Objectives: The authors propose to characterize a population in the area of reference of the Hospital de São João (HSJ) in Oporto, with severe manifestations of Systemic Sclerosis (SS), and need of hospitalization in this institution. Given the lack of data referring to SS in inpatient set (and its inexistence in Portugal), this elements may obviate a parallelism with populations described in other countries, or even identify specificities of this population, that in the future, can be important to study measures directed to optimizing their care, in Portugal.
Methods: The cases were collected from a computerized database containing all discharge registers of the Rheumatology Department of HSJ from 1/1/2003 to 31/12/2008 (6 years), diagnosed of SS (ICD9-CM code of 710,1). All the 40 inpatient records, reporting to 25 patients, were submitted to medical review, and for each of them, fulfilled a clinical protocol, focusing on multiple epidemiological and clinical characteristics, relative to evolution, manifestations and therapies, reason and outcome of hospitalization, and co-morbidities.
Results: The characteristics found in this population approach, globally, to the ones described previously, in other countries. However, the prevalence and severity of the cutaneous lesions suggest a higher impact of the cutaneous attainment in this casuistic. It would be of all interest to develop studies that could properly evaluate this observation.
Discussion: It is essential to profoundly know the disease, and the individual and population particularities, so we can act adequately. This study may have put on evidence some problems that, in the future, will probably have major impact on personal life and public health care resources in Portugal. They reflect direct and indirect costs, explained by the high rate of recurrent and long hospitalizations, making use of more expensive diagnostic tests and treatments, and the risk of higher morbidity. Early intervention may be modifying of the natural course of the disease in the long term.