[Goodpasture syndrome: treatment initiation with plasmapheresis before histologic diagnostic verification].
A 28 years old male patient presented, after a history of previous recurrent hemoptysis, with diffuse bilateral air space consolidation at chest radiography (CXR). Within 48 hours, partial respiratory insufficiency developed and required intubation. On a clinical and roentgenographic basis, the diagnosis of a Goodpasture syndrome was suspected. Plasmapheresis and immunosuppressive therapy with prednisone and cyclophosphamide were started immediately. Three days after admission, macrohematuria developed and serum creatinine began to rise to a maximum of 3.9 mg/dl. Totally, 13 plasmaphereses were performed within 27 days. Clinical, laboratory and radiological findings improved markedly. 30 days after admission, the patient was discharged and followed on an outpatient basis. Serum creatinine eventually decreased to 1.1 mg/dl. Initially, circulating antibodies against glomerular basement membrane (GBM) were positive, controls remained negative. Renal biopsy was performed after the acute phase and showed glomerulonephritis and linear immunoglobulin deposition along the GBM. Radiologic findings at CXR and high resolution computed tomography are demonstrated.
Gartenschläger
M
M
Klinikum der Johannes Gutenberg Universität Mainz.
Lenci
G
G
Schinzel
H
H
Schweden
F
F
Dienes
H P
HP
Weilemann
L S
LS
D000328:Adult; D019867:Anti-Glomerular Basement Membrane Disease / Q000175:diagnosis / Q000473:pathology / Q000628:therapy*; D001706:Biopsy; D001999:Bronchoscopy; D003422:Critical Care; D006801:Humans; D007668:Kidney / Q000473:pathology; D008168:Lung / Q000473:pathology; D008297:Male; D010956:Plasmapheresis; D012131:Respiratory Insufficiency / Q000175:diagnosis / Q000473:pathology / Q000628:therapy*
Goodpasture-Syndrom (GPS): Behandlungsbeginn mit Plasmapherese vor histologischer Diagnosesicherung.