Three cases presenting with systemic lupus erythematosus and minimal change nephrotic syndrome

Three cases presenting with systemic lupus erythematosus (SLE) and minimal change nephrotic syndrome (MCNS) are reported in this paper. All cases were female; they abruptly developed nephrotic syndrome at the age of 30, 11 and 23 years, respectively. In Case 1, the diagnosis of SLE was based on fever, butterfly rash, Raynaud's phenomenon, leukopenia, lymphopenia, hypocomplementemia, a high titer of anti-DNA antibodies, positive DNA and LE test, and the presence of anti-nuclear antibodies (speckled pattern). In Case 2, the diagnosis was based on butterfly rash, central nervous system involvement, lymphopenia, hypocomplementemia, a positive LE cell phenomenon, a high titer of anti-DNA antibodies and a positive DNA test. In Case 3, the diagnosis was based on photosensitivity, alopecia, lymphopenia, hypocomplementemia, a high titer of anti-DNA antibodies, a positive DNA test and a positive LE cell phenomenon. In these three cases, initial symptoms were puffy face and pretibial edema which occurred suddenly. These symptoms disappeared completely after either corticosteroid therapy or a combination therapy using corticosteroids and immunosuppressive drugs. These patients took a favorable course and no aggravation was noted in the findings of urinalysis and renal functions. In two of these cases, the diagnostic criteria for SLE were satisfied, but the remaining patient fulfilled only three criteria except for renal disorder. In each of these cases, minor glomerular abnormalities were disclosed by renal histology. It seems likely that SLE was complicated by MCNS in these cases. From these cases, it is suggested that there is a possibility of immunological abnormalities associated with SLE and MCNS.     

An aged woman with minimal change nephrotic syndrome complicated with reversible acute renal failure

Reported was an aged woman (80-year-old) of minimal change nephrotic syndrome which was complicated with reversible oliguric acute renal failure. The patient presented massive proteinuria, anasarca, and severe azotemia. She recovered conservatively from the acute renal failure and subsequently remitted from the nephrotic syndrome after the treatment which comprised albumin infusion, diuretics, adrenocorticosteroid hormones (including the pulse therapy), antiplatelet drug, and anticoagulants. The histopathologic findings of renal biopsy were compatible with minor glomerular abnormalities and acute tubular necrosis with many tubular casts. The previously reported cases older than 80-year-old which remitted from minimal change nephrotic syndrome complicated with reversible acute renal failure, were very rare. The present case was the second case among the literatures.     

Postpartum renal failure in systemic lupus erythematosus

The present report describes an unusual association between postpartum renal failure and systemic lupus erythematosus. Two healty young women developed progressive renal failure several weeks after delivery accompanied by the presence in their serum of strongly reactive anti-nuclear antibodies and positive anti-DNA antibodies. In both cases kidney biopsy disclosed light and electron microscopy pictures typical of idiopathic postpartum renal failure, with multiple intravascular thrombi and no evidence of active lupus nephritis. Intrarenal microthrombi formation may represent a form of exacerbation of systemic lupus erythematosus after delivery. The early recognition of this syndrome may have therapeutic implications.     

A case of elderly-onset systemic lupus erythematosus presenting as acute renal failure due to disseminated intravascular coagulation

Herein we describe a case of a patient with elderly-onset systemic lupus erythematosus presenting as acute renal failure due to disseminated intravascular coagulation. A 78-year-old man was admitted to our hospital with fever and generalized lymphadenopathy. He was diagnosed as having systemic lupus erythematosus on the basis of renal involvement, hematological abnormality and positivity for antinuclear and anti-double-stranded DNA antibodies. Renal biopsy revealed lupus nephritis (class III and V (A/C)) with focal glomerular thrombosis. He responded to hemodialysis and corticosteroid therapy with remission of serological values and renal function. Possible mechanisms underlying the coexistence of these conditions are discussed.     

Rapid deterioration of renal function in minimal change nephrotic syndrome

This report concerns two boys with minimal change nephrotic syndrome progressed to renal failure. The first case aged 17 being a steroid sensitive infrequent relapse developed acute renal failure at his third relapse and recovered soon after the treatment with diuretics and corticosteroids. The second case aged 15 being a steroid dependent frequent relapse became steroid resistant at his 11th relapse and progressed to renal failure seven months later. As the causes of renal failure, acute tubular necrosis and tubular obstruction by casts were suspected in the former. Renal vein thrombosis, morphological transition of renal histology, hemodynamic change and change in glomerular permeability might be occurred in the latter. Renal failure is a rare complication of minimal change nephrotic syndrome and the cause is variable. Precise diagnosis and prompt treatment should be needed to improve the prognosis.     

