Behçet disease (BD) is classified as a primary variable vessel vasculitis , emphasizing the involvement of any size and type (arterial, venous) of blood vessel. BD is also recognized as an autoinflammatory disease. Originally described with recurrent oral ulcerations, uveitis,…
Juvenile scleroderma encompasses a range of conditions unified by the presence of fibrosis of the skin. Juvenile scleroderma is divided into 2 major categories, juvenile localized scleroderma ( JLS , also known as morphea ), which is largely limited to…
Juvenile dermatomyositis (JDM) is the most common inflammatory myositis in children, distinguished by proximal muscle weakness and a characteristic rash. Inflammatory cell infiltrates result in vascular inflammation, the underlying pathology in this disorder. Etiology Evidence suggests that the etiology of…
Systemic lupus erythematosus (SLE) is a chronic autoimmune disease characterized by multisystem inflammation and the presence of circulating autoantibodies directed against self-antigens. SLE occurs in both children and adults, disproportionately affecting females of reproductive age. Although nearly every organ may…
In addition to causing arthritis by means of direct microbial infection (i.e., septic arthritis; see Chapter 705 ), microbes activate innate and adaptive immune responses, which can lead to the generation and deposition of immune complexes as well as antibody…
The diseases collectively referred to as spondyloarthritides include ankylosing spondylitis (AS) , arthritis associated with inflammatory bowel disease (IBD) or psoriasis, and reactive arthritis following gastrointestinal (GI) or genitourinary (GU) infections ( Table 181.1 and Table 181.2 ). Spondyloarthritis is…
Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease in children and one of the more common chronic illnesses of childhood. JIA represents a heterogeneous group of disorders sharing the clinical manifestation of arthritis. The etiology and pathogenesis of…
Nonpharmacologic as well as pharmacologic interventions are often necessary to meet the desired goals of disease management. Optimal disease management requires family-centered care delivered by a multidisciplinary team of healthcare professionals providing medical, psychological, social, and school support. Rheumatologic conditions…
Rheumatic diseases are defined by the constellation of results of the physical examination, autoimmune marker and other serologic tests, tissue pathology, and imaging. Defined diagnostic criteria exist for most rheumatic diseases. Recognition of clinical patterns remains essential for diagnosis because…
Adverse drug reactions can be divided into predictable (type A) and unpredictable (type B) reactions. Predictable drug reactions , including drug toxicity, drug interactions, and adverse effects, are dose dependent, can be related to known pharmacologic actions of the drug,…