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[Neurological involvement in rheumatic disorders and vasculitis in childhood]
1Unitat integrada Hospital Clinic-Hospital Sant Joan de Déu, Barcelona, España. pineda@hsjdbcn.org
Insights
Rheumatic disorders and vasculitis involve inflammation affecting connective tissues, leading to autoimmune responses and potential neurological complications in children. Treatment typically involves corticosteroids and immunosuppressants.
Area of Science:
- Rheumatology
- Immunology
- Neurology
Context:
- Rheumatic disorders and vasculitis are inflammatory conditions impacting connective tissues across various organs.
- These processes involve immune system dysregulation, including the production of antibodies and autoimmune complexes.
- Inflammation can lead to tissue damage through the release of destructive enzymes.
Purpose:
- To review the classification of rheumatic disorders and vasculitis.
- To describe the most frequent neurological complications observed in pediatric patients.
- To highlight specific conditions like infantile systemic lupus erythematosus, juvenile idiopathic arthritis, and Sydenham's rheumatic chorea.
Summary:
- Infantile systemic lupus erythematosus affects multiple organs, with 25% of patients experiencing neurological symptoms such as convulsions and chorea.
- Juvenile idiopathic arthritis can present with neurological involvement in 5% of cases, including cerebral vasculitis and peripheral nerve issues.
- Vasculitis, characterized by inflammation of blood vessel walls, can lead to neurological complications, though uncommon in some infantile forms like Kawasaki disease.
Impact:
- Provides an overview of pediatric rheumatic and vasculitic conditions with neurological manifestations.
- Aids in understanding the diverse range of neurological complications associated with these inflammatory disorders in children.
- Informs clinical awareness and diagnostic approaches for pediatric rheumatological and neurological presentations.
Introduction:
The rheumatic disorders and vasculites are inflammatory processes affecting connective tissue in different organs. The inflamed cells liberate destructive enzymes which harm tissues, producing IgE, IgM, IgG antibodies and autoimmune complexes.
Objective:
To review the current classification of these conditions and describe the commonest neurological complications in children. Infantile systemic lupus erythematosus is the commonest; it affects the dermis, joints, blood vessels, heart, kidney and brain. Neurological features: vertigo, ataxia, convulsions, chorea and headache are seen in 25% of the patients. Juvenile idiopathic arthritis is another inflammatory disorder of unknown origin which affects joints, connective tissue and visceras. Neurological involvement is seen in 5%, in the form of cerebral vasculitis, cervical myelopathy, trapping and compression of peripheral nerves and neuropathy due to arteritis. Sydenham s rheumatic chorea, caused by b haemolytic streptococcal A infection, causing a pathological auto immune response is the best known and has increased in frequency again in recent years. Vasculitis is caused by inflammatory changes in the walls of large, medium and small blood vessels. They lead to deposits of auto immune antigen antibody complexes in the blood vessel walls. In the commonest infantile vasculites neurological complications are uncommon, but not rare, as occurs with Kawasaki disease, infantile Wegener s granulomatosis, Schönlein-Henoch purpura and Behçet's disease. The usual treatment for this group of disorders is corticosteroids and immunosuppressive drugs.