Registry of the Spanish Network for Systemic Sclerosis: Survival, Prognostic Factors, and Causes of Death.

Systemic sclerosis (SSc) is a rare, multisystem disease showing a large individual variability in disease progression and prognosis. In the present study, we assess survival, causes of death, and risk factors of mortality in a large series of Spanish SSc patients. Consecutive SSc patients fulfilling criteria of the classification by LeRoy were recruited in the survey. Kaplan-Meier and Cox proportional-hazards models were used to analyze survival and to identify predictors of mortality. Among 879 consecutive patients, 138 (15.7%) deaths were registered. Seventy-six out of 138 (55%) deceased patients were due to causes attributed to SSc, and pulmonary hypertension (PH) was the leading cause in 23 (16.6%) patients. Survival rates were 96%, 93%, 83%, and 73% at 5, 10, 20, and 30 years after the first symptom, respectively. Survival rates for diffuse cutaneous SSc (dcSSc) and limited cutaneous SSc were 91%, 86%, 64%, and 39%; and 97%, 95%, 85%, and 81% at 5, 10, 20, and 30 years, respectively (log-rank: 67.63, P < 0.0001). The dcSSc subset, male sex, age at disease onset older than 65 years, digital ulcers, interstitial lung disease (ILD), PH, heart involvement, scleroderma renal crisis (SRC), presence of antitopoisomerase I and absence of anticentromere antibodies, and active capillaroscopic pattern showed reduced survival rate. In a multivariate analysis, older age at disease onset, dcSSc, ILD, PH, and SRC were independent risk factors for mortality. In the present study involving a large cohort of SSc patients, a high prevalence of disease-related causes of death was demonstrated. Older age at disease onset, dcSSc, ILD, PH, and SRC were identified as independent prognostic factors.

[Program for the substitution of therapeutic equivalents in the hospital setting]. / Programa para la sustitución de equivalentes terapéuticos en el medio hospitalario.

BACKGROUND: A Program of Therapeutic Equivalents (TEP) is here reported which was elaborated and is currently in force at a third level university teaching hospital. MATERIALS AND METHODS: Therapeutic equivalents were selected within the same pharmacologic group on the basis of approved indications and both efficiency and safety data. RESULTS: TEP considers: a) the substitution of drugs which are considered therapeutic equivalents; b) withdrawal of drugs which have not proved efficiency or are of no interest for inpatients; c) continuation of therapies when changes are not advisable, and d) indistinct use of homologous drugs. From August 1998 up to April 1999, TEP was applied in 505 occasions; it was accepted in 499 (99%) and rejected in 6 (1%). DISCUSSIONS: The substitution of therapeutic equivalents should be viewed in the context of selecting the most appropriate drugs to be used in the hospital setting. TEP should be a consensus document and supervised by the Pharmacy and Therapeutics Commission.