Long-term effects of steroid treatment on nephrotic syndrome associated with Kimura's disease and a review of the literature.

A 68-year-old Japanese male (Case 1) and a 59-year-old Japanese male immigrant to Brazil (Case 2) who suffered from subcutaneous eosinophilic lymphoid granuloma (Kimura's disease) of several years duration, developed nephrotic syndrome. Renal biopsy demonstrated membranous nephropathy in Case 1 and minimal-change lesion in Case 2. Both patients were treated with prednisolone for several months. There was complete remission of nephrotic syndrome in eight years in Case 1 and in three months in Case 2. Proteinuria did not reappear after remission of nephrotic syndrome in either case. In contrast subcutaneous tumors subsided in both cases during steroid treatment but again became active immediately following discontinuation of the drug. These clinical observations suggest that, though some common factor(s) might mediate the development of both skin and renal lesions, the mechanism of action on these two target organs may differ, perhaps serving only as a "trigger" for the initiating mechanism of renal disease.     

A Case with Acute Renal Failure and Subsequent Nephrotic Syndrome

Nephrotic syndrome due to secondary amyloidosis is not so common, and the prognosis depends on primary disease. We report a case of secondary amyloidosis caused by Takayasu's arteritis. Sustained high fever and acute renal failure proceeded to the occurrence of nephrotic syndrome. Secondary amyloidosis was diagnosed by renal biopsy before the diagnosis of primary disease. She was completely recovered from nephrotic syndrome after two years' treatment with prednisolone, aspirin, and dimethyl sulfoxide. This rare case provides meaningful suggestions for the diagnosis and treatment of acute renal failure and nephrotic syndrome caused by secondary amyloidosis.     

Remission of nephrotic syndrome due to AA amyloidosis and initiation of glomerular repair after surgical resection of localized Castleman's disease

To shed further light on the eventual destiny of amyloid kidney deposits after interruption of amylogenic stimulus, we report a case of a 47-year-old woman with nephrotic syndrome due to renal amyloidosis, complicating abdominal Castleman's disease. After 5 courses of therapy with melphalan and prednisolone which failed to improve the nephrotic syndrome or her general clinical condition, and 1 year after the diagnosis of renal amyloidosis, surgical excision of the abdominal mass was performed. Whereas her clinical symptoms and other laboratory findings rapidly improved, the proteinuria took 18 months to disappear. A second renal biopsy, performed 30 months after surgical resection, showed persistence of the amyloid deposits in the same extent. However, electron microscopy revealed subtle reparative phenomena at the epithelial site of the basement membrane. We conclude that proteinuria associated with amyloidosis does not only depend on structural damage and that the new synthesized segment of basement membrane observed by us probably represents a mechanism of repair and the start of a long healing process.     

Kimura's disease of parotid gland presenting as solitary parotid swelling

BACKGROUND: Kimura's disease is a chronic inflammatory disorder of unknown etiology commonly seen among orientals and characterized histologically by lymphatic follicles, vascular proliferation, and marked eosinophilic infiltration. It has a predilection for the head and neck region. The lesion is benign but can be mistaken to be a malignant lesion. METHODS: Between January 1987 and December 1999, eight cases of Kimura's disease were treated at the Department of Plastic Surgery, Hospital Kuala Lumpur. All the patients underwent surgical excision followed by initial high-dose steroid therapy and low-dose maintenance. RESULTS: All the patients are men, seven belong to the Malay race and one is Chinese. The patients' ages at diagnosis are 18 to 46 years. All were initially seen with swelling in the parotid gland, ranging from 1 year to 20 years' duration. Pruritus of the overlying skin was seen in seven patients. Treatment by surgical excision and steroid therapy has led to no recurrences. CONCLUSION: Kimura's disease of parotid gland is rare. It is a chronic inflammatory condition also called eosinophilic hyperplastic lymphogranuloma. Treatment by surgical excision and steroid therapy has led to no recurrences.     

