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A5659 - Double-Positive Goodpasture's Syndrome in a Child: Use of Rituximab
Author Block: M. E. Dumas, J. Tremblay Roy, A. Bruns, C. Girardin; Pediatrics, CHUS, Sherbrooke, QC, Canada.
Introduction : Goodpasture’s disease (GD) is rare. Co-presentation with both ANCA and anti-GBM antibodies is exceptional in children with only eight patients reported to date, five of them had an unfavorable outcome. Optimal therapy for double-positive patients has not been identified yet.
We report a case of a 15 year-old young patient with concomitant renal dysplasia who was treated with Rituximab with a subsequent drop of anti-GBM and anti MPO antibodies and no recurrent pulmonary symptoms. He presented with life threatening acute respiratory and renal failure with elevated creatinine, hematuria,hypovolemic shock and anemia. Bronchoscopy findings were consistent with severe alveolar hemorrhage ( presence of numerous hemosiderin-laden macrophages.). . A Chest CT showed patchy areas of ground-glass opacities consistent with pulmonary haemorrhage and a right upper lobe atelectasis . A renal biopsy was performed, which showed 90% cellular crescentic glomerulonephritis and positive linear GBM staining for IgG. Serology was positive for antiglomerular basement antibody (1/640),. ANCA with anti-MPO specificity were present more than 300 . Pulmonary function testing showed restrictive lung disease .The patient had undergone plasmapheresis treatments, Rituximab was initiated as induction and maintenance therapy with significant clinical response.Cyclophosphamide was continued for three months, after which it was switched to azathioprine. At six months follow-up, he is doing well on tapering doses of steroids and azathioprine. His antiglomerular basement membrane and MPO level had decreased significantly,.Pulmonary function tests and Chest tomography were normal. He continued to be dialysis dependent but denied any recurrent haemoptysis. The patient is compliant with his peritoneal dialysis regimen and is currently being reviewed for kidney transplant candidacy.Discussion: Here we reported a very rare case of pediatric patient with coexistence of MPO-ANCAs and anti-GBM antibodies. The disease course was severe.Some studies suggest the presence of both ANCA and anti-GBM is associated with a worse renal prognosis than is the presence of either antibody alone. One of the interesting features of our case is that it highlights the role of early aggressive immunosuppressive therapy along with plasmapheresis as being potentially lifesaving . For GD a review of the literature demonstrated a handful of cases in which Rituximab has been used for treatment in adults.
Conclusion: Goodpasture syndrome represents a serious medical emergency with significant morbidity and mortality. Rapid institution of plasmapheresis and aggressive immunosuppressive therapy can induc pulmonary remission in dual positive patients