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Acute renal failure as the initial manifestation of systemic lupus erythematosus in children
Acute renal failure as the initial manifestation of systemic lupus erythematosus in children In two patients with systemic lupus erythematosus, acute renal failure was the initial manifestation. The diagnosis was eventually established on the basis o f serologic tests and characteristic renal histopathologie findings. I'Ve emphasize the need to consider systemic lupus erythematosus as a cause o f acute renal failure o f glomerular origin, because appropriate therapy may alter the outcome o f the disease. (J PEDIATR 105:38, 1984)
Kishore Phadke, M.D., Howard Trachtman, M.D., Anthony Nicastri, M.D., C. K. Chen, M.D., and Amir Tejani, M.D. Brooklyn, N.Y.
FOURTEEN CLINICAL CRITERIA have been established by the American Rheumatology Association to aid in the identification of patients with systemic lupus erythematosus~; the presence of four or more criteria in an individual patient is considered necessary for the diagnosis. Since then (1971), there have been numerous reports of patients with biopsy-proved lupus nephritis who lacked the requisite number of diagnostic criteria at the time of initial clinical presentation2-9; nearly all of these patients had moderate proteinuria or the nephrotic syndrome. There have been no reports of patients with S L E with acute renal failure in the absence of extrarenal manifestations of the disease. We describe two pediatric patients with S L E in whom the sole presenting manifestation was acute renal failure. Although they had no extrarenal manifestations of S L E at the time of their initial evaluation, and thus failed to fulfill the A R A criteria for the diagnosis, specific serologic and renal histopathologic studies ultimately led to the recognition of SLE. An awareness of this clinical variant of S L E may have important clinical implications, because treatment with glucocorticoids may alter the clinical course of severe lupus nephritis.~~
From the Departments o f Pediatrics (Renal Division) and Pathology, Downstate Medical Center, State University o f New York. Reprint requests." Amir Tejani, M.D., Downstate Medical Center, 450 Clarkson Ave., Box 49, Brooklyn, N Y 11203.
38
The Journal o f P E D I A T R I C S
CASE REPORTS
Patient l. This 15-year-old white boy had a 10-day history of progressive edema and gross hematuria. He denied 9ny prior history of renal disease, sore throat, joint pains, fever, rash, diarrhea, or drug ingestion. There was no family history of renal disease. On admission his temperature was 37~ pulse 90 bpm, respiratory rate 20 per minute, and blood pressure 130/90 mm Hg. Physical examination demonstrated periorbital and pedal edema but no rash or arthritis. Urinalysis showed proteinuria (4+), hematuria (3+), and granular and hyaline casts. A 24-hour urine collection contained 15 gm protein and showed a creatinine clearance of 18 ml/ See related article, p. 57.
SLE
Systemic lupus erythematosus
I
I rain/1.73 m2. The serum Na § concentration was 132 mEq/L, K + 4.1 mEq/L, BUN 35 mg/dl, creatinine 2.0 mg/dl, albumin 1.6 gm/dl, and cholesterol 350 mg/dl. The hematocrit was 35%, leukocyte count 7500/ml, and platelet count 196,000/ml; a Coombs test yielded negative results. Serologic tests showed negative ASO, ANA, and anti-DNA titers; C3 concentration was 35 mg/dl (normal 55 to 120 mg/dl). A kidney biopsy specimen revealed severe endocapillary proliferation with mesangial interposition along the glomerular basement membrane; three of 19 glomeruli had crescents (Figs. 1 and 2). Immunofluorescence examination demonstrated granular gtomerular capillary wall and mesangial deposition of C3, IgG, IgA, and lgM. The initial diagnosis was membranoproliferative glomerulonephritis type I.
Volume 105 Number 1
Acute renal failure re S L E
39
, ~I o
-L 7 k 1 I
I
4 i 0
N
Fig. 1. Light micrograph showing diffuse endocapillary proliferation of the glomcrulus. Note accentuated Iobular appearance of the glomerulus. (Original magnification x400.)
