Rheumatic diseases are a diverse group of chronic diseases characterised by the presence of chronic inflammation, usually of unknown cause, affecting structures of the musculoskeletal system, blood vessels, and other tissues. Pediatric rheumatology is the study of rheumatic diseases in children and adolescents .
In 1891, Diamant-Berger published the first detailed account of chronic arthritis in 38 children ,whose cases had been documented in the literature.Although these publications that described arthritis in childhood marked importance in the early development of pediatric rheumatology, other rheumatic diseases were identified in children in the 19th century.
Juvenile Idiopathic Arthritis :
Arthritis is one of the most common chronic diseases of children and youth, and an important cause of short- and long-term disability.There are many causes of chronic arthritis, but the most common are those grouped under the name juvenile idiopathic arthritis which involves onset before the age of 16 years, joint pain or swelling or limitation of movement for a duration of atleast 6weeks , with no metabolic , genetic, malignancy, infection or any other CTD cause.Delayed physical development as shown by growth and puberty may occur in all childhood rheumatic diseases.
Juvenile SLE :
Lupus is a complex illness at any age. The diagnosis can be particularly challenging during childhood and adolescence. Its nonspecific features, include fever, fatigue, weight loss, mouth ulcers, arthralgia, and headaches. The butterfly rash may be subtle or absent in children , but many exhibit marked photosensitivity. Hematologic and renal involvement is frequent as compared to adult lupus .It is important to assess patients carefully for complications associated with JSLE and adverse effects arising from their therapy.
Neonatal lupus syndrome is caused by transplacental passage of maternal autoantibodies - SSA/Ro and SSB/La to the fetus.Regular fetal monitoring from 16 weeks gestation is advised in all affected mothers to detect evidence of cardiac conduction defects and careful postnatal monitoring is essential. Skin, hepatic, and hematologic manifestations can be seen but are self-limiting .
It is the most common of the pediatric idiopathic inflammatory myopathies (IIMs).The mean age at onset is around 7 years of age. It typically presents with proximal muscle weakness and rash but frequently affects other organs like gastrointestinal, lung and heart. Proximal weakness in a child needs to be distinguished from infection , a neuromuscular disease such as a dystrophy; and myositis, which is part of another connective tissue disorder.
It affects the skin causing skin thickening , subcutaneous tissues, and underlying muscle or bone. In SSc, the disease involves internal organs and can cause pulmonary arterial hypertension, interstitial lung disease , gi involvement like reflux, malabsorption, pseudo obstruction, rectal incontinence. These are often preceded by Raynaud phenomenon.
As awareness of the broader spectrum of rheumatic diseases in children and adolescents emerged, it slowly became apparent that there was a body of knowledge and expertise—pediatric rheumatology— that was related to, but quite separate from, adult rheumatology, paediatrics.This speciality requires a diagnostic and therapeutic approach to the “whole” child and family unit and careful observation over long periods.
Dr. Spoorthy Kothapalli
Consultant Rheumatology
KIMS HOSPITALS, Kondapur.