Etiology, Prevalence, and Epidemiology Takayasu arteritis, also known as pulseless disease or Martorell syndrome, is an idiopathic chronic granulomatous arteritis affecting medium and large arteries with a predilection for the aorta and its main branches. It is seen most commonly…
Etiology, Prevalence, and Epidemiology Behçet disease is an uncommon systemic disorder of unknown etiology characterized by vasculitis and the triad of recurrent ulcers of the oral and genital mucosa with relapsing uveitis. Vascular complications develop in 20% to 40% of…
Etiology, Prevalence, and Epidemiology Goodpasture syndrome, also known as anti–basement membrane antibody disease, is an autoimmune disorder characterized by repeated episodes of pulmonary hemorrhage, usually associated with glomerulonephritis and the presence of anti–glomerular basement membrane (anti-GBM) antibodies. Goodpasture syndrome is…
Etiology, Prevalence, and Epidemiology Pulmonary vasculitis is traditionally characterized by size of vessel involvement as defined by the Chapel Hill nomenclature. Within the small-vessel vasculitides, antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis represents a diverse group of entities, and although the inciting…
Interstitial pneumonia with autoimmune features (IPAF) refers to the clinical entity of interstitial lung disease (ILD) in patients with features of autoimmunity but without overt connective tissue disease (CTD). IPAF was introduced in 2015 by an international consensus panel in…
Etiology Mixed connective tissue disease (MCTD) is a disease with certain features of polymyositis, scleroderma, and systemic lupus erythematosus. Much of the evidence that MCTD is a distinct clinical entity stems from the identification of antiribonucleoprotein (anti-RNP) antibody, disease-specific human…
Etiology Sjögren's syndrome, or “sicca syndrome,” is a disorder of the immune system that is largely defined by its two most common symptoms—dry eyes and a dry mouth; these symptoms frequently accompany other autoimmune disorders. Distinction is usually made between…
Etiology Immune-mediated muscle inflammation and vascular damage are the hallmarks of polymyositis (PM) and dermatomyositis (DM). In PM the immune system is primed to act against muscle antigens, whereas in DM there is complement-mediated damage to both endomysial vessels and…
Etiology The pathogenesis of systemic lupus erythematosus (SLE) involves genetic and environmental factors, hormonal influences, and cell-mediated responses. In SLE, B lymphocytes lose self-tolerance and inappropriately produce autoantibodies. Serologic positivity for antinuclear antibodies (ANAs) is found in nearly all patients,…
Etiology Systemic sclerosis (scleroderma) has three cardinal features: excessive collagen production, vascular damage, and inflammation The pathogenesis of systemic sclerosis is obscure, but there are several contributing factors, including genetic, environmental, and autoimmune influences. Familial aggregation for systemic sclerosis, although…