SESSION TITLE: Diffuse Lung Disease Case Report Posters
SESSION TYPE: Case Report Poster
PRESENTED ON: Sunday, March 23, 2014 at 01:15 PM - 02:15 PM
INTRODUCTION: From 2009 to 2013, we identified four Goodpasture disease as severe alveolar hemorrhage.
CASE PRESENTATION: 2 women and 2 men average Age was 20 to 29 years (sex ratio: 2 women/2 men). Hemoptysis was constant. The average PAO2 admission was 44 mmHg. The hemoglobin averaged 6.9 mg / dl. Diffuse infiltrates with alveolar filling were described in chest CT when it was available. Two cases necessitated invasive ventilation. Anti-glomerular basement membrane antibodies were present in three cases. By cons, one case was diagnosed ventilated by renal biopsy (negative anti GBM). No renal functional impairment was present except a moderate proteinuria. Three patients underwent a renal biopsy showing the scars of extracapillary glomerulonephritis with linear deposits of IgG immunofluorescence on the capillary basement membrane. Tobacco consumption was constant, the use of cannabis in three cases and cocaine in two cases. The four patients were treated with prednisolone and cyclophosphamide. Plasmapheresis for 2 cases was initiated following renal biopsies and proteinuria. One patient, 29 weeks pregnant, received a full treatment without sequelae. One of the cases recurred within two months following a therapeutic noncompliance.
DISCUSSION: Note the extreme severity of the potentially lethal respiratory distress at admission. The diagnosis must be given rapidly. The dosage of anti-GBM is critical but renal biopsy must be necessary. Toxic factors are usually presents. Prognosis is favorable with treatment in these cases.
CONCLUSIONS: The incidence of Goodpasture's syndrome is rare (0.5 to 1 case / million )the, to find 4 cases in 4 years demonstrate the use of good algorithms in our medical departments.
Reference #1: Réanimation 14 (2005) 614-620. Acute respiratoriys failure from alveolar Haemorrhage. A.Parrot, C.Picard, M. Fartoukh, F.Vincent, C. Mayaud
Reference #2: N ENGL J MED 348;25 June 2003. Alport's Syndrome, Goopasture'Syndrome, and Type IV Collagen. B.G.Hudson and coll.
Reference #3: Rev Med Brux 200; 27: 162-6. Goodpasture disease. G.Fomegné and coll.
DISCLOSURE: The following authors have nothing to disclose: Olivier Gilbert, Alain Van Meerhaeghe, Benoit Guillaume, Thibault Richard, Maxime Vancutsem, Philippe Pierard
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