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IgG4-related disease is a systemic disorder of unknown cause marked by highly characteristic fibroinflammation that contains IgG4 plasma cells and can infiltrate virtually any organ. The disorder chiefly affects men (75% of patients) > age of 50 years. Diagnostic Essentials…
Sjögren syndrome is a systemic autoimmune disorder whose clinical presentation is usually dominated by dryness of the eyes and mouth due to immune-mediated dysfunction of the lacrimal and salivary glands. The disorder is predominantly seen in women, with a ratio…
Many patients with symptoms and signs of a connective tissue disease have features of more than one type of rheumatic disease. Special attention has been drawn to a subset of antinuclear antibody–positive patients who have high titers of RNP autoantibodies…
Immune-mediated inflammatory myopathies include polymyositis, dermatomyositis, myositis resulting from a rheumatic disease or overlap syndrome, inclusion body myositis (IBM), and immune-mediated necrotizing myopathy. These disorders are characterized by progressive muscle weakness, and all but IBM demonstrate an inflammatory infiltrate in…
Systemic sclerosis (scleroderma) is a rare chronic disorder characterized by diffuse fibrosis of the skin and internal organs. Symptoms usually appear in the third to fifth decades, and women are affected 2- to 3-times as frequently as men. Two forms…
Raynaud phenomenon (RP) is a syndrome of paroxysmal digital ischemia, most commonly caused by an exaggerated response of digital arterioles to cold or emotional stress. The initial phase of RP, mediated by excessive vasoconstriction, consists of well-demarcated digital pallor or…
The clinical features of primary antiphospholipid syndrome (APS) are venous or arterial occlusions or certain pregnancy complications. Laboratory criteria include the identification of at least one of the following three antiphospholipid antibodies: IgG or IgM anti-cardiolipin, IgG or IgM antibodies…
Drug-induced lupus shares several clinical and serologic features with SLE but is due to ongoing exposure to a drug and resolves when the offending drug is discontinued. In contrast to SLE, the sex ratio is nearly equal. Diagnostic Essentials Clinical…
SLE is an inflammatory autoimmune disorder characterized by autoantibodies to nuclear antigens. It can affect multiple organ systems. Many of its clinical manifestations are secondary to the trapping of antigen-antibody complexes in capillaries of visceral structures or to autoantibody-mediated destruction…
Still disease is a systemic form of juvenile chronic arthritis in which high spiking fevers are much more prominent, especially at the outset, than arthritis. This rare syndrome also occurs in adults. Most adults are in their 20s or 30s;…