Intravenous methylprednisolone in idiopathic childhood nephrotic syndrome

The aim of our study was to determine the clinical course of children with idiopathic childhood nephrotic syndrome (ICNS) who received intravenous methylprednisolone (ivMP) following failure to achieve remission with standard oral prednisolone therapy. This study was designed as a retrospective case record review from 1993 to 2007. Sixteen children received ivMP over the 15-year study period, of whom ten responded, achieving clinical remission. The remaining six children with steroid resistant nephrotic syndrome (SRNS) underwent biopsy [four focal segmental glomerulosclerosis (FSGS), two minimal change disease (MCD)]. Three responders developed late secondary steroid resistance (two FSGS, one MCD). At the latest follow-up (mean 6.7 years), three of the ten ivMP responders and none (0/6) of the children with SRNS had heavy proteinuria and chronic kidney disease (CKD) stage 3-5. The remaining 13 children demonstrated significant steroid dependency but had achieved stable remission following cyclophosphamide and/or ciclosporin therapy. The majority of children with ICNS who do not respond to 4 weeks of daily prednisolone therapy will enter remission following three to five doses of ivMP, thus avoiding a renal biopsy at initial presentation. These children are likely to develop steroid dependency, and the majority will require treatment with alkylating agents and/or ciclosporin to maintain remission. The requirement for ivMP in this setting appears to be associated with a risk of developing CKD in the longer term.     

End-stage renal failure in a child with X-linked ichthyosis

We describe an 8-year-old boy who presented with steroid-resistant nephrotic syndrome (SRNS) associated with X-linked ichthyosis (XLI). At birth, the patient exhibited scaly skin, cryptorchidism, and steroid sulfatase (STS) deficiency. DNA analysis showed deletion of exons 1-10 of the STS gene. Proteinuria developed at 6 years and was resistant to steroid therapy. Kidney biopsy findings prior to steroid therapy were compatible with minimal change nephrotic syndrome. By immunofluorescence, glomerular basement membranes exhibited diffuse linear staining for the alpha5 chain of collagen IV, making X-linked Alport syndrome an unlikely explanation for the association of SRNS and ichthyosis. Despite immunosuppressive therapy together with oral prednisolone, no clinical response was achieved. He rapidly reached end-stage renal failure and finally underwent renal transplantation. We propose that SRNS should be considered as one of the highly variable phenotypes associated with XLI.     

Strongyloidiasis as a possible cause of nephrotic syndrome

Chronic strongyloidiasis is a mild disease and has never been reported to be associated with nephrotic syndrome. Disseminated strongyloidiasis is known to have high mortality, but it frequently is not diagnosed until autopsy. We report a patient with nephrotic syndrome developing disseminated strongyloidiasis after steroid therapy. The findings in renal biopsy, the time course of the development, and resolution of nephrotic syndrome after thiabendazole treatment suggested a possible causal relationship between chronic strongyloidiasis and nephrotic syndrome. The case also demonstrated the importance of early diagnosis in disseminated strongyloidiasis and the good clinical outcome of early treatment before the development of organ failure.     

Mesangio-proliferative glomerulonephritis associated with Kimura's disease

A 16-year-old Saudi male with angiolymphoid hyperplasia and eosinophilia (Kimura's disease) for six years developed nephrotic syndrome. Renal biopsy revealed mesangio-proliferative glomerulonephritis. The patient was treated with prednisone which resulted in complete remission. This case is unique as it occurred in a Saudi patient and responded well to therapy.     