A case of acute dissecting aneurysm of the aorta in systemic lupus erythematosus

A 28 year-old female patient who has been diagnosed as having systemic lupus erythematosus (SLE) developed an acute dissecting aneurysm of the aorta (DeBakey type I). The long-term, large dose corticosteroid therapy (i.e., accumulative dose of about 60 g) administered for the treatment of lupus nephritis (WHO class III----IV) was considered to be responsible for a hypercholesterolemia (300-560 mg/dl) and a steroid-dependent hypertension (WHO class III) in this patient. The autopsy findings for the aorta were compatible with atherosclerotic changes but not with lupus arteritis. While atherosclerotic cardiovascular complications have been considered to be rare in patients with SLE, a growing body of evidence suggests that the incidence of such a complication may be increasing along with a dramatic improvement in the longevity of patients with SLE after an introduction of a large dose, long-term corticosteroid therapy.     

Captopril treatment of hypertension and renal failure in systemic lupus erythematosus

Captopril, an angiotensin-converting enzyme inhibitor, was used to treat 14 patients with lupus nephritis and severe hypertension. All patients had reduced renal function and were on regular immunosuppressive therapy with corticosteroids and azathioprine. The initial dosage of captopril was reduced according to the level of renal impairment. 11 patients were treated for more than 6 months. Excellent blood pressure control was achieved with captopril, from a mean of 178 +/- 7/110 +/- 4 to 145 +/- 5/92 +/- 3 mm Hg at 6 months, usually in combination with a diuretic only. In 5 cases, a beta-blocker was added. In 3 patients, captopril therapy was discontinued within the 1st month of treatment. 1 patient did not respond to captopril at all; 1 patient had a rejection crisis and required dialysis; in 1 case, a general exanthema developed within 3 weeks and captopril medication was stopped. In addition to blood pressure control, renal function improved in 7 of the long-term-treated patients (mean increase in glomerular filtration rate 73 +/- 34%). In 3 patients, a continued slow deterioration renal function occurred, and in 1 patient, renal function remained unchanged. It is concluded that captopril is an effective antihypertensive drug in patients with systemic lupus erythematosus (SLE). Captopril treatment increased renal function in 64% of patients on long-term therapy. Not only optimal blood pressure control but other factors may also contribute to this beneficial effect, such as drug-induced prostaglandin release potentiating immunosuppressive treatment. Captopril may in fact be the drug of choice for the treatment of SLE patients with severe hypertension.     

Bilateral renal malacoplakia in systemic lupus erythematosus and adrenogenital syndrome

We report a case of an 8-year-old girl with adrenogenital syndrome secondary to adrenocortical hyperplasia. Thirteen years later systemic lupus erythematosus developed with lupus nephritis. In spite of complex continuous immunosuppressive therapy, she died from terminal renal failure. At autopsy, extensive bilateral renal malacoplakia was discovered. The role of recurrent urinary tract infections and of immunological disturbances in the pathogenesis of malacoplakia is discussed.     

A case of acute pancreatitis in a patient with systemic lupus erythematosus

Systemic lupus erythematosus (SLE) is an auto-immune disease which can affect multiple organs. It may also involve the pancreas and in rare cases may cause acute pancreatitis. The etiology of acute pancreatitis in SLE is a matter of controversy. We present a case diagnosed with SLE that developed acute pancreatitis while being treated with corticosteroids. During the course of the disease, pancreatic pseudocysts developed and were treated by percutaneous drainage. There are few reports in the literature about the therapy of acute pancreatitis and percutaneous drainage of pseudocysts in SLE. We discuss the pathogenesis and therapy for acute pancreatitis in SLE patients.     

Kimura's disease associated with minimal change nephrotic syndrome: A case report

Kimura's disease is a rare disorder that involves regional lymph nodes and the major salivary glands, which become infiltrated by eosinophils and lymphocytes. Renal lesions associated with Kimura's disease are rare. We describe the case of a 60-year-old Japanese woman who first noted a nodular mass in a salivary gland. As the nodule grew, nephrotic syndrome and heart failure developed. A biopsy of the nodule revealed Kimura's disease, and surgical excision was performed. After the operation, the heart failure and nephrotic syndrome, which were diagnosed as minimal change disease on renal biopsy, improved rapidly without steroid therapy. Four months later, the nephrotic syndrome recurred without recurrence of Kimura's disease. The patient showed marked improvement during prednisolone therapy (40 mg/d) and was in complete remission 4 weeks after the initiation of steroid therapy. This case shows that surgical excision and prednisolone therapy are useful for nephrotic syndrome associated with Kimura's disease.     

