July 16, 2021 9:59:26 am
By Dr Anju Singh
Rheumatological diseases in children arise from dysregulation in their immune system. As a result, there is a state of exaggerated immune response in the form of hyper-inflammation that attacks its own cells and organs such as joints, eyes, skin, lung, heart, gut, and brain; and produces a spectrum of various diseases known as autoimmune rheumatic diseases. Most of them are idiopathic in nature, i.e., the underlying cause is not known. Broadly speaking, these can be categorised as juvenile idiopathic arthritis (JIA), connective tissue disorders (CTDs), primary vasculitis, and autoinflammatory syndromes.
Juvenile idiopathic arthritis is the most common type of rheumatic disease in children, affecting 1 in 1000 children. It includes various subtypes classified based on the number of joints, presence of fever, rash, RF, Anti-CCP autoantibodies, and HLA B27 antigen. Most of the time, these children present with pain, swelling, and stiffness in joints or limping. Most of them are first seen by orthopaedic surgeons. Some of them can present with just fever for more than two weeks, sometimes with non-fixed rash and pain in joints. Others can present with complaints in eyes such as diminution of vision, redness of eyes, whiteness in eyes (cataract), or co-incidental finding of uveitis (inflammation of the middle vascular layer of the eye called uvea) on routine eye screening.
Children with connective tissue disorders can have prolonged fever with oral ulcers, photosensitive skin rash, diffuse hair fall, and weight loss (as in systemic lupus erythematosus). Another group of children with juvenile dermatomyositis can present with fever, rash over eyelids, knuckles of fingers and toes with easy fatigability, inability to climb stairs/raise hands above the head or lift up the head off the pillow. Children with scleroderma or juvenile systemic sclerosis can present with thickening of the skin of the face (producing expressionless and wrinkleless face), arms and legs, and difficulty in swallowing food with reflux episodes.
Some children can have inflammation of blood vessels called vasculitis and can present with prolonged fever with weight loss, body aches, altered sensorium or shock-like state, or just with urinary complaints of blood in urine with skin rash. For example, children with Kawasaki disease can present with fever, redness of eyes and mouth, rash all over the body, and swelling of hands and feet.
Another group of children belonging to autoinflammatory syndrome can have recurrent episodes of fever with rash, joint pains, persistent loose stools, sore throat, chest, and abdominal pain. However, in between these episodes, the child remains healthy with normal growth and development.
Broad knowledge about the various types of pediatric rheumatic diseases is essential to recognise the disease pattern and offer the child early diagnosis and the best possible treatment, thus ensuring the most optimal outcome.
In the current covid pandemic, though children have asymptomatic to mild illness, a surge in postcovid 19 autoimmune and autoinflammatory complications has been observed in children, such as MIS-C (Multisystem Inflammatory Syndrome-Covid related) and chilblain skin lesions. MIS-C usually mimics Kawasaki disease or presents just as prolonged fever. In recent times, another entity identified post covid illness is steroid-induced avascular necrosis (AVN) of bones, especially hips and knees. Since kids with MIS-C and severe covid illness require a high dose of steroids as the primary therapy, they make them prone to AVN. These children can be managed by a team of paediatric rheumatologists and orthopaedicians. Most of these kids respond well to medical therapy, and some may require surgical intervention depending upon the severity.
Tips and care for autoimmune diseases:
Most of these diseases have no known cause. But, kids must be fully vaccinated against various diseases as sometimes, autoimmune diseases get triggered by infection.
A healthy, nutritious diet and hygiene maintenance play a crucial role in preventing various infections and autoimmune diseases such as enthesitis-related arthritis and inflammatory bowel disease-associated arthritis.
Kids with juvenile idiopathic arthritis should avoid prolonged inactivity as it aggravates arthritis and should be enrolled in regular physiotherapy exercises for the range of movement of various joints.
Children with connective tissue diseases such as systemic lupus erythematosus and juvenile dermatomyositis should use sun-protective measures, e.g., sunblock creams on exposed parts with at least 30 SPF.
Children with Raynaud’s phenomenon (the fingers, toes, ears, and tip of the nose get numb, blue and icy on exposure to cold temperatures or stress) should keep their extremities warm by wearing gloves and socks in the winter season specifically.
Any child with prolonged fever for more than a month or so not responding to antibiotics with no detection of infection should consult a pediatric rheumatologist as in such a situation, rheumatic diseases constitute the second most common cause after infections.
Any child who has been detected with a type of uveitis called chronic (initially asymptomatic) anterior uveitis (inflammation of the middle layer of the eye called uvea) should consult a paediatric rheumatologist as idiopathic (unknown cause) uveitis or uveitis associated with juvenile idiopathic arthritis form the most common types, which are autoimmune in nature.
(The writer is Paediatric Rheumatologist, Senior Consultant, Madhukar Rainbow Hospital.)
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