RESUMO
Resumen La esclerosis sistèmica (esclerodermia) es la enfermedad reumática autoinmunitaria asociada más comúnmente con cirrosis biliar primaria. La relación entre la forma cutánea limitada de esclerosis sistémica y cirrosis biliar primaria se describió en el decenio de 1970. Se comunica el caso de una paciente de 65 años de edad con esclerosis sistémica cutánea limitada de larga evolución, positiva a anticuerpos contra centròmero y mitocondria. La biopsia hepática confirmó cirrosis biliar primaria en fase portal (estadio 1).
Abstract Systemic sclerosis (scleroderma) is an autoimmune rheumatic disease most commonly associated with primary biliary cirrhosis. The relationship between limited cutaneous systemic sclerosis and primary biliary cirrhosis was first described in the early 1970's decade. The case of 65 year-old woman with limited cutaneous systemic sclerosis and primary biliary cirrhosis seropositive for anticentromere and anti-mitochondrial antibodies is reported. Liver biopsy confirmed primary biliary cirrhosis in stage 1.
Assuntos
Linfadenite Histiocítica Necrosante/diagnóstico , Linfadenite Histiocítica Necrosante/patologia , Lúpus Eritematoso Sistêmico/diagnóstico , Lúpus Eritematoso Sistêmico/patologia , Adulto , Feminino , Linfadenite Histiocítica Necrosante/fisiopatologia , Humanos , Lúpus Eritematoso Sistêmico/fisiopatologiaRESUMO
OBJECTIVE: To assess the metoclopramide response in patients with early diffuse systemic sclerosis (dSSc) and the acute effects of intravenous (IV) metoclopramide on the lower esophageal sphincter (LES). METHODS: Twenty-one patients with early dSSc (mean age 41.4 +/- 9.8 yrs., mean disease duration 2.47 +/- 0.75 yrs.) were prospectively evaluated. Six patients with late dSSc (mean age 52.6 +/- 9.1 yrs., mean disease duration 9.5 +/- 2.5 yrs.) were used as control group. All underwent solid-state esophageal manometry at rest and 15 minutes later received 10 mg of metoclopramide in an intravenous single bolus. RESULTS: We found that the mean LES pressures measured by the station pull-through technique significantly increased in both early and late dSSc patients after metoclopramide administration (p < 0.05). While early dSSc patients did improve the mean residual pressures (p < 0.05), late dSSc patients did not (p > 0.05). In the esophageal body (EB), the mean contractions amplitude at 18, 13, 8, and 3 cm above the LES was < 20 mm Hg for both groups. However, peristaltic contraction velocitiy was significantly higher in early dSSc patients (< 0.05) than in that with late dSSc (p > 0.05). Our study did not show any major differences when comparing both groups. No side effects were seen. CONCLUSIONS: The results of our study show that metoclopramide may improve LES pressures in patients with early and late dSS. Metoclopramide improve the mean residual pressure in patients with early dSSc, but not in late dSSc patients. Although esophageal contractions amplitude were significantly improved, they did not achieve a pressure > 20 mm Hg. Because metoclopramide can be used orally, it may mitigate both dysphagia and heartburn.
