This systemic connective tissue disorder is three times more common in women than in men. It classically involves multiple joints symmetrically, and extremes such as atlantoaxial joint instability with erosion of the odontoid can lead to cord compression. These patients usually present for joint replacement surgery, release of contractures, and repair of ruptured tendons. Extra-articular disease manifestations are common, and include fever, rash, lymphadenopathy, and anemia. Cardiac involvement occurs in a third of patients and presents as pericarditis with pericardial effusion; tamponade may occur. Pulmonary involvement includes fibrosis, which may be diffuse or localized into nodules, and is usually associated with pleural effusions. Renal disease is common and may be due to the disease itself or to drug therapy. Long-standing disease may result in amyloid deposition.
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