Over a 3-week period, the patient's renal function continued to deteriorate. BUN and creatinine values rose to 100 and 7.0 mg/dl, respectively, necessitating hemodialysis. Repeat serologic testing revealed C3 34 mg/dl, ANA 1:80, and anti-DNA 1:20. A second renal biopsy specimen showed progression, with crescents in 15 of 16 glomeruli (World Health Organization class 4). Pulse therapy with methylprednisolone, t gm total dose, was given over 3 days, but had to be discontinued because of severe hypertension requiring treatment with minoxidil. The patient failed to recover renal function, and maintenance hemodialysis was initiated. One month later, he developed a malar rash and knee and ankle arthralgias. At that time, serum C~ was 53 mg/dl, ANA 1:640, and anti-DNA 1:320. Patient 2. This 12-year-old Chinese girl had edema and oliguria for 1 week. She denied any history of kidney disease, rash. joint pain, fever, or drug ingestion. On admission her temperature was 37~ pulse 84 bpm, respiratory rate 18 per minute, and blood pressure 110/70 mm Hg. Physical examination demonstrated periorbilal and pretibial edema but no rash or arthritis. Urinalysis showed proteinuria (4+), hematuria (2+), and granular and hyaline casts. A 24-hour urine collection contained 7 gm protein. The serum Na + concentration was 134 mEq/L, K+ 6.9 mEq/L, BUN 59 mg/dl, creatinine 2.2 mg/dl, albumin 2.0 gm/dl, and cholesterol 189 mg/dl. The hematocrit was 31%, leukocyte count 8600/ml, and platelet count 200,000/ml. Serologic tests showed a negative ASO titer, C3 <10 mg/dl, and ANA 1:640 and antiDNA 1:40. Peritoneal dialysis was required for hypcrkaLemia. A renal biopsy specimen revealed crescents in five of 26 glomeruli, and focal segmental capillary thrombi (WHO class 4). Immunofluorescence examination demonstrated granular glomerular cap-
Fig. 2. Electron micrograph showing interposition of the mcsangial matrix (arrowhead) between the glomcrular basement membrane (GBM) and the endothelial cell (Endo). Asteri~,ks indicate presence of paramesangial electron-dense deposits. (Original magnification x5400.)
40
Phadke et al.
The Journal of Pediatrics July 1984
Fig. 3. Immunoftuorescencephotomicrograph showing diffuse glomerular capillary wall and mesangial deposition of IgG. (Original magnification •
illary wall and mesangial deposits of C3, lgG, lgA, and lgM (Fig. 3). Pulse therapy with methylprednisolone,2 gm total dose, was given intravenously over 5 days. A repeat kidney biopsy specimen after completion of pulse therapy showed an attenuation of endocapillary proliferation and crescents in two of eight glomeruli. BUN and creatinine values declined to 38 and 1.5 mg/dl, respectively. At the time of discharge, creatinine clearance was 52 ml/min/1.73 m2, and 24-hour urinary protein excretion rate was 6.4 gm. The patient has been given prednisone 60 mg/m2/day orally in divided doses; her C3 is 73 mg/dl, ANA 1:80, anti-DNA 1:10. Over a 3-month follow-up, she has not developed any cxtrarenal manifestations of SLE. DISCUSSION We have described two patients with acute renal failure as the initial clinical manifestation of SLE. At the time of presentation, they had no extrarenal manifestations of the disease and thus failed to satisfy the standard ARA criteria for diagnosis of SLE. Even during follow-up, only one patient developed any of the classic findings of SLE. The diagnosis of SLE in these two patients was established on the basis of serologic tests and characteristic renal histopathologic findings. Weinstein et al. 13 concluded that measurements of anti-DNA antibody titers and serum complement levels are highly sensitive and specific markers for SLE; the presence of both abnormalities had 100% predictive value for the diagnosis. Moses and Barland ~4 concur that depressed levels of C3 in conjunction with elevated titers of anti-DNA antibodies are extremely valuable criteria for the diagnosis of SLE. Because these serologic tests have a
