RESUMEN

Goodpasture's syndrome is characterized by pulmonary hemorrhage, rapid progressive glomerulonephritis and the presence of anti-glomerular basement membrane (anti-GBM) antibodies. Here, we report a case of Goodpasture's syndrome that we treated with double filtration plasmapheresis (DFPP) combined with immunosuppression therapy. The patient was a 32-year-old man with the main complaints of low-grade fever, general fatigue and dyspnea. The clinical diagnosis was renal-pulmonary syndrome based on pulmonary hemorrhage on chest X-ray, rapid progressive renal insufficiency, and elevated C-reactive protein (CRP). Goodpasture's syndrome was diagnosed because the patient was negative for MPO-ANCA and PR3-ANCA, and positive for anti-GBM antibodies. Renal biopsy showed crescentic glomerulonephritis. Hemodialysis, immunosuppression therapy (methylprednisolone and cyclophosphamide) and DFPP were performed. Anti-GBM antibodies were followed pre- and post-DFPP, and removal efficiency, cost performance and complications were evaluated. The antibody levels were 121 and 84 EU/mL before and after the first DFPP procedure, respectively, giving a removal efficiency of 24%. Subsequently, the removal efficiencies were 52%, 55% and 60% after the second, third and fourth DFPP procedures. For comparison, the immunoglobulin G (IgG) removal efficiencies were 53%, 57%, 60% and 55% after the four respective DFPP procedures; therefore, the removal efficiencies were similar for anti-GBM antibodies and IgG in all except the first DFPP procedure. The serum anti-GBM antibody and IgG concentrations decreased from pre- to post-DFPP, indicating that DFPP may be an effective therapeutic approach in Goodpasture's syndrome.

Asunto(s)

Enfermedad por Anticuerpos Antimembrana Basal Glomerular/terapia , Autoanticuerpos/inmunología , Plasmaféresis/métodos , Adulto , Enfermedad por Anticuerpos Antimembrana Basal Glomerular/diagnóstico , Enfermedad por Anticuerpos Antimembrana Basal Glomerular/inmunología , Ciclofosfamida/uso terapéutico , Humanos , Inmunoglobulina G/inmunología , Inmunosupresores/uso terapéutico , Masculino , Metilprednisolona/uso terapéutico

RESUMEN

Effects of anti-oketsu drug, Keishibukuryogan (Gui-zhi-fu-ling-wan) and Tokishakuyakusan (Dang-gui-shao-yao-san) in vivo and in vitro on platelet aggregation were investigated in normal volunteers.<br>Of 20 volunteers who were given Keishibukuryogan, there were 6, 3 and 11 subjects whose dose-response curves of collagen-induced aggregations were shifted to the right, to the left, or who had no shift, respectively. The control aggregations of these 20 people were in the same range. In ADP-induced aggregation, there were 5 curves shifted to the right. Their potencies in the control aggregation were higher than those of 9 subjects who were not affected by the drug. There were 6 curves shifted to the left, and their potencies were lower than those of the 9 unaffected subjects. Of 12 volunteers who were given Tokishakuyakusan, there were 2, 2 and 8 subjects whose dose-response curves in collagen-induced aggregation were shifted to the right, the left, or who had no shift respectively. With ADP-induced aggregation, there were 1, 1 and 10 subjects whose doseresponse curves were shifted likewise. In vitro, Keishibukuryogan caused inhibition of ADP-induced aggregation but not that of collagen-induced aggregation.