title: Patient with antibody-negative relapse of Goodpasture syndrome.
abstract: Smoking in young men may trigger anti-GBM disease manifesting with hemoptysis. We present a male adolescent in whom hemoptysis was mistaken to be a sign of airway infection for several months and who later on underwent an unusual antibody-negative relapse. The 16-year-old patient had a history of smoking and therapy-refractant hemoptysis and, later, acute macrohematuria with renal insufficiency necessitating hemodialysis (initial creatinine 4.2 mg/ dl). Chest X-ray showed diffuse lung infiltration. Renal biopsy revealed linear IgG deposits along the glomerular basement membrane (GBM) and cellular crescents in 13/16 glomeruli, simultaneously increased anti-GBM antibodies were detected. Thus, anti-GBM glomerulonephritis was diagnosed. After treatment with prednisone, oral cyclophosphamide and plasmapheresis, chest X-ray and hemoptysis improved, but renal failure persisted. Anti-GBM antibodies were negative. 4 weeks later, the patient presented again with a clinical relapse of severe hemoptysis and respiratory insufficiency after smoke exposition. Despite negative anti-GBM antibodies, he was treated similarly to a relapse and after the second course of plasmapheresis the patients' general condition improved and hemoptysis subsided. During the next 10 months the patient was stable with negative antibodies. He was under intermittent hemodialysis until laboratory measurements showed improved renal function. Now, 30 months after the acute episode, the patient is off dialysis for 17 months with stable creatinine values of 1.9 - 2.4 mg/dl, and is currently being treated with antihypertensive medicaments, calcitriol, calciumacetate, natriumhydrogencarbonate and allopurinol. The prognosis of anti-GBM glomerulonephritis depends on serum creatinine and the need of dialysis at initial presentation. In these patients, one-year survival rate is 67% and 5% for kidney function. Of note, despite the unfavorable prognosis in our patient, renal function recovered after 1 year of hemodialysis treatment. It is important to consider that in patients with anti-GBM disease antibody-negative relapses are possible.
authors.affiliation: Kinder- und Jugendklinik, Universität Erlangen-Nürnberg. kerstin.benz@kinder.imed.uni-erlangen.de
authors.forename: K
authors.initials: K
authors.lastname: Benz
authors.forename: K
authors.initials: K
authors.lastname: Amann
authors.forename: K
authors.initials: K
authors.lastname: Dittrich
authors.forename: C
authors.initials: C
authors.lastname: Hugo
authors.forename: K
authors.initials: K
authors.lastname: Schnur
authors.forename: J
authors.initials: J
authors.lastname: Dötsch
mesh_terms: D000293:Adolescent; D019867:Anti-Glomerular Basement Membrane Disease / Q000175:diagnosis* / Q000276:immunology* / Q000628:therapy; D003937:Diagnosis, Differential; D006469:Hemoptysis / Q000175:diagnosis* / Q000276:immunology* / Q000628:therapy; D006801:Humans; D008297:Male; D012008:Recurrence; D012907:Smoking / Q000009:adverse effects*