sensitivity and specificity that exceed those of the established clinical criteria, these investigators suggest that these data should be incorporated into revised ARA guidelines or be included in a new set of laboratory criteria designed to replace the ARA diagnostic protocol. Both of our patients had renal histopathologic abnormalities that were compatible with the diagnosis of SLE. 15-17 Although a pattern of membranoproliferative glomerunephritis may be seen in the renal biopsy of patients with SLE, ~5it has never been reported to occur in association with acute renal failure, as in our patient 1. In pediatric patients, the initial renal involvement of SLE usually is manifested as asymptomatic hematuria and proteinuria, or the nephrotic syndrome ~s-2~;extrarenal signs and symptoms have always been reported to be present at the time of diagnosis. Similarly, in adult patients lupus nephropathy is a rare initial manifestation of SLE. In one series of 150 patients with SLE, only 6% had lupus nephritis as the initial manifestation; of these, nearly all had nephrotic syndrome at onset, and none had acute renal failure.9 A critical review of the report of Lynn et alfl purporting to document the occurrence of lupus nephropathy as the sole initial manifestation of SLE, suggests that even these patients had extrarenal findings, including fever, arthralgias, or rash, that aided in making the correct diagnosis. Acute renal failure in patients with SLE has been described almost exclusively in adult patients, and always in association with extrarenal findings.22-24 It has been postulated that acute renal failure is a result of either severe interstitial nephritis22.23 or severe diffuse prolifera-
Volume 105 Number 1
tive changes in the glomeruli? 4 A 14-year-old girl with S L E with acute renal failure has been described; however, complications including concomitant lupus pneumonitis and R B C aplasia 25 facilitated correct recognition of the underlying disease. Although acute renal failure secondary to glomerular disease is much less common than acute tubular necrosis, 26 the occurrence of heavy proteinuria, urinary casts, or hypertension should suggest the possibility of an underlying glomerular cause such as SLE. Several reports of uncontrolled studies describe a beneficial effect of pulse steroid therapy in patients with severe proliferative glomerulonephritis, and lupus nephritis in particular?0 ~2 In the series of Rose et al., ~2 all patients given treatment within 1 to 4 months of onset of disease demonstrated sustained improvement in renal function, irrespective of the underlying disease. The patients who did not respond to high doses of methylprednisolone given intravenously were those who had long duration of disease (mean 19 months) before receiving pulse therapy. Although the effectiveness of large doses of steroids is quite variable and can sometimes be hazardous in terms of increasing fluid retention and causing severe hypertension, these data suggest that such therapy warrants consideration in a child with rapidly progressive glomerulonephritis. In our patient 1, results of the initial serologic tests for S L E were negative and led to an unavoidable delay in establishing the diagnosis. Whether earlier treatment would have had an effect on the eventual outcome is open to speculation. However, the clinical course and histologic improvement observed in patient 2 tentatively support the use of pulse steroid therapy in children with S L E and severe proliferative glomerulonephritis. We emphasize the importance of considering the diagnosis of S L E in patients with acute renal failure, even in the absence of extrarenal manifestations, especially when there are signs and symptoms of a nephritic or nephrotic syndrome to suggest a glomerular cause rather than acute tubular necrosis. Awareness of the possibility of S L E as a cause of acute renal failure may help in formulating a therapeutic strategy that can improve the eventual outcome in affected patients. REFERENCES
1. Cohen AS, Reynolds WE, Franklin EC, et al: Preliminary criteria for the classification of systemic lupus erythematosus. Bull Rheum Dis 21:643, 1971. 2. Lynn Rl, Siegel N J, Hayslett JP: Lupus nephropathy as the initial manifestation of systemic lupus erythematosus. Yale J Biol Med 53:353, 1980. 3. Libit SA, Burke B, Michael AF, et al: Extramembranous glomerulonephritis in childhood: Relationship to systemic lupus erythematosus. J PEDIATR88:394, 1976. 4. Kallen R J, Lee SK, Aronson A J, et al: Idiopathic membra-
A c u t e renal f a i l u r e re S L E
5.
6. 7.
8. 9.
10.
11.
12.
13.
14. 15.
16. 17.
18. 19. 20.
21.
22.
23.
24.
25.
26.
