The term vasculitis indicates the presence of inflammation in a blood vessel wall. The inflammatory infiltrate may be one that is predominantly neutrophilic, eosinophilic, or mononuclear. Perivasculitis describes inflammation around…
Introduction and Historical Review In 1937, the Turkish dermatologist Hulusi Behçet described the syndrome that bears his name, which manifests with the clinical triad of aphthous stomatitis, genital ulceration, and…
Etiology and Pathogenesis 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. It is…
Definition and Classification Enthesitis-related arthritis (ERA), a term introduced in the International League of Associations for Rheumatology (ILAR) classification of juvenile idiopathic arthritis (JIA), is defined according to the inclusion…
The antiphospholipid syndrome (APS) is an autoimmune multisystem disease characterized by thromboembolic events, pregnancy morbidity, hematological, dermatological, neurological, and other manifestations in the presence of elevated titers of antiphospholipid antibodies…
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…
Scleroderma refers to hard skin that develops because of an excessive accumulation of collagen. In the localized form (localized scleroderma [LS], also called morphea ), inflammation in the skin and…
The association of arthritis with psoriasis was described almost 200 years ago, but it was not reported in children until the 1950s. Juvenile psoriatic arthritis (JPsA) is a heterogeneous entity,…
Definition and Classification The arthropathies of inflammatory bowel disease (IBD) may be defined as any noninfectious arthritis occurring before or during the course of Crohn’s disease (CD), indeterminate colitis (IC),…
Connective tissue diseases (CTDs) are inflammatory conditions with characteristic signs and symptoms defining specific disorders. Classification criteria developed by the American College of Rheumatology have provided guidelines for diagnosis in…
