What is Systemic Lupus Erythematosus?

Systemic lupus erythematosus is also known as SLE or lupus. SLE is a long term autoimmune disorder which can affect various organs in the body like the skin, joints, kidneys, brain, heart, lungs and blood. The number of organ systems affected varies between patients and also during the course of disease.

How common is SLE in children?

SLE is a rare disease and in Taiwan, it affects 6 in every 100,000 children. We do not currently have any data from Malaysia but Asians are more commonly affected compared to Caucasians.

Who gets SLE?

SLE normally affects women of the reproductive age group (between 20-40 years old). However, 15-20% of SLE cases occur in children and teenagers. SLE is rare under the age of 5 years and most children present around puberty. As a whole, girls tend to be affected more than boys.

What causes SLE?

The exact cause of SLE is unknown. It is likely that a combination of genetic predisposition, hormonal influence and multiple environmental factors are necessary to induce the disease. Unfortunately these factors have yet to be determined. Some known triggers of SLE include sun exposure, some viral infections, certain medications (e.g. procainamide, hydralazine) and hormonal imbalance at puberty.

Is SLE inherited?

SLE is not a hereditary disease as it cannot be passed directly from parents to their children. However, a parent may pass on some genes which makes the child more susceptible to develop SLE but may not necessarily pre-destined the child to develop it. It is not unusual for a child with SLE to have other family members with SLE or other autoimmune disease.

What happens in SLE?

In a healthy immune system, our body can distinguish between our own tissues which is harmless and that which is foreign and potentially dangerous, which it destroys. In SLE, the immune system loses this ability and mistakenly produces various autoantibodies which attack its own healthy tissues. This autoimmune reaction causes inflammation of the affected organs, and if untreated will result in organ damage and impairment of function.

What are the symptoms and signs?

Every child is different and the symptoms can vary between children. They can present acutely or insidiously over weeks or months, and symptoms may range from mild to severe and life threatening. Sometimes the symptoms may also come and go. Some symptoms may develop at the beginning or appear at any time during the course of the disease.

Common symptoms include:

• Fever which is prolonged, usually intermittent
• Feeling very tired, and generally unwell
• Loss of appetite and loss of weight

However, these are non-specific symptoms and do not necessarily indicate SLE.

Other more specific symptoms according to the affected organs are :

1. Skin and mucosa:
   • Various types of rashes, especially malar or butterfly rash. Note that malar rash is not present in all children with SLE at diagnosis, and the absence of such a rash does not exclude the diagnosis
   • Hair loss , ulcers in the mouth or inside the nose
   • Hands turning white, blue and red upon exposure to cold (Raynaud’s)
2. Musculoskeletal
   • Joint pains and/ or swelling
   • Muscle pains and/ weakness
3. Kidney
   • Swelling of both eyelids and legs, frothy urine or blood in the urine
4. Brain
   • Headache, dizziness, fits, numbness of hands and /feet, stroke
5. Heart
   • Palpitations, breathlessness on exertion, chest pain
6. Lungs
   • Breathlessness, chronic cough, coughing out blood
7. Intestines
   • Diarrhoea, vomiting, abdominal pain
8. Blood
   • Pale, jaundice, easy bruising, swollen lymph nodes

Is SLE in children then same as SLE in adults?

SLE in children is generally similar to SLE in adults. However, the disease in children tends to be more aggerssive and also changes more rapidly.

How is childhood SLE diagnosed?

Your doctor will take a detailed history of your child’s symptoms, perform a physical examination looking for specific signs and perform various laboratory tests before coming to a diagnosis. The diagnosis in children can sometimes be difficult as the symptoms and signs may not be obvious especially at the beginning of the illness resulting in a delayed diagnosis.

What and why laboratory tests are needed?

