Juvenile systemic lupus erythematosus (SLE): Also, termed as pediatric systemic lupus erythematosus (pSLE), juvenile systemic lupus erythematosus is a severe, multi-system, autoimmune disorder that occurs due to the failure of the immune system to demarcate between self and non-self cells. Consequently, the immune system mistakenly attacks the body’s own cells and tissues by considering them as foreign invaders and elicits an inflammatory response.
The term juvenile refers to its early onset in children, belonging to the age group of 3-15 years, with the mean onset age being 9 years. Accounting for about 20% of all systemic lupus erythematosus (SLE) cases, the rate of incidence of juvenile systemic lupus erythematosus is about 4 times more in girls than in boys.
The word systemic originates from the ability of the disease to affect various body parts like heart, lungs, kidney, skin, hematopoietic system and the central nervous system. However, joints is the most common form of connective tissue that is damaged by systemic lupus erythematosus and hence SLE is often considered as a form of arthritis, which is typically characterized by prolonged inflammation and degeneration of the joints.
Causes of Juvenile Systemic Lupus Erythematosus
The progression of the diseases changes unpredictably with alternating periods of sudden pain called as flare ups with periods of remission or temporary disappearance of the symptoms. Like any other auto-immune disorder, the causes of juvenile SLE are not very well known, with just about 5% of the causative factors being attributed to genetic and environmental agents.
Dysfunctional Immune System
Our immune system, which is basically involved in killing and warding off foreign substances has an inherent mechanism for differentiating self from non-self cells. However, this protective function of the body can become detrimental when the immune system produces antibodies against its own antigens and elicits a prolonged immune response, thus leading to the uncontrollable destruction of the body’s own cells and tissues.
The presence and amount of auto-antibodies found in the serum of patients suffering from SLE has been directly associated with the intensity and progression of the disease. The precipitation of antigen-antibody complexes leads to a Type III hypersensitivity reaction, which triggers inflammation and causes considerable damage to the surrounding tissues.
Over-Reactive B-lymphocytes and T Cells
Abnormal proliferation of B-lymphocytes, a type of immune cells that produces antibodies and inappropriate instigation of self-reactive memory B-cells leads to the production of antibodies against the body’s own cells. Additionally, when the T cells encounter antigen-antibody complexes, they accelerate the release of cytokines and other inflammatory agents by several folds.
This impairment in the activity of immune cells, namely B and T lymphocytes leads to inflammation and joint aches. Also, the serum levels of regulatory T cell that enables T-lymphocytes to identify foreign invaders from the body’s own cells have been found to be very less in children suffering from SLE.
Symptoms Associated with Juvenile Systemic Lupus Erythematosus
Derived from the word wolf in Latin, lupus was thought to occur as a result of a wolf bite due to the presence of round, disc shaped rashes on the skin that resembled wolf-bites.
Early Warning Signs
In its early stages, juvenile systemic lupus erythematosus is manifested in the form of fever, fatigue, anemia, alopecia, loss of appetite, color change in fingers, when exposed to cold or stress and ulcers in the mouth, nose and vagina.
Skin manifestations like butterfly-shaped eruptions or malar rashes on the face and skin lesions that show extreme sensitivity to sunlight are the most prominent features of systemic lupus erythematosus.
Facial rashes appear as thick red or purple scaly patches and are especially found at the bridge of the nose and the cheek.
The recruitment of inflammatory markers to the joints as a result of an increase in the formation of antigen-antibody complexes leads to severe joint pains and swelling of the joints. Muscle aches often occur due to the damage and injury caused to the muscle tissue.
Juvenile SLE carries additional health risks in children by gradually affecting and damaging the organ system in the body. Over a period of time, prolonged inflammation facilitates the progression of the disease, gradually spreading the damage to other internal organs. Hence, organ damage is common in 55% of the children suffering from SLE, as per the standards of American College of Rheumatology.
Damage to Kidneys
Dark urine, swelling of feet, legs and eyelids, breathlessness and chest pain are the most noticeable signs of kidney damage due to juvenile SLE. Children suffering from SLE are far more susceptible to kidney damage than adults. The accumulation of auto-antibodies in the kidneys and the subsequent inflammation and damage to kidneys leads to renal failure.
Damage to the Brain
The most devastating effects of juvenile SLE is unveiled in the form of brain damage, especially to the areas that are involved in memory and learning. Furthermore, disturbances in nerve impulse transmission lead to the onset of seizures, headaches and momentary loss of cognitive abilities.
Even though, there is no cure for juvenile systemic lupus erythematosus, the rise in medical and technological inventions have dramatically increased the number of children surviving after being affected with this debilitating condition. Besides, it is important to bear in mind that the symptoms of juvenile systemic lupus erythematosus can be easily confused with other diseases due to the varying and multiple symptoms that this incapacitating disease is associated with.
In fact, SLE is also called as “the great imitators”, since it is most often mistaken for signs of other infectious diseases and remains undiagnosed for years together. Hence, a very stringent examination and diagnosis is required to establish juvenile SLE, which is often done by a professional health care practitioner on the basis of the criteria set on the basis of the standards set by the American College of Rheumatology (ACR).