RESUMO
BACKGROUND: Systemic connective tissue diseases are relatively rare disorders of unknown origin, possibly related to autoimmunity. In the retrospective study, we analysed the records of nine patients suffering from connective tissue disorders who were treated during a two-year period in an intensive therapy unit. METHODS: Hospital medical records, observational charts and all other available medical documents were analysed. RESULTS: The clinical courses of 9 patients were studied. They included 4 with scleroderma, 3 with systemic lupus erythematosus, and 2 with inflammatory myopathy. They were admitted to the ITU because of sepsis and/or pneumonia, pulmonary haemorrhage, or cardiovascular failure. Three patients (2 with systemic sclerosis, 1 with systemic lupus erythematosus) died. CONCLUSIONS: A multidisciplinary approach to treatment is a key factor in the successful management of patients with rare diseases, such as connective tissue pathologies. Frequent infections with respiratory and/or circulatory complications are the most common causes of severe morbidity in these patients.
