Treatment of goodpasture's syndrome with plasmapheresis

Rosenblatt, Steven G.; Knight, W. David; Bannayan, George A.; Wilson, Curtis B.; Stein, Janice Gross

doi:10.1016/0002-9343(79)91186-0

Cited by 61 publications

(15 citation statements)

References 31 publications

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“…The case described here, in agreement with another report [4], suggests that treatment with plasma exchange and im munosuppressive drugs is beneficial even in the presence of severe renal failure requiring haemodialysis, and ex tensive crescent formation. Early diagnosis and treat ment are mandatory for these patients.…”

Section: Sirsupporting

confidence: 91%

Good Therapeutical Response of Goodpasture’s Syndrome with Severe Renal Failure

Salvidio¹,

Garibotto²,

Saffioti³

et al. 1989

Nephron

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Sir,The clinical course of anti-glomerularbasement mem brane (GBM) antibody-induced Goodpasture's syn drome is typically characterized by rapidly progressive glomerulonephritis, leading to end-stage renal failure, and by lung haemorrhages [1], It is generally accepted that patients with anti-GBM nephritis whose serum creati nine exceeds 6.5 mg/dl or with severe crescent involve ment, oliguria and advanced renal failure requiring haemodialytic treatment, do not respond to the appropriate treatment [2,3].We describe the case of a patient presenting with advanced renal failure whose conditions dramatically improved after plasma exchange and immunosuppres sive therapy.A 21-year-old woman was referred to our division for the devel opment of rapidly progressive renal failure, with serum creatinine rising from 1.2 to 7.7 mg/dl in 12 days. Three months before admis sion, she had experienced recurrent episodes of haemoptysis fol lowed by severe anaemia. She had a history of heavy smoking. On admission, physical examination revealed pallor and a systolic murmur, blood pressure was normal. Oliguria was not present. Laboratory data showed: haemoglobin 6.2 g/dl, haematocrit 18.8%, WBC 4.200/mmJ, ESR 85 mm at the 1st hour, BUN 62 m g/dl, serum creatinine 7.7 mg/dl. Urine analysis showed microscopic haematuria, 24-hour protein excretion was 2.5 g. Protein electrophoresis, serum complement, cryoglobulins and antinuclear antibodies were normal. A chest X-ray was also normal. A renal biopsy immediately performed showed 20 glomeruli on light microscopy; all of them presented circumferential cellular crescents. Scarring and tubular alterations were not present. At immunofluorescence, heavy linear staining for IgG and C3 along glomerular basement membranes was present. An indirect immunofluorescence performed on normal renal tissue was positive for anti-G BM antibodies with a titer of 1:32.The day following admission, serum creatinine rose to 8.5 mg/ dl, and treatment was started with 3-liter plasma exchange every other day, pulse méthylprednisolone 20 m g/kg/day for 3 days, then reduced to I m g/kg/day of oral prednisone, and cyclophosphamide 1.5 mg/kg/day. Three days later, serum creatinine was 9.6 mg/dl, and haemodialysis was started. After 3 weeks of treatment, serum creatinine decreased progressively to 4.2 m g/dl, and haemodialysis could be stopped. At the same time, circulating anti-GBM antibod ies were no longer detectable so that plasma exchange was inter rupted after a total of 10 sessions. The patient was dismissed after 8 weeks of hospitalization: serum creatinine was 3 mg/dl, creatinine clearance 22 ml/min and circulating anti-GBM antibodies were absent. Cyclophosphamide treatment was interrupted, and corti costeroid therapy was tapered to a maintenance dosage of 8 m g/day of prednisolone. After 12 months from the first manifestation of the disease, the patient maintains a stable renal function with absence of circulating anti-GBM antibodies. No other episodes of pulmonary haemorrhages have occurred.

