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Treatment of anti-GBM antibody (Goodpasture's) disease

INTRODUCTION

Anti-GBM antibody disease is one of the three major forms of rapidly progressive (or crescentic) glomerulonephritis. Although some patients present with relatively mild renal insufficiency, this disorder is typically associated with severe renal injury that, if untreated, progresses quickly to end-stage renal failure.

An important determinant of the response to therapy and long-term prognosis is early diagnosis [1,2]. There is a direct correlation between the initial plasma creatinine concentration and the percent of glomeruli with crescents; in particular, crescents are present in more than 75 percent of glomeruli when the plasma creatinine concentration is above 5 mg/dL (442 micromol/L). Avoidance of maintenance dialysis is rare in patients who require dialysis within 72 hours of presentation, particularly in those who have crescents involving all glomeruli [3]. In comparison, prevention of end-stage renal disease can usually be achieved in less severe cases, although some do progress. The proportion of preserved glomeruli may be the best determinant of prognosis (see 'Selection of patients to be treated' below).

The treatment of anti-GBM antibody disease is discussed in this review. The pathogenesis, prognosis and clinical manifestations of this disorder are discussed elsewhere. (See "Pathogenesis and diagnosis of anti-GBM antibody (Goodpasture's) disease".)

TREATMENT

The treatment of choice in anti-GBM antibody disease is plasmapheresis combined with prednisone and cyclophosphamide [1,3-8]. Plasmapheresis removes circulating anti-GBM antibodies and other mediators of inflammation (such as complement), while the immunosuppressive agents minimize new antibody formation.

Initial therapy — Most of the reported studies have been uncontrolled. Reviews of available reports suggest that approximately 40 to 45 percent of patients will benefit by not progressing to end-stage renal disease or death, when treated with plasmapheresis in combination with immunosuppression [4,5,8]. However, recovery is much more likely in patients who begin treatment before oliguria ensues, and is rare in patients who require dialysis or who have 100 percent crescents on biopsy.

             

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Literature review current through: Jul 2014. | This topic last updated: Sep 24, 2013.
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