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Thoracic outlet syndromes result from compression of the neurovascular structures supplying the upper extremity. Symptoms and signs arise from intermittent or continuous pressure on elements of the brachial plexus (> 90% of cases) or the subclavian or axillary vessels (veins…
Fibromyalgia is a common syndrome, affecting 3–10% of the general population. It shares many features with myalgic encephalomyelitis/chronic fatigue syndrome, namely, an increased frequency among women aged 20–50, absence of objective findings, and absence of diagnostic laboratory test results. While…
Polyarteritis nodosa is a necrotizing arteritis of medium-sized vessels that has a predilection for involving the skin, peripheral nerves, mesenteric vessels (including renal arteries), heart, and brain but spares the lungs. Polyarteritis nodosa is rare, with a prevalence of 30…
Takayasu arteritis is a granulomatous vasculitis of the aorta and its major branches. Rare in North America but more prevalent in the Far East, it primarily affects women and typically has its onset in early adulthood. Clinical Findings Takayasu arteritis…
Polymyalgia rheumatica and giant cell arteritis probably represent a spectrum of one disease. Both affect the same population (patients > age of 50), and the incidence of the disease increases with each decade of life. Both show preference for the…
Livedo reticularis produces a mottled, purplish discoloration of the skin with reticulated cyanotic areas surrounding paler central cores (Figure 1–7ck). This distinctive “fishnet” pattern is caused by spasm or obstruction of perpendicular arterioles, combined with pooling of blood in surrounding…
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…