41
nous nephropathy preceding the emergence of systemic lupus erythematosus. J PEDIATR90:72, 1977. Yamamuchi H, Rooney P, Hopper J: Nephrotic state as the chief manifestation of systemic lupus erythematosus. Ann Intern Med 57:981, 1962. Simenhoff ML, Merill JP: The spectrum of lupus nephritis. Nephron 1:348, 1964. Lief PD, Barland P, Bank N: Diagnosis of lupus nephritis by skin immunofluorescence in the absence of extrarenal manifestations of systemic lupus erythematosus. Am J Med 63:441, 1977. Meislin AG, Rothfield N: Systemic lupus crythematosus in childhood. Pediatrics 42:37, 1968. Estes D, Christian C: The natural history of systemic lupus erythematosus by prospective analysis. Medicine 50:85, 1971. Fessel WJ: Reversal of azotemia in lupus nephritis by megadose corticosteroid therapy. Arthritis Rheum 20:1564, 1977. Cathacart ES, Idelson BA, Scheinberg MA, Couser WC: Beneficial effects of methylprednisotone pulse therapy in diffuse proliferative lupus nephritis. Lancet 1:163, 1976. Rose GM, Cole BR, Robson AM: The treatment of severe glomerulonephritis in children using high-dose intravenous methylprednisolone pulses. Am J Kidney Dis 1:148, 198 I. Weinstein A, Brodwell B, Stone B, Tibbetts C, Rothfield N: Antibodies to native DNA and serum complement (C3) levels. Am J Med 74:206, 1983. Moses S, Barland P: Laboratory criteria for a diagnosis of systemic lupus erythematosus. JAMA 242:1939, 1979. McCluskey RT: Lupus nephritis. In Sommers SC, editor: Kidney pathology decennial 1966-1975. New York, 1975, Appleton-Century-Crofts, pp 435-460. Churg J, Gishman E: Electron microscopy of glomerulonephritis. Curr Topics PathoI 61:I07, I976. Dujorne E, Pollack V, Pirani C, Di[lard M: The distribution and character of glomerular deposits in systemic lupus erythematosus. Kidney Int 2:33, 1972. Walravens PA, Chase HP: The prognosis of childhood systemic lupus erythematosus. Am J Dis Child 130:929, 1976. Robinson M J, Williams A J: Systemic lupus erythematosus in childhood. Aust Pediatr J 3:36, 1967. Fish A J, Blau EB, Westberg NG, Burke BA: Systemic lupus erythematosus within the first two decades of life. Am J Med 62:99, 1977. Morris MC, Cameron JS, Chantler C, Turner DR: Systemic lupus erythematosus with nephritis. Arch Dis Child 56:779, 1981. Tron F, Ganeval D, Droz D: Immunologically mediated acute renal failure of nonglomerular origin in the course of systemic lupus erythematosus. Am J Med 3:529, 1979. Cunningham E, Brentjens J, Venuto R: Acute renal failure secondary to interstitial lupus nephritis. Arch Intern Med 138:1560, 1978. Ponticelli C, lmbasciati E, Brancoccio D, Tarantion A, Rivolta E: Acute renal failure in systemic lupus erythematosus. Br Med J 3:716, 1974. Isbister J, Ralston M, Wright F: Fulminant lupus pneumonitis with acute renal failure and RBC aplasia. Arch Intern Med 141:1081, 1981. McPhaul J J: Acute glomerular disease presenting as acute renal failure. Semin Nephrol 1:21, 1981.
Kishore Phadke, M.D., Howard Trachtman, M.D., Anthony Nicastri, M.D., C. K. Chen, M.D., and Amir Tejani, M.D. Brooklyn, N.Y.
FOURTEEN CLINICAL CRITERIA have been established by the American Rheumatology Association to aid in the identification of patients with systemic lupus erythematosus~; the presence of four or more criteria in an individual patient is considered necessary for the diagnosis. Since then (1971), there have been numerous reports of patients with biopsy-proved lupus nephritis who lacked the requisite number of diagnostic criteria at the time of initial clinical presentation2-9; nearly all of these patients had moderate proteinuria or the nephrotic syndrome. There have been no reports of patients with S L E with acute renal failure in the absence of extrarenal manifestations of the disease. We describe two pediatric patients with S L E in whom the sole presenting manifestation was acute renal failure. Although they had no extrarenal manifestations of S L E at the time of their initial evaluation, and thus failed to fulfill the A R A criteria for the diagnosis, specific serologic and renal histopathologic studies ultimately led to the recognition of SLE. An awareness of this clinical variant of S L E may have important clinical implications, because treatment with glucocorticoids may alter the clinical course of severe lupus nephritis.~~
From the Departments o f Pediatrics (Renal Division) and Pathology, Downstate Medical Center, State University o f New York. Reprint requests." Amir Tejani, M.D., Downstate Medical Center, 450 Clarkson Ave., Box 49, Brooklyn, N Y 11203.
38
The Journal o f P E D I A T R I C S
CASE REPORTS
Patient l. This 15-year-old white boy had a 10-day history of progressive edema and gross hematuria. He denied 9ny prior history of renal disease, sore throat, joint pains, fever, rash, diarrhea, or drug ingestion. There was no family history of renal disease. On admission his temperature was 37~ pulse 90 bpm, respiratory rate 20 per minute, and blood pressure 130/90 mm Hg. Physical examination demonstrated periorbital and pedal edema but no rash or arthritis. Urinalysis showed proteinuria (4+), hematuria (3+), and granular and hyaline casts. A 24-hour urine collection contained 15 gm protein and showed a creatinine clearance of 18 ml/ See related article, p. 57.
