SjÖgren Syndrome The syndrome of chronic inflammation of the exocrine glands, principally the salivary and lacrimal glands, was first described by Henrik Sjögren, a Swedish ophthalmologist, who published in 1930 the first complete description of a disorder he named keratoconjunctivitis…
Scleroderma refers to hard skin that results from an excessive deposition of collagen. In the localized form (localized scleroderma [LS], also called morphea ), inflammation in the skin and subcutaneous tissues triggers the process of fibrosis. LS and the systemic…
Introduction The scleroderma disorders in children encompass both systemic sclerosis (SSc) and localized scleroderma (LS), also termed morphea . Juvenile systemic sclerosis (JSSc) is much less common than juvenile localized scleroderma (JLS) and comprises less than 10% of all SSc…
Idiopathic inflammatory myopathies (IIM) are a heterogenous group of diseases characterized by muscle weakness (which is often more pronounced proximally) and inflammatory changes in skeletal muscle. IIM comprise different subtypes: dermatomyositis, polymyositis, inclusion body myositis, and amyopathic dermatomyositis. Juvenile dermatomyositis…
Neonatal lupus erythematosus (NLE) is a disease of the developing fetus and neonate defined by characteristic clinical features in the presence of specific maternal autoantibodies. The first reported case of congenital heart block (CHB) associated with maternal autoimmune disease (i.e.,…
Antiphospholipid syndrome (APS) is an autoimmune multisystem disease primarily manifested as intravascular thrombosis and mediated by antiphospholipid antibodies (APLA). APS may occur as an isolated clinical entity (primary APS) or in association with other autoimmune diseases (secondary APS), particularly systemic…
Systemic Lupus Erythematosus Systemic lupus erythematosus (SLE) is the prototypic systemic autoimmune disease. It is characterized by multiple autoantibodies associated with a multisystem illness where any organ can be targeted. Autoantibodies most commonly associated with SLE are antinuclear antibodies (ANAs)…
Inflammatory eye diseases comprise some of the most devastating complications of childhood rheumatic diseases, especially juvenile idiopathic arthritis (JIA). Chronic (initially asymptomatic) uveitis accompanying JIA is one of the most common causes of uveitis in childhood. It is predominantly anterior,…
The association of arthritis with psoriasis was described almost 200 years ago, but it was not reported in children until the 1950s. Psoriatic juvenile idiopathic arthritis (PsJIA) is a heterogeneous entity, recognized in patients with frank psoriasis but also where…
Definition and Classification Under the International League of Associations for Rheumatology (ILAR) classification of juvenile idiopathic arthritis (JIA), , enthesitis-related arthritis (ERA) is defined according to the inclusion and exclusion criteria shown in Table 20.1 . Children with ERA have…