Objectives To investigate the clinical course and outcome of patients diagnosed with mixed connective tissue disease (MCTD).
Methods Since 1981, forty-four patients with MCTD have been diagnosed and followed up until December 2000. We reevaluated all these patients by entering the demographic, clinical, laboratory, radiological and therapeutic disease characteristics in a database.
Results Thirty-nine patients had the typical feature of MCTD, 3 developed diffuse systemic sclerosis and 2 systemic lupus erythematosus. Thirty-six were female and 3 male. The mean age was 51.6 (11), the disease duration was 12.9 (5.2) (range 1-25 years) and the mean follow up was 6.6 (4.9) (range 1-19 years). Almost all patients (95%) presented symptoms and signs of Raynaud’s phenomenon, arthritis and arthralgias evolving the small joints of the hands. Pulmonary dysfunction was observed in 44%, while pulmonary hypertension and renal involvement in 5% of patients. All patients had positive U1Sn-RNP antibodies and high titers of antinuclear antibodies. Only 10% of our patients also had Ro (SSA) antibodies.
Conclusion It seems that our patients presented the typical clinical and serological features of MCTD. The majority of our patients had favorable outcomes, with pulmonary dysfunction being the most frequent, while pulmonary hypertension and renal involvement was observed in only a few cases.
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