SLE
Systemic lupus erythematosus
I
I rain/1.73 m2. The serum Na § concentration was 132 mEq/L, K + 4.1 mEq/L, BUN 35 mg/dl, creatinine 2.0 mg/dl, albumin 1.6 gm/dl, and cholesterol 350 mg/dl. The hematocrit was 35%, leukocyte count 7500/ml, and platelet count 196,000/ml; a Coombs test yielded negative results. Serologic tests showed negative ASO, ANA, and anti-DNA titers; C3 concentration was 35 mg/dl (normal 55 to 120 mg/dl). A kidney biopsy specimen revealed severe endocapillary proliferation with mesangial interposition along the glomerular basement membrane; three of 19 glomeruli had crescents (Figs. 1 and 2). Immunofluorescence examination demonstrated granular gtomerular capillary wall and mesangial deposition of C3, IgG, IgA, and lgM. The initial diagnosis was membranoproliferative glomerulonephritis type I.
Volume 105 Number 1
Acute renal failure re S L E
39
, ~I o
-L 7 k 1 I
I
4 i 0
N
Fig. 1. Light micrograph showing diffuse endocapillary proliferation of the glomcrulus. Note accentuated Iobular appearance of the glomerulus. (Original magnification x400.)
Over a 3-week period, the patient's renal function continued to deteriorate. BUN and creatinine values rose to 100 and 7.0 mg/dl, respectively, necessitating hemodialysis. Repeat serologic testing revealed C3 34 mg/dl, ANA 1:80, and anti-DNA 1:20. A second renal biopsy specimen showed progression, with crescents in 15 of 16 glomeruli (World Health Organization class 4). Pulse therapy with methylprednisolone, t gm total dose, was given over 3 days, but had to be discontinued because of severe hypertension requiring treatment with minoxidil. The patient failed to recover renal function, and maintenance hemodialysis was initiated. One month later, he developed a malar rash and knee and ankle arthralgias. At that time, serum C~ was 53 mg/dl, ANA 1:640, and anti-DNA 1:320. Patient 2. This 12-year-old Chinese girl had edema and oliguria for 1 week. She denied any history of kidney disease, rash. joint pain, fever, or drug ingestion. On admission her temperature was 37~ pulse 84 bpm, respiratory rate 18 per minute, and blood pressure 110/70 mm Hg. Physical examination demonstrated periorbilal and pretibial edema but no rash or arthritis. Urinalysis showed proteinuria (4+), hematuria (2+), and granular and hyaline casts. A 24-hour urine collection contained 7 gm protein. The serum Na + concentration was 134 mEq/L, K+ 6.9 mEq/L, BUN 59 mg/dl, creatinine 2.2 mg/dl, albumin 2.0 gm/dl, and cholesterol 189 mg/dl. The hematocrit was 31%, leukocyte count 8600/ml, and platelet count 200,000/ml. Serologic tests showed a negative ASO titer, C3 <10 mg/dl, and ANA 1:640 and antiDNA 1:40. Peritoneal dialysis was required for hypcrkaLemia. A renal biopsy specimen revealed crescents in five of 26 glomeruli, and focal segmental capillary thrombi (WHO class 4). Immunofluorescence examination demonstrated granular glomerular cap-
Fig. 2. Electron micrograph showing interposition of the mcsangial matrix (arrowhead) between the glomcrular basement membrane (GBM) and the endothelial cell (Endo). Asteri~,ks indicate presence of paramesangial electron-dense deposits. (Original magnification x5400.)
40
Phadke et al.