A case of focal segmental glomerulosclerosis(FSGS) complicated with chronic hepatitis B and treated with steroid and LDL apheresis

A 52-year-old man was admitted to our hospital because of nephrotic syndrome. He had been monitored at our outpatient clinic for chronic hepatitis B, and had experienced histologically proven minimal change nephrotic syndrome at the ages of 40 and 51 years. Because of HBsAg positivity in his serum, steroid therapy was withheld in his earlier episodes and he recovered from nephrotic syndrome spontaneously. However, in the most recent episode the nephrotic syndrome was found difficult to control and the findings of renal biopsy showed FSGS, which is not expected in HBV-associated nephropathy. Finally, prednisolone was administered at the dose of 40 mg/day for four weeks, after which the dose was tapered. LDL apheresis was also administered three times because of the patient's incomplete response to prednisolone. His proteinuria was reduced from > 10 g/day to < 1 g/day, but the ALT levels and HBsAg titer increased. With stronger neominophagen C induction and very careful tapering of glucocorticoid, ALT levels and the HBsAg titer decreased. During the two-year period since the induction of glucocorticoid therapy, urinary protein excretion has been maintained at less than 1 g/gcr, and ALT levels and HBsAg titer have not increased. We conclude that attention must be paid to dose modification of steroid therapy and strategies without immunosuppressive agents such as LDL apheresis should be considered in the case of treatment of nephrotic syndrome with HB virus.     

Angioimmunoblastic T-cell lymphoma and membranous nephropathy: a still unreported association

A 21-year-old man with lymphadenopathy and Coombs-positive hemolytic anemia had been treated with steroid maintenance therapy. He developed nephrotic syndrome with size increase of lymphadenopathy. Lymph node examination disclosed angioimmunoblastic T-cell lymphoma (AITL). Light microscopy of a renal biopsy specimen showed typical features of membranous nephropathy (MN), such as bubbling appearance and spike formation. Immunofluorescence studies revealed no significant deposition of immunoglobulins. Electron microscopy showed sparse degenerative materials on the epithelial side of the glomerular basement membranes, with intervening spikes. These unique histological findings suggested secondary MN. High-dose steroid therapy followed by six courses of cyclophosphamide, doxorubicin, vincristine, and prednisolone (CHOP) therapy improved his symptoms. One-year follow-up revealed the patient in good health without any signs of relapse. Glomerular manifestations have rarely been reported in association with AITL. To our knowledge, this is the first reported case of nephrotic syndrome due to MN associated with AITL.     

Younger onset myeloperoxidase-specific antineutrophil cytoplasmic antibody- (MPO-ANCA) related glomerulonephritis accompanied with nephrotic syndrome

It is known that nephrotic syndrome rarely accompanies myeloperoxidase-specific antineutrophil cytoplasmic antibody- (MPO-ANCA) related glomerulonephritis. We present a case of younger onset MPO-ANCA-related glomerulonephritis accompanied with nephrotic syndrome in a female patient. It was diagnosed through the renal biopsy and the detection of a high titer of MPO-ANCA and steroid therapy (intravenous steroid pulse therapy and oral administration), anticoagulant therapy and antiplatelet therapy were initiated. Since her nephrotic syndrome persisted in spite of the decrease of MPO-ANCA, we conducted a second renal biopsy. We found active necrotizing crescentic glomerulonephritis with a small deposition of immunoglobulin and fibrinogen on the glomeruli. To suppress her disease activity, we administered second steroid-pulse therapy and MPO-ANCA titer disappeared. However, as her nephrotic syndrome, which was accompanied by severe hyperlipidemia, persisted, we tried to treat her using low-density lipoprotein (LDL) apheresis. It was effective temporarily, but she finally fell into end-stage renal failure. We discuss here the possibility of double nephropathy by considering her clinical and renal pathologic features.     

Tacrolimus-induced HUS: an unusual cause of acute renal failure in nephrotic syndrome

Acute renal failure (ARF) is an uncommon complication in children with nephrotic syndrome. We report here the case of a 10-year-old male child with primary steroid-resistant nephrotic syndrome who was non-responsive to steroids and cyclophosphamide. A kidney biopsy revealed that he had focal segmental glomerulosclerosis. His treatment was initiated with tacrolimus (dose of 0.15 mg/kg/day) in two divided doses along with prednisolone 60 mg/m²/daily. After 1 month of treatment, he was diagnosed as having acute renal failure secondary to HUS. This was postulated to be due to the tacrolimus therapy, which was withdrawn. Two weeks after stopping the adminsitration of tacrolimus, his urine output improved, and the hemoglobin and serum creatinine normalized. Thus, tacrolimus-induced HUS is a rare cause of ARF in nephrotic syndrome. With the increasing use of tacrolimus in steroid-resistant nephrotic syndrome, the treating physicians need to be aware of this rare, but potentially life-threatening side effect.     

