Plasma exchange in Goodpasture syndrome associated with Turner’s syndrome: A case report

LP Jiao, JF Fan, Q Sun, Y Shen

Abstract

Background: Good pasture syndrome (GPS) has been paid much attention recently for the dangerous illnessand high mortality.

Objective: To investigate the efficiency of plasma exchange (PE) to treat Goodpasture syndrome (GPS) in children associated with Turner’s syndrome.

Method: We report a case of a 15 year old female with GPS and Turner’s syndrome. The patient has intermittent fever and cough for 45 days and oliguria for 6 days. Turner’s syndrome was determined through blood karyotype analysis, and GPS was diagnosed because the patient was negative for antinuclear antibodies and antineutrophil cytoplasmic antibodies (ANCA), but positive for anti-glomerular basement membrane (anti-GBM) antibodies (200 RU/ml). PE was carried out in combination with immunosuppression therapy.

Results: The results show PE treatment can efficiently decrease the levels of anti-GBM antibodies. The antibody levels were >200 RU/ml and 184 RU/ml before and after the first PE treatment, respectively. The removal efficiency were 40%, 47%, 42%, 54%, 52% for the fifth, sixth, seventh, eighth and ninth PE procedures, respectively.

Conclusion: The therapy with PE, hemodialysis, pulse methylprednisolone followed by oral prednisone and cyclophosphamide greatly contributed to improvement of this patient’s condition, and resolved the patient’s pulmonary haemorrhage. All these results demonstrate that PE contributed efficiently to the treatment for GPS in children.

Keywords: Goodpasture syndrome (GPS), Turner’s syndrome, plasma exchange (PE).