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Section: Sirsupporting

confidence: 91%

Good Therapeutical Response of Goodpasture’s Syndrome with Severe Renal Failure

Salvidio¹,

Garibotto²,

Saffioti³

et al. 1989

Nephron

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“…This is unsatisfactory as it is obviously important to have a concurrent group of patients treated conservatively. In this present study, although the groups are small, the favorable outcomes in the untreated group and the group treated with immunosuppression alone make the excellent results recorded elsewhere with plasma exchange of less impact [2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20],…”

Section: Discussionmentioning

confidence: 99%

“…However, 5 of these 11 patients had serum creatinine levels of >; 0.3 mmol/1 at the time of presentation. With the addition of the 8 pa tients described in this paper who have had plasma exchange and immunosuppression, there are now 74 pa tients adequately described in the literature [2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20]. 37 (50%) have been reported as having stable or im proved renal function.…”

Section: Discussionmentioning

confidence: 99%

“…it seems log ical to attempt to reduce the severity of renal and lung disease by measures to hasten the disappearance of the antibody. An improved clinical course and more rapid fall in anti-GBM antibody with plasma exchange, cyto toxic agents and corticosteroids was originally described by Lockwood and co-workers [2][3][4][5], and subsequently by other groups [6][7][8][9][10][11][12][13][14][15][16][17][18][19][20], Earlier reports of the natural history of the disease emphasized the poor prognosis in Goodpasture's syndrome [1], but with the routine use of immu nopathologie studies (tissue and scrum) milder forms of this disease are now recognized. In addition, the im proved management of renal failure, dialysis and the use of corticosteroids for pulmonary hemorrhage, have also improved the outlook for patients with this disease.…”

Section: Introductionmentioning

confidence: 99%

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Plasma Exchange in Goodpasture’s Syndrome

et al. 1982

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The clinical course and levels of anti-glomerular basement membrane (GBM) antibody were compared in 20 patients with Goodpasture’s syndrome treated with plasma exchange and immunosuppression (8 patients), immunosuppression alone (4 patients) or no specific therapy (8 patients). There was a more rapid fall in the level of anti-GBM antibody and pulmonary hemorrhage was less protracted in the 8 patients treated with plasma exchange and immunosuppression. In this group, 1 patient who presented with severe renal failure showed a marked improvement of renal function and there was no progression of disease in the 4 with milder renal involvement. 2 of the 4 patients treated with immunosuppression alone, and only 2 of the 8 patients who received no specific therapy, maintained normal renal function. In the group which received no specific therapy, 1 of the 6 patients who progressed to renal failure had mild renal involvement initially. There was a significant correlation between the level of anti-GBM antibody and the severity of the morphological changes seen at renal biopsy but not between the level of anti-GBM antibody and the severity of lung hemorrhage. The course and outcome of the disease in those patients not treated, or treated with immunosuppression alone, was better than that described in early reports of this disease, while those patients with plasma exchange and immunosuppression fared even better. An adequately stratified controlled trial of immunosuppression and plasma exchange versus immunosuppression alone is in order.

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“…Uncontrolled studies have shown very prom ising results in both Goodpasture's syndrome and idio pathic anti-GBM RPGN [126][127][128][129][130][131][132][133]. Anti-GBM antibody levels have been lowered by intensive plasmapheresis in most patients, but not all [133,135,136], Stabilization or improvement in renal function has been reported in over 50% of treated patients [32,38,[131][132][133], These results are clearly better than the 10-15% survival previously re ported in this disease [137] [128,131,134], It also reverses impaired reticuloendothelial system function in some patients which may further reduce circulating im mune complex levels [132,133,142], Not surprisingly, it is also associated with significant morbidity, usually bacte rial or opportunistic infections and pulmonary hemor rhage [132,133,143], This complication rate, as well as the very high cost ($ 750-1,000/4-liter exchange treatment in the US), make it unlikely that plasma exchange will ulti mately emerge as the treatment of choice for non-anti-GBM antibody-mediated forms of RPGN. However, more controlled data is required to answer this ques tion.…”

Section: Treatmentmentioning

confidence: 99%

Idiopathic Rapidly Progressive Glomerulonephritis

Couser

1982

Am J Nephrol

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Treatment of goodpasture's syndrome with plasmapheresis

Cited by 61 publications

References 31 publications

Good Therapeutical Response of Goodpasture’s Syndrome with Severe Renal Failure

Good Therapeutical Response of Goodpasture’s Syndrome with Severe Renal Failure

Plasma Exchange in Goodpasture’s Syndrome

Idiopathic Rapidly Progressive Glomerulonephritis

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Treatment of goodpasture's syndrome with plasmapheresis

Cited by 61 publications

References 31 publications

Good Therapeutical Response of Goodpasture’s Syndrome with Severe Renal Failure

Good Therapeutical Response of Goodpasture’s Syndrome with Severe Renal Failure

Plasma Exchange in Goodpasture&rsquo;s Syndrome

Idiopathic Rapidly Progressive Glomerulonephritis

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Plasma Exchange in Goodpasture’s Syndrome