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Saudi Journal of Kidney Diseases and Transplantation
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CASE REPORT Table of Contents   
Year : 2007  |  Volume : 18  |  Issue : 2  |  Page : 235-238
Goodpasture's syndrome - Four Case Reports


Nephrology hemodialysis Department, Ibn Rochd Hospital Center, Morocco

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   Abstract 

Goodpasture's syndrome (GPS) is a rare but severe immunological disease, which is characterised by rapidly progressive glomerulonephritis and intraalveolar hemorrhage (IAH) with the presence of anti-glomerular basement membrane (GBM) antibodies. We report four cases of GP's syndrome referred to the nephrology unit at Ibn Rochd UHC in Casablanca from January 1995 to December 2003. All patients had rapidly progressive glomerulonephritis (RPGN) with proteinuria and microscopic hematuria. Elevated blood pressure was noted in one case. IAH was manifested as hemoptysis in two cases, radiological signs in three cases and confirmed by broncho-alveolar lavage in all cases. Laboratory assessment revealed anemia in all cases. Renal biopsy showed extracapillary glomerulonephritis with linear deposits of IgG along the GBM. Renal failure was severe and hemodialysis was required in all cases. All patients were treated with prednisone and cyclophosphamide and none recovered renal function. Two patients died due to severe lung hemorrhage.

Keywords: Goodpasture′s, syndrome, Renal, Failure, Pulmonary Hemorrhage.

How to cite this article:
Salam N, Rezki H, Fadili W, Hachim K, Ramdani B. Goodpasture's syndrome - Four Case Reports. Saudi J Kidney Dis Transpl 2007;18:235-8

How to cite this URL:
Salam N, Rezki H, Fadili W, Hachim K, Ramdani B. Goodpasture's syndrome - Four Case Reports. Saudi J Kidney Dis Transpl [serial online] 2007 [cited 2014 Oct 22];18:235-8. Available from: http://www.sjkdt.org/text.asp?2007/18/2/235/32316

   Introduction Top


Goodpasture's syndrome (GPS) is characterized by the association of rapidly progressive glomerulonephritis (RPGN) to intraalveolar hemorrhage (IAH) and the presence of anti-glomerular basement membrane (anti-GBM) antibodies. It is a rare but severe immunological disease. The diagnosis can be confirmed by the presence of circulating anti-GBM antibodies and/or deposition of antibodies on the glomerular basement membrane that is usually revealed by immunofluorescence (IF) staining of the renal biopsy specimen.

We report four cases of GPS syndrome referred to our nephrology unit at Ibn Rochd UHC in Casablanca from January 1995 to December 2003.


   Case report 1 Top


Mrs. MH, 29-years old woman, was admitted with advanced oligo-anuric acute renal failure (ARF) and acute respiratory failure with hemoptysis to the nephrology ward in 2003. The patient was hypertensive on admission. Chest radiography detected alveolar infiltrates. IAH was confirmed by broncho-alveolar lavage (BAL) with the presence of siderophages. Laboratory investi­gations also revealed microscopic hematuria, proteinuria (1.5 g/day), hypochronic micro­cytic anemia with hemoglobin (Hgb, 70 g/L), positive anti-neutrophilic cytoplasmic anti­bodies (ANCA), and negative anti-GBM antibodies. Renal biopsy revealed extra­capillary glomerulonephritis with crescents in 100% of glomeruli, and IgG linear deposits of the GBM with IF. The patient was treated with corticosteroid intravenous (i.v.) pulse therapy and oral dosing in addition to i.v. cyclophosphamide boluses. Moreover, the patient received two sessions of plasma exchange. The severe IAH required assisted ventilation. Eventually, the patient died due to uncontrolled respiratory failure.


   Case report 2 Top


Miss FN, 21-years old woman, treated for pulmonary tuberculosis in 2000, was admitted to the nephrology ward in 2001 for advanced oligo-anuric ARF, hyper­tension, macroscopic hematuria and protei­nuria. The patient did not present with sym­ptoms or signs of pulmonary hemorrhage on admission. Laboratory investigations revealed normochromic normocytic anemia with Hgb (60 g/L). Anti-nuclear antibodies and anti­DNA were negative, pANCA was positive, but anti-GBM antibodies were not obtained. Renal biopsy revealed crescentic glomeru­lonephritis in 100% of the glomeruli, and linear IgG deposits were confirmed along the GBM by IF. IAH was suggested by the presence of siderophages with BAL. The patient received three boluses of methyl­prednisolone followed by oral prednisolone without any other immunosuppressive agents. However, the patient became dependent on chronic hemodialysis until she successfully received renal transplantation three years later.


   Case report 3 Top


Mr A.S., a 40 -year-old male, was admitted to the hospital for severe advanced ARF, microscopic hematuria and protei­nuria. The patient also complained of dyspnea with alveolar opacities on x-ray, and IHA was confirmed with BAL. Laboratory investi­gations revealed normocytic normochromic anemia with Hgb (52 g/L). cANCA was positive, anti-nuclear antibodies and anti-DNA were negative, and circulating anti-GBM antibodies were negative. Renal biopsy confir­med the diagnosis of crescentic glomeru­lonephritis in 100% of glomeruli with signs of chronicity. Eventually the patient was not treated with any immunosuppressive agents and became dependent on chronic hemodialysis.


