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WHAT ARE RHEUMATIC DISEASES?
Rheumatic diseases are a diverse group of chronic diseases united by the presence of chronic inflammation, usually of unknown cause, affecting structures of the musculoskeletal system, blood vessels, and other tissues. Pediatric rheumatology, the study of rheumatic diseases in children and adolescents, had its origins in the first half of the 20th century, principally as a study of chronic inflammatory arthritis, the most common of the childhood rheumatic diseases.
Archeological evidence supports the existence of chronic arthritis in children as long ago as 900 AD.1,2 The first English-language reference to “rheumatism” in children is in the 1545 text by Thomas Phaire.3 In this work, the author refers to the “stifnes or starckenes of the limmes” resulting from exposure of a child to cold, a complaint that may not represent any specific rheumatic disease. Three hundred years later (1864) Cornil described a woman in whom polyarthritis had developed when she was 12 years old.4 Autopsy at 28 years of age documented ankylosis of some joints and synovial proliferation with marked destruction of cartilage in others. Several small case series were published in the last half of the 19th century,5,6 but the disease was thought to be very rare.7 The diversity of chronic arthritis was recognized in the latter part of the 19th century.
In 1883, Barlow chaired a discussion on rheumatism in childhood at a meeting of the British Medical Association, Section of Diseases of Children.8 In the report of this meeting, the term rheumatism was used to describe poststreptococcal disease, including acute rheumatic fever. Barlow recognized the extent and complexity of these disorders in childhood: “For there are in children many affections of joints, and of structures around joints, which do not suppurate, and yet are not rheumatic; and there is much rheumatism in children which does not affect joints.” Disorders known today as toxic synovitis of the hip, acute pyogenic arthritis, syphilitic arthritis, hemophiliac arthropathy, Henoch–Schönlein purpura, poststreptococcal arthritis, and acute rheumatic fever, including carditis,arthritis, nodules, erythema marginatum, and chorea, are all identifiable in this paper.
In 1891, Diamant-Berger published the first detailed account of chronic arthritis in 38 children whom he had seen or whose cases had been documented in the literature. In 1896, George Frederic Still described 22 cases of acute and chronic arthritis in children, almost all of whom were observed at the Hospital for Sick Children, London.This treatise, written under the mentorship of Barlow,11 documented the clinical characteristics and the differing modes of onset of disease in these children. Still was the first English physician to confine his practice to diseases of children and the first professor of pediatrics at King’s College Hospital Medical School, London. After his classic study, however, he rarely returned to the field of pediatric rheumatology. In the same year, Koplick12 described the first American child with chronic arthritis.
Although these publications that described arthritis in childhood marked important milestones in the early development of pediatric rheumatology, other rheumatic diseases were identified in children in the 19th century. The clinical characteristics of leukocytoclastic vasculitis were described by Schönlein13 and Henoch14 in the early to mid1800s. Juvenile dermatomyositis was first identified by Unverricht15 and others in 1887, although it was not until the mid-1960s that significant experience with this disease in childhood was reported. Systemic lupus erythematosus (SLE) has been recognized in children since at least 1904.16 The original description of scleroderma was in a 17-year-old girl,17 but the disease was rarely diagnosed in children until the early 1960s. Ankylosing spondylitis was perhaps first identified in a child18—it was certainly known to occur in childhood in the 1950s19 but specific studies of the disorder in children did not emerge until the late 1960s.