Nephrotic syndrome associated with interferon-β-1b therapy for multiple sclerosis

A 43-year-old woman with multiple sclerosis (MS) had nephrotic syndrome 21 months after starting treatment with interferon (IFN)-β-1b (subcutaneous administration). She had taken no drug except for the IFN-β-1b. Because nephrotic syndrome may be induced by IFN therapy, the IFN was stopped. Percutaneous renal biopsy revealed that she had minimal change nephrotic syndrome. As nephrotic-range proteinuria, hypoalbuminemia, and general edema were worsening even 2 weeks after cessation of the drug, oral corticosteroid therapy (prednisolone 40 mg/day) was started. The nephrotic syndrome was treated successfully with prednisolone. The dosage of prednisolone was tapered, without a relapse, and then the corticosteroid therapy was stopped. IFN-β-1b therapy was then resumed, and the patient is in remission for both nephrotic syndrome and MS. Though proteinuria and nephrotic syndrome is a rare adverse effect of IFN-β-1b therapy, physicians treating MS patients with this agent should pay careful attention to new clinical symptoms and laboratory findings.     

Minimal-change nephrotic syndrome associated with subcutaneous eosinophilic lymphoid granuloma (Kimura's disease)

A 29-year-old Japanese male with a 19-year history of subcutaneous eosinophilic lymphoid granuloma (Kimura's disease) was referred to the Nephrology Service of the Nihon University Hospital for evaluation of edema and massive proteinuria. The renal biopsy disclosed minimal glomerular lesions. In this paper a case of nephrotic syndrome associated with eosinophilic lymphoid granuloma is reported.     

Nephrotic syndrome associated with Kimura's disease

Kimura's disease consists of lesions that appear as single or multiple red-brown papules or as subcutaneous nodules with a predilection for the head and neck region. Although it principally affects the skin and soft tissues, there is a high prevalence of associated renal disease. We report a case of nephrotic syndrome associated with Kimura's disease. Our patient is distinctive in that his disease first manifested while residing in the Western hemisphere; the renal disease was characterized as mesangial proliferative glomerulonephritis with renal impairment, and his nephrotic syndrome remitted with standard doses of prednisone.     

Renal failure and nephrotic syndrome associated with sulindac

Four elderly patients developed nephrotic syndrome while receiving sulindac. Sulindac treatment had commenced 4-12 months prior to presentation with the nephrotic syndrome. Two patients also developed oliguric renal failure. Renal biopsy in one showed minimal change nephropathy and in three cases membranous nephropathy. Interstitial nephritis was present on renal biopsy in all cases. The nephrotic syndrome and renal failure resolved in all cases after withdrawal of sulindac. Two patients received steroid therapy and improvement in renal function and disappearance of proteinuria seemed to be temporarily related to steroid therapy in both cases. Despite the fact that sulindac is less likely to cause renal failure due to inhibition of renal prostaglandin secretion this report shows that sulindac treatment can be associated with renal failure and the nephrotic syndrome.     

