Management and outcomes of childhood Goodpasture's disease

BACKGROUND: In an attempt to improve knowledge about childhood Goodpasture's disease, we performed a retrospective analysis of patients with Goodpasture's disease from several pediatric nephrology centers. METHODS: We analyzed the responses to 27 questions that elicited information about the following: incidence, demographics, patient history and clinical presentation, diagnostics performed, acute and chronic therapy, course of disease, and outcome. RESULTS: Goodpasture's disease, which is extremely rare in this age group, may manifest in 2-year-old toddlers and does not typically present with pulmonary findings before puberty Goodpasture's disease has a poor outcome with more than 50% of patients progressing to end-stage renal disease. No deaths were reported in this cohort, and renal improvement was observed in children with severe biopsy findings who required renal replacement therapy during the acute phase. CONCLUSION: The present investigation gives detailed information about childhood Goodpasture's disease under real life conditions and reveals that very few pediatric cases have been reported. Nearly 50% of children progressed to end stage renal disease. However, long term outcome in children might be better than in adults. Aggressive immuno suppressive therapy might be necessary for all affected children, even in patients who require renal replacement therapy or have severe biopsy findings.