A 15-year-old boy was admitted for vomiting, diarrhea, fatigue, crampy
abdominal pain and oliguria. A renal failure was diagnosed (creatinine
2523 μmol/, urea 53,1 mmol/l) with severe aregenerative anemia
(80 g/l), metabolic acidosis, hyperkalemia, elevated inflammatory
markers and normal platelet count. A nephrotic proteinuria was noticed
(350 g/mol). Patient's creatinine was normal 4 months before. The
diagnosis of rapidly progressive glomerulonephritis was suspected. C3
and C4 were normal, ANA and ANCA were negative; anti-glomerular
basement membrane antibody (anti-GBM) was positive (1/320) which
lead to the diagnosis of Goodpasture's disease. Chest X-ray showed
bilateral hilar infiltration and CT-scan revealed multiple alveolar
haemorrhages, confirmed by broncho-alveolar lavage. Renal
ultrasound showed swollen and hyperechogenous kidneys with loss of
corticomedullary differentiation. Renal biopsy revealed a global
extracapillary necrotising glomerulonephritis, with IgG lining the
membrane at immunofluorescence. The patient was treated with
continuous venovenous hemodia- filtration, plasmapheresis and
immunosuppressive therapy (cyclophosphamid and corticoids) which
lead to normalisation of anti-GBM level and favourable respiratory
evolution with no sequelae. The renal evolution was unfavourable and
the patient developed end stage renal disease and was treated with
haemodialysis. Goodpasture's disease is an autoimmune process in
which anti-GBM are produced against collagen IV present in the
kidneys and pulmonary alveolae, resulting in acute or rapidly
progressive glomerulonephritis and altering the pulmonary alveolae. It
is a rare disease concerning mostly infants and young adults. Clinical
presentation consists in an acute renal failure with proteinuria.
Pulmonary symptoms (60-70% of the total cases) are dyspnea, cough,
and haemoptysis. Diagnosis is made with the dosage of immunological
anti-GBM and with renal biopsy. Factors of poor prognosis are initial
oliguria, alteration of >50% of the glomerulus, very high creatinine or
need of dialysis. Anti-GBM dosage is used for follow up. Patients are
treated with immunosuppressive therapy for 6 to 9 months and
plasmapheresis. Few recurrences are seen. Goodpasture's disease
should be evoqued whenever a young patient is seen with
glomerulonephritis, especially if pulmonary abnormalities are present.
The disease requires an aggressive treatment in order to prevent
respiratory and kidney failure.