Reavaliação da abordagem clinica e laboratorial da esclerose sistemica : analise prospectiva de 56 pacientes

AUTOR(ES)
DATA DE PUBLICAÇÃO

1993

RESUMO

The author performed a prospective analysis of 56 patients with systemic sclerosis (SSc), attended at the Rheumatology Clinic at the State University of Campinas, according to a previous protocolo The patients were subdivided in limited (lSSc) and diffuse (dSSc) forms, according LeRoy and colleagues (1988) and analysed regarding demographic variables, clinic and laboratory manifestations. The clinic evaluations included the total skin score (TSS), standardized skin biopsy, hands and wrists radiography, roentgenographic examination of esophagus, stomach, small and large intestine, chest x-ray, pulmonary function test, diffusing capacity of carbon monoxide, conventional electrocardiogram, Doppler ecocardiography (evaluating pulmonary hypertension), creatine clearance and renal cintilography (tecnecium 99-DTPA). Laboratory evaluation included the search of antinuclear antibody (ANA, using indirect immunofluorescency with HEp-2 cells), anti-Scl 70, anti-centromere antibody (ACA), anti-RNP, anti-Sm, anti-Ro and anti-La. From the demographic view point, the variables sex, race and age at onset didn t demonstrate different behaviour in the two clinic forms of SSc. Female patients showed earlier age at onset than male, independent of the clinic form, in the childbearing ages. Regarding clinic manifestations, the lSSc patients showed TSS = 20 and calcinosis, and the dSSc patients showed TSS >20, articular contractures and pigmentation disturbances. There wasn t statistical difference regarding visceral manifestations in the two forms of SSc. Independent od the clinic form, the visceral manifestations most frequently found were esophageal hipomotility (69.1%), restrictive pulmonary disease (53.57%), pulmonary hypertension (33.92%, with five cases of primary Ph in the ISSc patients), valvular disturbances at the ecocardiography (29.09%), altered renal cintilography (28.57%), pericardial effusions (16.36%, frequently asymptomatic) and cardiac rhytm disturbances (14.28%). Regarding laboratory manifestations, ANA presented different patterns in the two clinic forms. Anti-Scl 70 antibody was positive in 25% of the patients, without predominance in the dSSc, associated with TSS >20. ACA was positive in 17.85% of the patients, alI of them with lSSc, associated with TSS = 20, calcinosis and telangiectasias. The factors associated with worst prognosis in this study were the male sex and the visceral manifestations (restrictive pulmonary disease, symptomatic heart disease and renal crisis)

ASSUNTO(S)

epidemiologia - pesquisa escleroderma sistemico - epidemiologia

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