   Case report 4 Top


Mr. B.A., a 53 -year-old man, a chronic smoker for 15 years, was admitted in 2003 for RPGN with oligo-anuric ARF associated with microscopic hematuria and proteinuria (1.29 g/day). Laboratory investigations revealed normocytic normochromic anemia with Hgb (86 g/L). Serological investigations were all negative, including anti-GBM, ANCA, anti­DNA, and antinuclear antibodies.

Renal biopsy revealed crescentic GN with linear anti-GBM antibodies by IF staining.

The patient received three boluses of methylprednisolone followed by a bolus of cyclophosphamide. During his stay in the hospital, the patient developed acute respira­tory distress syndrome with the presence of siderophages at BAL, compatible with IAH that warranted treatment with plasmapheresis. The renal biopsy was complicated with arteriovenous fistula that required nephre­ctomy. The patient eventually died because of respiratory distress syndrome and pulmonary failure.


   Discussion Top


GPS was described for the first time in 1919 by Ernest Goodpasture as the association of IAH and RPGN.[1],[2],[3],[4],[5],[6] The role of anti-GBM antibodies in its pathogenesis was elucidated in 1967 by Lerner, Glassok and Dixon.[3],[6]

GPS is a very rare syndrome with an incidence of 1 case/million/year, and it is predominant in young men. Genetic factors that predispose to GPS include the histo­compatibility groups HLA DR 15 and HLA DR 4. Exposure to environmental factors such as viral infections, hydrocarbons and tobacco may precipitate the disease and worsen the pulmonary lesions. [3],[4],[6]

GPS is characterized by the presence of auto-antibodies against the non-collagenic epitope at the end of the alpha3 chain of type 4 collagen (Alpha 3 IV NCI) called Goodpasture's antigen,[1],[2],[6] and found mainly in alveolar and glomerular basement membranes.

In our series, all patients had oligo-anuric renal failure on admission to the hospital. The pulmonary injury was suspected with the presence of hemoptysis, and/or alveolar pattern infiltrates on the chest x-ray, and the diagnosis was confirmed by the presence of siderophages with BAL; hemoptysis was observed in two cases, alveolar infiltrates on chest radiography in three, and the siderophages in all. [2],[3],[6]

The detection of circulating and/or deposited anti-GBM antibodies detected by IF exam of the renal tissue confirmed the diagnosis of GPS. Moreover, the circulating antibodies are predicative of the severity of the renal and pulmonary injury, as well as the immediate and long-term prognosis. [2],[6] In our series, the anti-GBM antibodies were negative in two tested patients.

The detection of ANCA in the GPS patients was reported in 15 to 30% of the cases. They can be present at the beginning of the disease or develop during the evolution or even after the disappearance of the anti-GBM antibodies; pANCA is found in ¾ of the cases and cANCA in ¼ of the cases. [2],[3],[6] In our series, ANCA was positive in three patients; cANCA was found in two cases.

Pulmonary biopsy is rarely indicated because the renal tissue is more easily attainable with less morbidity.[3] None of our patients had pulmonary biopsy.

The management of GPS should be aggressive and carried out immediately upon suspicion. Therapy is based on the corticosteroids, immunosuppressive agents such as cyclophosphamide in addition to plasmapheresis, especially if the pulmonary injury is severe. [2],[3],[5],[6]

Renal transplantation can be performed in patients requiring chronic hemodialysis after disappearance of the circulating anti­GBM antibodies. In our series, one patient received renal transplant three years after the start of the disease with a good result.

Without proper management, the short­term renal prognosis is generally very guarded. Advanced renal failure on first presentation and/or presence of crescents affecting more than 50% of glomeruli are serious prognostic signs.[3] In our series, all patients presented with anuria, and crescents in 100% of the glomeruli.

Mortality from GPS varies from 11 to 21% of the cases.[3] It is usually determined by the extent of the pulmonary injury. In our series, the two patients who had pulmonary distress syndrome died shortly after admission to the hospital.

We conclude that GPS is a rare but serious disease. Prognostic factors include the level of renal function and the degree of renal injury at the time of presentation, besides the degree of pulmonary injury. The clinical management should be aggressive in order to have the best prognosis.

 
   References Top

1.Salama AD, Dougan T, Levy JB, et al. Good Pasture's disease in the absence of circulating anti-glomerular basement mambrane anti-bodies as detected by standard techniques. Am J Kidney Dis 2002; 39(6):1162-7.  Back to cited text no. 1    
2.Richard G. Phelips, A Neil Turner. Good Pasture's syndrome: new insights into pathogenesis and clinical picture. Journal of Nephrology, vol 9, n° 3­01996(111-117).  Back to cited text no. 2    
3.Plaisier E., Rossert J. syndrome de good pasture. Maladies et syndromes systemiques (763-776). Editions Flammarion.  Back to cited text no. 3    
4.Droz D. Hemorragies pulmonaires et glomerulo­nephrites rapidement progressives (Syndromes pneumo-renaux). Sem Hop Paris 1990;66:407-12.  Back to cited text no. 4    
5.Bolton WK. Gooopasture's syndrome. Kidney Int 1996;50:1753-66.  Back to cited text no. 5    
6.Salama AD, Levy JB, Lightstone L, Pusey CD. Goodpasture's disease. Lancet 2001;358:917-20.  Back to cited text no. 6    

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Correspondence Address:
H Rezki
Rue 3, No 3, Hay Echems, Hay Essalam, Casablanca 20200
Morocco
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PMID: 17496401

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    Abstract
    Introduction
    Case report 1
    Case report 2
    Case report 3
    Case report 4
    Discussion
    References
 

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