Steroid-Resistant Nephrotic Syndrome Secondary to Primary Focal Segmental Glomerulosclerosis and Smoldering Multiple Myeloma

Shah, RR; Shah, Nishi; Shah, Arun Kumar; Mehta, Ankit

doi:10.1080/08998280.2014.11929039

Cited by 3 publications

(4 citation statements)

References 8 publications

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“…The results of our study are similar to those of previous reports. 7 The results indicate that LT3S is associated with the severity of the disease in NS patients, suggesting that LT3S may be a self-protective mechanism under the disease state. By reducing the concentration of thyroid hormone, protein catabolism is reduced, resulting in a decrease in the basal metabolic rate and reduced energy consumption.…”

Section: Discussionmentioning

confidence: 79%

Low triiodothyronine syndrome is associated with platelet function in patients with nephrotic syndrome

Guo

Chen

et al. 2019

Rev. Assoc. Med. Bras.

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SUMMARY OBJECTIVE The objective of this study was to investigate the effects of low triiodothyronine syndrome (LT3S) on platelet function and clotting factors in patients with nephrotic syndrome(NS). METHODS Patients with primary nephrotic syndrome were divided into two groups, normal thyroid function (group A) and LT3S (group B), based on whether they had LT3S or not. Healthy subjects were selected as the control group (group C). Blood coagulation function was detected in each group. The platelet activation function (CD62P, CD63) was determined by flow cytometry. The platelet aggregation rate was detected by an optical method using adenosine diphosphate and arachidonic acid as inducers. RESULTS The proportion of primary nephrotic syndrome with LT3S was 23.2% (69/298). Compared with group C, group A had higher CD62P and PAgTADP, and group B had higher CD62P, CD63, PAgTAA, and PAgTADP; the difference was statistically significant (all P < 0.05). There was no significant difference in renal pathology between group A and group B (X2 = 4.957, P = 0.421). Compared with group A, the 24-hour urine protein, CD63, PAgTAA, and PAgTADP were higher in group B, and APTT and Alb were lower. The difference was statistically significant (P < 0.05). Logistic regression analysis showed that LT3S was associated with CD36 (OR: 3.516; 95% CI: 1.742~8.186; P = 0.004) and PAgTAA (OR: 0.442; 95% CI: 1.001~1.251; P = 0.037). CONCLUSION NS patients are prone to LT3S. Patients with LT3S may have abnormal platelet activation and increase of platelet aggregation.

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Section: Discussionmentioning

confidence: 79%

Low triiodothyronine syndrome is associated with platelet function in patients with nephrotic syndrome

Guo

Chen

et al. 2019

Rev. Assoc. Med. Bras.

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“…Of these studies, three suggested that there was little or no correlation between FSGS and plasma cell proliferative disorders. In a study by Shah et al ( 12 ), the NS of the patient was not resolved by hormone therapy, however, the patient's smoldering MM did improve. Paueksakon et al ( 1 ) identified that 13 out of 87 (14.9%) patients with MGUS and renal damage had FSGS-like lesions, and thus, the FSGS was not considered to be primary.…”

Section: Discussionmentioning

confidence: 89%

“…All of these factors support the notion that the two diseases are associated. While the patients in a study by Shah et al ( 12 ) exhibited a reduction in serum monoclonal protein and light chain protein levels following the use of hormones for 3 months, the proteinuria did not resolve. It is possible that the improvement in secondary FSGS required a longer time than that required to control the primary disease.…”

Section: Discussionmentioning

confidence: 91%

“…Of these studies, seven demonstrated an association between FSGS and plasma cell proliferative disorders ( 2 , 5 – 10 ). However, the other two studies indicated little or no correlation between the two conditions ( 11 , 12 ). As FSGS and MM occur infrequently in the same patient and there are few relevant previous studies, the association between them remains unclear.…”

Section: Introductionmentioning

confidence: 93%

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Multiple myeloma with a previous diagnosis of focal segmental glomerulosclerosis: A case report and review of the literature

Dong¹,

Xing²,

Wang³

et al. 2015

Oncology Letters

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The presentation of focal segmental glomerulosclerosis (FSGS) and multiple myeloma (MM), either together or in succession, is extremely rare. Only nine studies have previously reported this poorly understood association. The present study reports the case of a 45-year-old male with FSGS that was diagnosed by a renal biopsy performed for nephrotic syndrome (NS). The patient was admitted to the Chinese People's Liberation Army General Hospital one year later with a fever, anemia, unresolved NS and renal insufficiency. The patient was diagnosed with MM and a renal biopsy was repeated, the results of which suggested renal amyloidosis. The MM was treated with three cycles of vincristine, doxorubicin and dexamethasone chemotherapy. A review of the literature indicated that monoclonal gammopathy may lead to FSGS. It suggested that FSGS patients who are >40 years old should be routinely screened for plasma cell proliferative disorders to guide the treatment, determine a prognosis, achieve primary disease remission and avoid end-stage renal disease.

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Podocytopathy in patients with monoclonal gammopathy: three patients and literature review

Ribas

Puche

Gimeno

et al. 2021

Clinical Kidney Journal

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Background Renal manifestations of monoclonal gammopathies are of increasing interest among nephrologists. Typical manifestations include light chain cast nephropathy, amyloidosis, or renal damage mediated by monoclonal immunoglobulin deposition. Podocytopathies in the setting of an underlying monoclonal gammopathy constitute a rare manifestation of these diseases and, although being described in the literature, remains a challenge since most data derive from case reports. Methods A retrospective review of the clinical data of Hospital del Mar and Hospital VallHebron was performed, to identify patients with minimal change disease (MCD) or focal and segmental glomerulosclerosis (FSGS) in the setting of neoplasms that produce monoclonal protein (M protein). Additionally, a literature review on this topic was performed. This study aims to describe the clinical characteristics and outcomes of these patients. Results Three patients were identified to have podocytopathy and monoclonal gammopathy between the years 2013–2020. All three were > 65 years old, and males. Two patients were diagnosed with MCD and one patient was diagnosed with FSGS. All patients underwent a kidney biopsy and light and electron microscopic studies were performed. The underlying causes of monoclonal gammopathy were multiple myeloma (MM) in two cases, and Waldeström macroglobulinemia (WM) in one case. Two patients developed nephrotic syndrome during the follow-up. All patients were under active hematological treatment. One patient presenteda complete remission of proteinuria whereas the other two presented a partial remission. Conclusions Podocytopathies may infrequently be found in patients with monoclonal gammopathies. Patients with overt glomerular proteinuria and hematologic disorders with M protein shall undergo a kidney biopsy to promptly diagnose and specify a prognosis. In addition, further study on this matter must be done to understand the pathophysiology and treat these patients appropriately.

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Steroid-Resistant Nephrotic Syndrome Secondary to Primary Focal Segmental Glomerulosclerosis and Smoldering Multiple Myeloma

Abstract: We present a patient with steroid-resistant nephrotic syndrome due to focal segmental glomerulosclerosis along with smoldering multiple myeloma. While investigating the cause of proteinuria, a monoclonal gammopathy with a negative kidney biopsy for myeloma-related pathology was discovered.

Cited by 3 publications

References 8 publications

Low triiodothyronine syndrome is associated with platelet function in patients with nephrotic syndrome

Low triiodothyronine syndrome is associated with platelet function in patients with nephrotic syndrome

Multiple myeloma with a previous diagnosis of focal segmental glomerulosclerosis: A case report and review of the literature

Podocytopathy in patients with monoclonal gammopathy: three patients and literature review

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