Blood and urine tests are routinely required. Other tests like chest x-ray, Echocradiogram (heart scan) and electrocardiogram (ECG) for the heart, electroencephalogram (EEG) and various scans (CT scan or MRI) for the brain, ultrasound scan of abdomen/kidneys, pulmonary function tests for the lungs, bone marrow examination or lumbar puncture and various tissue biopsies may be indicated depending on symptoms.

All these tests are important to make the diagnosis of SLE, to assess for internal organs involvement (if any) and also to look for complications of the disease. Subsequently, blood, urine and other tests are also required to monitor for disease activity and to look for side effects from the medications given.

What are the possible complications?

Complications can arise from the disease itself (e.g. uncontrolled kidney disease can lead to kidney failure) or from the effects of treatment. However, it is important to be compliant to your doctor’s instructions to ensure the best possible outcome for your child. SLE can be fatal.

Is there a cure?

There is currently no cure for SLE. Treatment is aimed at controlling the inflammation and preventing complications of untreated disease. At the initial stage, a child may need a lot of medications as the disease is very active. However, most children respond very well to treatment and this may then allow for gradual reduction of medications. Do not stop any medications yourself even though your child looks well as it may cause a flare of the SLE.

How is childhood SLE treated?

The type of treatment a child receives depends on the severity of symptoms and the organ system/s which are involved. Those with internal organ involvement like the kidney or brain will require more aggressive treatment. As SLE affects each child differently, it may take a while before the right combination of medicines are found to control the disease.

Some or all of the following drugs may be indicated.

• Corticosteroids – this is the mainstay of treatment. It is normally given by mouth or by a drip into the vein. Sometimes, steroid eyedrops (for eye inflammation) and steroid creams (for rashes) may be needed.
• Hydroxychloroquine
• Immunosuppresants : Azathioprine, Mycophenolate mofetil, Methotrexate, Cyclosporin A, Cyclophosphamide
• Non-steroidal anti-inflammatory drugs (NSAIDs)
• Others : vitamins like Folic acid, calcium and vitamin D supplements, antihypertensive medications, Aspirin/Heparin/Warfarin (for those with antiphospholipid syndrome)

What else can I do apart from taking medications?

It is also very important to:

• Avoid too much sun exposure as sun exposure can trigger a flare. Use sunscreen on sun exposed areas and practice sun avoiding techniques (e.g. umbrella, hat, long sleeve shirts and pants, tinted vehicles)
• Minimise stress and ensure adequate rest
• Try to avoid infection and illnesses e.g. wash hands regularly, avoid people who are sick, avoid crowded places
• Eat a healthy diet
• Exercise regularly while avoiding sun exposure

Are there any foods which SLE patients need to avoid?

There is no food that needs to be avoided in particular. Ensure that your child eats a healthy balanced diet which is required for growth. If your child is on steroids and has an increased appetite, it is advised to take a low calorie (low sugar & low fat) and low salt diet; and exercise regularly. Your child may need calcium and vitamin D supplements to prevent osteoporosis. No other vitamin supplement is scientifically proven to help SLE.

What about vaccinations?

Children with SLE are encouraged to take their vaccinations. However, there are certain vaccines containing live viruses that should be avoided by SLE patients on immunosuppressive medications. Please check with your child’s doctor before proceeding with any vaccinations.

What about unconventional / complementary therapies?

There are many unconventional therapies which are proposed for SLE patients and you have to think carefully about non-qualified medical advice and its complications. Please discuss with your paediatric rheumatologist if you wish to try any of these.

What is the outlook?

SLE usually lasts for many years and there may be periods of remissions and flares. We are unable to predict the course of disease in your child.

Generally, the future outlook depends on the severity of disease especially internal organ involvement. Some children may have mild disease which can be easily controlled with medications whilst for some less fortunate ones, the disease can be severe resulting in long term organ damage and sometimes even death.

Many children with SLE will do very well, but some may continue to have active disease into adulthood. However, the outlook today has improved dramatically with the early and judicious use of steroids and immunosuppressive agents. There is also a lot of research is also in progress to look for more effective medications to treat SLE.