain and sometimes can cause fluid to accumulate around the lungs or heart. Usually this type of chest pain worsens when you take a deep breath and in the case of pericarditis, improves when you lean forward.
If you are experiencing chest pain, don’t mess around, go to the emergency room. People with lupus have a higher risk of heart disease. In fact, cardiovascular disease is the leading cause of death in people with lupus. So you need to rule out a heart attack.
Kidney problems
Lupus can cause inflammation in the kidneys (lupus nephritis). There are 6 types. It’s important to know what type we’re dealing with when making treatment decisions. Lupus nephritis doesn’t cause “kidney pain”. The symptoms of kidney malfunction include:
- Weight gain (water weight)
- Uncontrolled high blood pressure
- Dark urine
- Frothy or foamy urine
- Leg swelling
- The need to urinate during the night
Neurologic and/or psychiatric changes
These are probably the most difficult to diagnose features of lupus. They vary widely are terribly non-specific. People with lupus who present with neurological symptoms can present with a stroke, limb weakness, small fiber neuropathy, seizures, and even psychiatric changes like schizophrenia or major depression. Close collaboration with a rheumatologist, neurologist, and psychiatrist is often required.
Anemia
Lupus can cause many different types of anemia: anemia of chronic disease, hemolytic anemia, kidney failure, etc. Some symptoms of anemia include:
- Loss of energy
- Rapid heart beat
- Shortness of breath
- Headache
- Difficulty concentrating
- Dizziness
- Pale skin
- Leg cramps
- Insomnia
- Hemolytic anemia can also cause yellowing the eyes/sclera (jaundice)
Frequent infections
Many people with lupus get frequent infections. For the most part this is caused by medications used to treat lupus but sometimes it can be the main culprit. Lupus can cause white blood cells and specialized white cells called lymphocytes to decrease in number. These cells are part of the immune system and help your body fight off infection. If they are critically low, you can get frequent infections.
Easy bruising and bleeding
Platelets are specialized cells in your blood that prevent bleeding and help stop bleeding. Lupus can cause a reduction in platelet levels. Some symptoms of low platelets (thrombocytopenia) include:
- Easy bruising with minimal trauma
- Spontaneous + superficial bleeding into the skin (petechiae)
- Prolonged bleeding
- Bleeding from the gums after brushing your teeth
- Unusually heavy menstrual flow
- Blood in your urine or stool
- Having a big spleen
I think I may have lupus?
If you think you may have lupus, talk to your primary care physician, your GP, or make a consultation with a licensed rheumatologist. Remember this is not a common disease, so more often than not, it isn’t lupus. Because the condition can affect many organ systems, it can mimic many diseases. Most of these are much more common than lupus.
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Medical Disclaimer
This information is offered to educate the general public. The information posted on this website does not replace professional medical advice, but for general information purposes only. There is no Doctor – Patient relationship established. We strongly advised you to speak with your medical professional if you have questions concerning your symptoms, diagnosis and treatment.
References
[1] West, SG. Rheumatology Secrets 3rd edition