Head and Neck Surgery

MIXED CONNECTIVE TISSUE DISEASE

Posted by headnecksurgery on April 25, 2009

The term mixed connective tissue disease was coined in 1972 to describe a distinct entity with coexisting features of SLE, systemic sclerosis, polymyositis, and dermatomyositis. This entity is characterized by high titers of anti-U1 RNP, a ribonucleoprotein antibody. The prevalence is unknown, and no consensus diagnostic criteria have been developed. However, 80% of patients are women, and onset usually occurs between the ages of 30 and 60. Death results primarily from pulmonary fibrosis and hypertension.

Head and neck manifestations are a combination of the features seen in the other connective tissue disorders. Mucocutaneous changes include malar rash, discoid lupus, sclerodermatous skin thickening, oral mucosal ulceration, and nasal septal perforation. Sicca complex has been described. Esophageal dysfunction is present in most cases, resulting in abnormal peristalsis, heartburn, and dysphagia in 60%, 48%, and 38% of patients, respectively. As with most other connective tissue diseases, corticosteroid and immunosuppressive agents are the mainstays of treatment.

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