Adult minimal change glomerulopathy with acute renal failure

Oliguric acute renal failure occurs in some adult patients with minimal change glomerulopathy. To look for clinical and pathologic factors that increase the risk for developing acute renal failure, 21 adults with minimal change glomerulopathy and a serum creatinine greater than 177 mumol/L (mean, 486 mumol/L; range, 194 to 1,344 mumol/L) (greater than 2.0 mg/dL [mean, 5.5 mg/dL; range, 2.2 to 15.2 mg/dL]) were compared with 50 adults with minimal change glomerulopathy and a serum creatinine less than 133 mumol/L (mean, 88 mumol/L; range, 53 to 124 mumol/L) (less than 1.5 mg/dL [mean, 1.0 mg/dL; range, 0.6 to 1.4 mg/dL]). Minimal change glomerulopathy patients with acute renal failure were older (59.5 v 40.3 years, P less than 0.001), and had higher systolic blood pressure (158 v 138 mm Hg, P = 0.001), more proteinuria (13.5 v 7.9 g/24 h, P = 0.01), and more arteriosclerosis in the renal biopsy specimen (1.7 + v 0.7 + on a scale of 0 to 4+, P = 0.005). Tubular epithelial simplification identical to that observed with ischemic acute renal failure (acute tubular necrosis) was observed in 71% of the patients with serum creatinine greater than 177 mumol/L (greater than 2.0 mg/dL) and 0% of those with less than 133 mumol/L (less than 1.5 mg/dL). All 18 patients with renal failure for whom follow-up data were available had recovery of function (mean creatinine, 539 +/- 301 mumol/L [6.1 +/- 3.4 mg/dL] at the time of biopsy and 106 +/- 27 mumol/L [1.2 +/- 0.3 mg/dL] at last follow-up), but sometimes only after weeks of dialysis support.(ABSTRACT TRUNCATED AT 250 WORDS)     

Renal failure with minimal change nephrotic syndrome: reversal with hemodialysis

A patient with acute renal failure accompanying nephrotic syndrome associated with minimal change nephropathy acutely reversed with hemodialysis and ultrafiltration. This response is felt to support the interstitial edema tubular obstruction theory of renal failure occurring with minimal change disease. Acute hemodialysis with significant fluid removal may dramatically reverse severe degrees of azotemia in this condition independent of corticosteroid therapy. The latter, however, may be necessary for remission of the nephrotic syndrome.     

Antiphospholipid syndrome in systemic lupus erythematosus

Renal biopsies occasionally show a combination of thrombotic microangiopathy as a result of antiphospholipid syndrome and lupus nephritis. The thrombosis in this case preceded the onset of lupus probably by approximately 8 yr, consisting of repeated fetal loss and venous thrombosis. More severe disease may have both arterial and venous thrombotic manifestations, including pulmonary emboli and cerebrovascular lesions. The antiphospholipid syndrome bears no relationship to the class of lupus nephritis but is accompanied by more frequent and greater hypertension and greater azotemia and interstitial fibrosis, and is associated with worse outcomes than lupus nephritis without antiphospholipid syndrome.     

Minimal change disease in systemic lupus erythematosus

We report the clinical and pathologic findings in 7 patients with systemic lupus erythematosus and minimal change disease. All 7 patients presented with full nephrotic syndrome including peripheral edema, nephrotic range proteinuria (mean 9.6 g/day), and hypoalbuminemia (mean 1.8 g/dl). In all cases, renal biopsy revealed diffuse foot process effacement in the absence of significant peripheral capillary wall immune deposits, findings consistent with minimal-change disease. In addition, 5 cases displayed mesangial electron-dense deposits, with or without associated mesangial proliferation, consistent with underlying lupus nephritis class II. In all cases, steroid therapy induced a rapid remission of nephrotic syndrome. Minimal change disease is an underrecognized and readily reversible form of nephrotic syndrome in systemic lupus erythematosus. Because it may occur superimposed on mild mesangial proliferative lupus nephritis, this entity may be misinterpreted as an atypical presentation of lupus nephritis class II. Proper recognition of this entity requires careful integration of the renal biopsy immunofluorescence and electron microscopic findings.     

Nephrotoxicity of once-daily cyclosporine A in minimal change nephrotic syndrome

Background  

Although once-daily cyclosporine (CsA) therapy may have greater nephrotoxic-sparing effects than standard twice-daily therapy, little information is available in children with steroid-dependent minimal change nephrotic syndrome (MCNS) regarding histological analysis after long-term once-daily administration.