Definition

Juvenile systemic lupus erythematosus (Juvenile lupus, SLE) is a systemic connective tissue disease that is identical to SLE in adults, but with the onset of illness in childhood or adolescence.

• SLE can cause skin, joint, kidney, lung, nervous system and some other organs
• Blood samples show markedly elevated ANA test (antinuclear antibodies) and most often in special subgroups of ANA
• SLE is a chronic disease that can last for many years

Occurrence

SLE is generally rare in children and is virtually absent before 5 years of age. Most people who become ill are adolescents and adult women between 15 and 45 years, but about 20% get SLE before 20 years of age. Women / girls are 10 times more frequent than men / boys. The prevalence is higher among Africans, African Americans and Asians than ethnic Norwegian.

Symptoms

• Ny tiredness / fatigue
• Night sweats
• Fever
• Joint Pain and joint swelling (often finger joints),
• Rashes on body, arms, legs and / or face (”butterfly rash")
• Convulsions
• Kidney problems,
• Low hemoglobin blood changes, low white blood cell count (leukocytes), low platelet count (platelets)

Results of medical examinations

Fever, rash, Arthritis (inflammed joints), urine with proteins and / or traces of blood, elevated sedimentation rate (ESR), low CRP, low blood cells (red, white, platelets), heavily elevated ANA test, antifosfolipid-Antibodies. Low complement factors (C3, C4).

Disease Cause

Unknown. No virus, bacteria or other infection has been detected. Hereditary (genetic) disposition may matter, but close relatives rarely have SLE. Environmental factors such as tanning and hormone changes (girls at puberty) are also believed to be contributing.

• Once the disease has begun, the immune system is too active and damages the body's own tissues by mistake. This causes rheumatic inflammation that damages the joints, skin, blood and internal organs

SLE-like skin changes + Vasculitis in children: STING mutation

Diagnosis

SLE is detected when a combination of several typical symptoms and examination findings are available. Since these are developed over time, it can take weeks to months before the diagnosis is certain.

• It is always important to exclude other diseases that may initially give similar signs of disease.
• Checklist for the investigation and follow-up of juvenil SLE, please see separate page here

Incorrect diagnosis (differential diagnoses, similar diseases)

• Aicardi-Goutieres Syndrome (AGS)
  • Neurological symptoms
  • Chilblain symptoms on fingers and toes
• Please see the page that also includes adults with SLE here

The treatment

Drugs that inhibit the overactive immune system prevent organ damage can be vital to juvenile lupus. They turn down the disease activity and keep it in check. One can not cure SLE. Proper drug selection and proper dosage require experience with SLE in children and is a specialist task. Frequently consulted in Norway is the National Competency Service for Child and Youth Rheumatology (NAKBUR) at Oslo University Hospital, Rikshospitalet consulted. Among the medications are Prednisone, Plaquenil, Azathioprine and / or other immune suppression most commonly used.

Diet

Studies do not indicate that particular diet or supplements of vitamins or trace elements have special effect.

• However, in the case of long-term illness and medication, regular meals with a well-balanced diet are important to prevent weight loss, lack of protein, vitamins, lime and other minerals. This is especially important for children in growing periods.
• Prednisolone (cortisone) is often supplemented with calcium and vitamin D to reduce the risk of osteoporosis (osteoporosis.).

Prognosis and prevention

Medication causes juvenile lupus to enter quiet phases, but can typically flare up again, even after several years. Long-term, regular use of drugs is therefore important.

• It is known that sun rays and tanning can both cause eczema and cause a general relapse of the disease.
• Some of the medications can also cause problems with tanning. Caution and use of high factor sunscreen is recommended. The course of the disease is very individual.
• Some are severely attacked, while others live an almost normal life with few symptoms.

To live with juvenil lupus

It can be very daunting to hear that you or your child have SLE. A good way to deal with the disease is often to inform you well about SLE, follow up on the doctor's advice and meet up with the controls. There should be a collaboration between the specialist and the GP, and one should have the opportunity to quickly get in touch if the disease requires it.

Research

At the Children's Rehabilitation Unit, Oslo University Hospital, Rikshospitalet, there are ongoing research projects. A doctoral dissertation was written in 2007.

Literature

• Habibi S, 2011 (pdf)
• More about SLE in children here (in English) (PRINTO)
• More about SLE here (Bindevevsykdommer.no)

Children with rheumatic disease, BINDEVEVSSYKDOMMER.no

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• Bindevevssykdommer.no
• Vaskulitt.no