Goodpasture's syndrome: survival after acute renal failure.

Abstract

Acute Renal Failure mied.Ja., Goodpasture's syndrome, the combination of haemoptysis and nephritis, is being recognized with increasing frequency (Davidson et al., 1966).In its classical form it comprises a distinct clinical entity to which the term " lung purpura with nephritis " has been applied (Rusby and Wilson, 1965).This paper provides the first report of a patient with this condition who recovered after an episode of acute renal failure. CASE REPORTA 27-year-old mechanical engineer was admitted to hospital on 9 October 1965.He gave a history of persistent haemoptysis for seven months, increasing breathlessness for three months, macro- scopic haematuria for one week, and failure to pass urine for 36 hours.He was afebrile, pale, and orthopnoeic.The blood pressure was 110/70 mm.Hg and there were crepitations over the right lower chest.Investigations.-Blood urea 170 mg./100 ml.; serum Na+ 129 mEq/l.;K+ 3.4 mEq/l.;HCO3-17 mEq/l.; calcium 9.2 mg./ 100 ml.; phosphate 6.0 mg./100 ml.Liver-function tests normal.Serum proteins: total 6.1 g./100 ml. (albumin 2.6 g., globulin alpha, 0.4 g., alpha2 1.2 g., beta 0.5 g., gamma 1.4 g.).Hb 8.7 g./ 100 ml.; P.C.V. 30% ; M.C.H.C. 29%; E.S.R. 94 mm./lst hour (Westergren); W.B.C. 13,700 /cu.mm. (normal differential count); reticulocytes less than 1% ; prothrombin time 17 seconds (control 11 seconds).Blood coagulation mechanism otherwise normal.Serum iron 25 pAg./100ml., total iron binding capacity 435 pg./ 100 ml.Coombs test, antinuclear factor, L.E. cells all negative.Antistreptolysin titre less than 100 Todd units; blood cultures negative; sputum and throat culture, a few beta-haemolytic strepto- cocci (Lancefield group A).The E.C.G. was normal, but chest x-ray examination showed patchy consolidation at both lung bases.Numerous haemosiderophores were present in the sputum.Bronchoscopy and bronchography were normal, as were cystoscopy and retrograde pyelography.Histological examination of renal tissue obtained on 12 October and reported by Dr. M. K. McDonald showed a moderately severe acute proliferative glomerulonephritis, with epithelial crescent forma- tion, a few small foci of glomerular necrosis, but no generalized obliteration of glomerular structure.There was some evidence of acute tubular necrosis.Arteritis was not demonstrable.