The Journal of Pediatrics July 1984
Fig. 3. Immunoftuorescencephotomicrograph showing diffuse glomerular capillary wall and mesangial deposition of IgG. (Original magnification •
illary wall and mesangial deposits of C3, lgG, lgA, and lgM (Fig. 3). Pulse therapy with methylprednisolone,2 gm total dose, was given intravenously over 5 days. A repeat kidney biopsy specimen after completion of pulse therapy showed an attenuation of endocapillary proliferation and crescents in two of eight glomeruli. BUN and creatinine values declined to 38 and 1.5 mg/dl, respectively. At the time of discharge, creatinine clearance was 52 ml/min/1.73 m2, and 24-hour urinary protein excretion rate was 6.4 gm. The patient has been given prednisone 60 mg/m2/day orally in divided doses; her C3 is 73 mg/dl, ANA 1:80, anti-DNA 1:10. Over a 3-month follow-up, she has not developed any cxtrarenal manifestations of SLE. DISCUSSION We have described two patients with acute renal failure as the initial clinical manifestation of SLE. At the time of presentation, they had no extrarenal manifestations of the disease and thus failed to satisfy the standard ARA criteria for diagnosis of SLE. Even during follow-up, only one patient developed any of the classic findings of SLE. The diagnosis of SLE in these two patients was established on the basis of serologic tests and characteristic renal histopathologic findings. Weinstein et al. 13 concluded that measurements of anti-DNA antibody titers and serum complement levels are highly sensitive and specific markers for SLE; the presence of both abnormalities had 100% predictive value for the diagnosis. Moses and Barland ~4 concur that depressed levels of C3 in conjunction with elevated titers of anti-DNA antibodies are extremely valuable criteria for the diagnosis of SLE. Because these serologic tests have a
sensitivity and specificity that exceed those of the established clinical criteria, these investigators suggest that these data should be incorporated into revised ARA guidelines or be included in a new set of laboratory criteria designed to replace the ARA diagnostic protocol. Both of our patients had renal histopathologic abnormalities that were compatible with the diagnosis of SLE. 15-17 Although a pattern of membranoproliferative glomerunephritis may be seen in the renal biopsy of patients with SLE, ~5it has never been reported to occur in association with acute renal failure, as in our patient 1. In pediatric patients, the initial renal involvement of SLE usually is manifested as asymptomatic hematuria and proteinuria, or the nephrotic syndrome ~s-2~;extrarenal signs and symptoms have always been reported to be present at the time of diagnosis. Similarly, in adult patients lupus nephropathy is a rare initial manifestation of SLE. In one series of 150 patients with SLE, only 6% had lupus nephritis as the initial manifestation; of these, nearly all had nephrotic syndrome at onset, and none had acute renal failure.9 A critical review of the report of Lynn et alfl purporting to document the occurrence of lupus nephropathy as the sole initial manifestation of SLE, suggests that even these patients had extrarenal findings, including fever, arthralgias, or rash, that aided in making the correct diagnosis. Acute renal failure in patients with SLE has been described almost exclusively in adult patients, and always in association with extrarenal findings.22-24 It has been postulated that acute renal failure is a result of either severe interstitial nephritis22.23 or severe diffuse prolifera-
Volume 105 Number 1
tive changes in the glomeruli? 4 A 14-year-old girl with S L E with acute renal failure has been described; however, complications including concomitant lupus pneumonitis and R B C aplasia 25 facilitated correct recognition of the underlying disease. Although acute renal failure secondary to glomerular disease is much less common than acute tubular necrosis, 26 the occurrence of heavy proteinuria, urinary casts, or hypertension should suggest the possibility of an underlying glomerular cause such as SLE. Several reports of uncontrolled studies describe a beneficial effect of pulse steroid therapy in patients with severe proliferative glomerulonephritis, and lupus nephritis in particular?0 ~2 In the series of Rose et al., ~2 all patients given treatment within 1 to 4 months of onset of disease demonstrated sustained improvement in renal function, irrespective of the underlying disease. The patients who did not respond to high doses of methylprednisolone given intravenously were those who had long duration of disease (mean 19 months) before receiving pulse therapy. Although the effectiveness of large doses of steroids is quite variable and can sometimes be hazardous in terms of increasing fluid retention and causing severe hypertension, these data suggest that such therapy warrants consideration in a child with rapidly progressive glomerulonephritis. In our patient 1, results of the initial serologic tests for S L E were negative and led to an unavoidable delay in establishing the diagnosis. Whether earlier treatment would have had an effect on the eventual outcome is open to speculation. However, the clinical course and histologic improvement observed in patient 2 tentatively support the use of pulse steroid therapy in children with S L E and severe proliferative glomerulonephritis. We emphasize the importance of considering the diagnosis of S L E in patients with acute renal failure, even in the absence of extrarenal manifestations, especially when there are signs and symptoms of a nephritic or nephrotic syndrome to suggest a glomerular cause rather than acute tubular necrosis. Awareness of the possibility of S L E as a cause of acute renal failure may help in formulating a therapeutic strategy that can improve the eventual outcome in affected patients. REFERENCES
1. Cohen AS, Reynolds WE, Franklin EC, et al: Preliminary criteria for the classification of systemic lupus erythematosus. Bull Rheum Dis 21:643, 1971. 2. Lynn Rl, Siegel N J, Hayslett JP: Lupus nephropathy as the initial manifestation of systemic lupus erythematosus. Yale J Biol Med 53:353, 1980. 3. Libit SA, Burke B, Michael AF, et al: Extramembranous glomerulonephritis in childhood: Relationship to systemic lupus erythematosus. J PEDIATR88:394, 1976. 4. Kallen R J, Lee SK, Aronson A J, et al: Idiopathic membra-
A c u t e renal f a i l u r e re S L E
5.