自身免疫性甲状腺疾病相关肾病综合征一例报告及文献复习

目的　探讨自身免疫性甲状腺疾病相关肾病综合征的临床与病理特点。方法　通过一例病人的诊治过程结合复习文献对该病的诊治经过、肾活检病理综合分析。结果　自身免疫性甲状腺疾病相关肾病综合征临床并不罕见 ,因甲状腺疾病与肾病综合征发病间隔不同、临床表现不典型 ,极易被忽视 ;肾脏病理多表现为膜性肾病 ;加强甲状腺疾病的治疗并应用激素或细胞毒药物对本病治疗有效。结论　自身免疫性甲状腺疾病可能是继发性肾病综合征的原因之一 ,应引起临床医师的重视。     

Minimal change nephrotic syndrome in association with strongyloidiasis

Although parasitic infections have been known to be associated with immune complex-mediated glomerular lesions, strongyloidiasis-related glomerulopathy has not been well documented. We report a patient with delayed-recognized disseminated strongyloidiasis who developed nephrotic syndrome 3 months after the beginning of the manifestations related to strongyloidiasis. A kidney biopsy showed minimal change disease. We treated strongyloidiasis and hesitated to give steroid therapy for the treatment of minimal change nephrotic syndrome (MCNS) because of the risk of aggravation of Strongyloides stercoralis infection. Surprisingly, resolution of heavy proteinuria occurred after anthelmintic therapy with ivermectin. This case suggests a possible causal relationship between S. stercoralis infection and MCNS. In addition, a review of another 4 cases previously reported in the literature demonstrates the importance of detecting underlying S. stercoralis infection in patients with nephrotic syndrome since steroid therapy can cause hyperinfection or disseminated strongyloidiasis, and which may lead to fatal outcome.     

A case of minimal change nephrotic syndrome manifesting acute renal failure in the course of systemic lupus erythematosus

A 51-year-old woman with systematic lupus erythematosus(SLE) associated with minimal change nephrotic syndrome(MCNS) is described. The patient was diagnosed as SLE at 33 years of age. After steroid therapy for two years, the patient's course was uneventful without therapy until June 2000, when facial erythema and facial, pretibial edema developed. On admission, proteinuria and renal dysfunction were detected. Subsequently, oliguric acute renal failure developed and hemodialysis was started. Laboratory examination showed no significant change in complements and anti ds-DNA antibody levels. Renal biopsy revealed minor glomerular abnormalities without the deposition of immune complexes. Electron microscopic examination showed foot process fusion and a vacuolar change in glomerular epithelial cells. The diagnosis of MCNS was made and administration of steroid(40 mg/day) was started. Urine volume and renal function improved after 2 weeks, and nephrotic syndrome remitted completely after 5 weeks. Although the association of SLE and MCNS is rare, the findings suggest that in the course of SLE manifesting acute ranal failure, not only lupus nephritis, but also the complication of MCNS should be considered.     

Complete remission of minimal-change nephrotic syndrome induced by apheresis monotherapy

We report a case of a 17-year-old male with relapse of minimal-change nephrotic syndrome (MCNS), in whom apheresis monotherapy without steroids or immunosuppressants resulted in complete remission. The patient initially developed nephrotic syndrome in February 1998. The first renal biopsy confirmed the diagnosis of MCNS. The patient was also found to be a carrier of hepatitis B virus. Steroid therapy was started with oral prednisolone 60 mg/day. Complete remission was achieved in 3 months, and the steroid treatment was tapered off in May 2001. During the steroid tapering, temporal exacerbation of liver function was noted. In July 2002, the patient was admitted to our hospital again due to relapse of nephrotic syndrome. Second biopsy reconfirmed the diagnosis of MCNS. Since the serum titer of HBV was elevated, apheresis monotherapy was selected to avoid the risk of steroid-induced fulminant hepatitis. Four sessions of low-density lipoprotein apheresis (LDL-A) and 5 sessions of double-filtration plasmapheresis (DFPP) reduced the proteinuria from 9.2 g/day to 0.2 g/day over 38 days without any additional medication. Proteinuria remained suppressed below 0.2 g/day for more than 12 months and no exacerbation of liver function was observed up to the final follow-up in September 2003. The present case suggested the potential of apheresis monotherapy to induce and maintain complete remission of MCNS and an important role of circulating factors in the pathogenesis of MCNS.