Pathological Case of the Month | Nephrology | JAMA Pediatrics | JAMA Network
[Skip to Navigation]
Sign In
Citations 0
Special Feature
December 2001

Pathological Case of the Month

Arch Pediatr Adolesc Med. 2001;155(12):1384. doi:10.1001/archpedi.155.12.1383

Diagnosis and Discussion: Goodpasture Disease

Figure 1. Marked crescent formation of polymorphonuclear leukocytes associated with generalized edema and fibrotic expansions (hematoxylin-eosin, original magnification × 20).

Figure 2. Inflammatory infiltration of polymorphonclear leukocytes (hematoxylin-eosin, original magnification × 40).

The association of rapidly progressive glomerulonephritis and pulmonary hemorrhage is classically known as Goodpasture syndrome (GPS). In the presence of autoantibodies directed against glomerular and alveolar basement membranes, it is called Goodpasture disease (GPD) or anti-GBM disease. Goodpasture disease accounts for 20% to 40% of cases of GPS. The rest are caused by systemic vasculitis: most commonly by Wegener granulomatosis, microscopic polyarteritis, and systemic lupus erythematosus and less commonly by Churg-Strauss syndrome, Henoch-Schönlein purpura, Behçet disease, essential mixed cryoglobulinemia, rheumatoid vasculitis, and drugs such as penicillamine and hydralazine hydrochloride.1

Whereas some patients may manifest glomerulonephritis and pulmonary hemorrhage in isolation, others may have either severe pulmonary and renal involvement or proteinuria and hematuria along with normal renal and pulmonary functions.1-3 The presence of pulmonary hemorrhage is closely related to that of pulmonary irritants, especially cigarette smoking as in our patient.2,4-6 Pulmonary hemorrhage is extremely rare in nonsmokers.1

The most common laboratory abnormality in GPD is a positive result for anti-GBM antibodies in 90% of patients. The kidney biopsy finding of crescent formation may involve 80% to 100% of glomeruli. Diffuse linear immunoglobulin G deposition is seen along the GBM on an immunofluorescent examination.1,2,4 The biopsy findings and the presence of anti-GBM antibodies in our patient were consistent with GPD.

Rapidly progressive glomerulonephritis and such factors as systemic lupus erythematosus, polyarteritis nodosa, Henoch-Schönlein purpura, hypersensitivity angiitis, mixed connective tissue disease, Wegener granulomatosis, and a drug reaction that may cause pulmonary-renal syndrome should be considered in the differential diagnosis.1 The clinical and laboratory findings in our patient were inconsistent with these disorders.

Most patients with GPS progress rapidly to end-stage renal disease. Increased creatinine levels, oligoanuria, and the severity of renal lesions indicate a poor prognosis.4

New therapeutic approaches such as plasmapheresis and treatment with cyclosporine, cyclophosphamide, and prednisolone may be effective in 80% of patients.1,7 However, patients with end-stage renal failure and those who require dialysis rarely improve.1,2

Accepted for publication May 23, 2000.

Corresponding author and reprints: Ahmet Özel, MD, Melikşah Mh, Müstehak S, Akyurt Sitesi, C Blok, No. 4/10, Konya, Turkey.

Turner  ANRees  AJ Antiglomerular basement membrane disease. Davison  AMCameron  JSGrünfeld  JPKerr  DNSRitz  EWinearls  CGeds. Oxford Textbook of Nephrology. Oxford, England Oxford University Press1998;647- 666Google Scholar
Glassock  RJ Goodpasture's disease. Massry  SGGlassock  RJeds. Textbook of Nephrology. Baltimore, Md Williams and Wilkins1995;818- 823Google Scholar
Knoll  GRabin  EBums  BF Antiglomerular basement membrane antibody-mediated nephritis with normal pulmonary and renal function.  Am J Nephrol. 1993;13494- 496Google ScholarCrossref
Herody  MBobrie  GGouvarin  CGrünfeld  JPNoel  LH Anti-GBM disease: predictive value of clinical, histological and serological data.  Clin Nephrol. 1993;40249- 255Google Scholar
Bombassei  GJKaplan  AA The association between hydrocarbon exposure and antiglomerular basement membrane antibody-mediated disease (Goodpasture's syndrome).  Am J Int Med. 1992;21141- 153Google ScholarCrossref
Garcia-Rostan y Perez  GMGarcia  BFPuras Gil  AM Pulmonary hemorrhage and antiglomerular basement membrane antibody-mediated glomerulonephritis after exposure to smoked cocaine (crack): a case report and review of the literature.  Pathol Int. 1997;47692- 697Google ScholarCrossref
Querin  SSchürch  WBeaulieu  R Cyclosporin in Goodpasture's syndrome.  Nephron. 1992;60355- 359Google ScholarCrossref