6. 7.
8. 9.
10.
11.
12.
13.
14. 15.
16. 17.
18. 19. 20.
21.
22.
23.
24.
25.
26.
41
nous nephropathy preceding the emergence of systemic lupus erythematosus. J PEDIATR90:72, 1977. Yamamuchi H, Rooney P, Hopper J: Nephrotic state as the chief manifestation of systemic lupus erythematosus. Ann Intern Med 57:981, 1962. Simenhoff ML, Merill JP: The spectrum of lupus nephritis. Nephron 1:348, 1964. Lief PD, Barland P, Bank N: Diagnosis of lupus nephritis by skin immunofluorescence in the absence of extrarenal manifestations of systemic lupus erythematosus. Am J Med 63:441, 1977. Meislin AG, Rothfield N: Systemic lupus crythematosus in childhood. Pediatrics 42:37, 1968. Estes D, Christian C: The natural history of systemic lupus erythematosus by prospective analysis. Medicine 50:85, 1971. Fessel WJ: Reversal of azotemia in lupus nephritis by megadose corticosteroid therapy. Arthritis Rheum 20:1564, 1977. Cathacart ES, Idelson BA, Scheinberg MA, Couser WC: Beneficial effects of methylprednisotone pulse therapy in diffuse proliferative lupus nephritis. Lancet 1:163, 1976. Rose GM, Cole BR, Robson AM: The treatment of severe glomerulonephritis in children using high-dose intravenous methylprednisolone pulses. Am J Kidney Dis 1:148, 198 I. Weinstein A, Brodwell B, Stone B, Tibbetts C, Rothfield N: Antibodies to native DNA and serum complement (C3) levels. Am J Med 74:206, 1983. Moses S, Barland P: Laboratory criteria for a diagnosis of systemic lupus erythematosus. JAMA 242:1939, 1979. McCluskey RT: Lupus nephritis. In Sommers SC, editor: Kidney pathology decennial 1966-1975. New York, 1975, Appleton-Century-Crofts, pp 435-460. Churg J, Gishman E: Electron microscopy of glomerulonephritis. Curr Topics PathoI 61:I07, I976. Dujorne E, Pollack V, Pirani C, Di[lard M: The distribution and character of glomerular deposits in systemic lupus erythematosus. Kidney Int 2:33, 1972. Walravens PA, Chase HP: The prognosis of childhood systemic lupus erythematosus. Am J Dis Child 130:929, 1976. Robinson M J, Williams A J: Systemic lupus erythematosus in childhood. Aust Pediatr J 3:36, 1967. Fish A J, Blau EB, Westberg NG, Burke BA: Systemic lupus erythematosus within the first two decades of life. Am J Med 62:99, 1977. Morris MC, Cameron JS, Chantler C, Turner DR: Systemic lupus erythematosus with nephritis. Arch Dis Child 56:779, 1981. Tron F, Ganeval D, Droz D: Immunologically mediated acute renal failure of nonglomerular origin in the course of systemic lupus erythematosus. Am J Med 3:529, 1979. Cunningham E, Brentjens J, Venuto R: Acute renal failure secondary to interstitial lupus nephritis. Arch Intern Med 138:1560, 1978. Ponticelli C, lmbasciati E, Brancoccio D, Tarantion A, Rivolta E: Acute renal failure in systemic lupus erythematosus. Br Med J 3:716, 1974. Isbister J, Ralston M, Wright F: Fulminant lupus pneumonitis with acute renal failure and RBC aplasia. Arch Intern Med 141:1081, 1981. McPhaul J J: Acute glomerular disease presenting as acute renal failure. Semin Nephrol 1:21, 1981.