A case of steroid-resistant nephrotic syndrome associated with systemic lupus erythematosus

Ito, I; Nishida, Masashi; Morioka, Shigefumi; Nozaki, Takako; Imamura, Toshihiko; Morimoto, Akira; Akioka, Shinji; Sugimoto, Takeshi; Hamaoka, Kenji; Hosoi, Hajime

doi:10.1177/0961203311432740

Cited by 13 publications

(13 citation statements)

References 10 publications

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“…Additional immunosuppressive agents including CTX, 14 AZA, 9,14 MMF, 3,31,32 cyclosporine (CsA), 12,15 and anti-CD20 monoclonal antibody, 33 as well as double filtration plasmapheresis 23 were reported to be combined with glucocorticoid for inducing remission of lupus podocytopathy. However, all of these publications were case reports and most were indicated in patients who were refractory to glucocorticoid.…”

Section: Discussionmentioning

confidence: 99%

Glucocorticoid with or without additional immunosuppressant therapy for patients with lupus podocytopathy: a retrospective single-center study

Chen

Bao

et al. 2015

Lupus

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Lupus podocytopathy is a newly recognized class of lupus nephritis characterized by extensive glomerular foot process effacement without capillary wall immune deposits. The treatment response and relapse of glucocorticoid with or without additional immunosuppressive agents has not been well investigated. In this study, 50 patients with lupus podocytopathy were included and received glucocorticoid alone (glucocorticoid monotherapy) or glucocorticoid plus additional immunosuppressive agents (combination therapy) for their induction or maintenance treatment regimens. The treatment response and relapse rate in the two groups were respectively analyzed. We found that the induction treatment with glucocorticoid monotherapy and combination therapy led to remission in 47 patients (94.0%) at 12 weeks treatment, with complete remission (CR) occurring in 38 patients (76.0%). The CR rate compared between glucocorticoid monotherapy and combination therapy showed no difference (76.7% vs 75.0%, p = 0.9), the median time to CR was four weeks (range: 2.0-6.0 weeks) in glucocorticoid monotherapy and 8.0 weeks (range: 3.7-12.0 weeks) in combination therapy (p = 0.076). Twenty-seven of 47 patients (57.4%) relapsed during maintenance, the relapse rate was much higher in the glucocorticoid monotherapy group than in the combination therapy group (89.5% vs 35.7%, p < 0.001), regardless of the induction regimens being glucocorticoid monotherapy or combination therapy. No patient developed end stage renal disease or died during follow-up for 6-125 months (median 62 months). In conclusion, the remission of lupus podocytopathy could be induced by glucocorticoid monotherapy or glucocorticoid plus other immunosuppressive agents, while the remission should be maintained by the combination regimen.

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Section: Discussionmentioning

confidence: 99%

Glucocorticoid with or without additional immunosuppressant therapy for patients with lupus podocytopathy: a retrospective single-center study

Chen

Bao

et al. 2015

Lupus

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“…Simple criteria to diagnose lupus podocytopathy are (1) lupus patient with nephrotic syndrome, (2) diffuse and severe foot process effacement and (3) the absence of subendothelial or subepithelial immune deposits [20]. Up till now, the frequency, prognosis and treatment of LP are not well established in p-SLE except for one case report of Ito et al [25] who reported an 11-year lupus girl who developed steroid-resistant nephrotic syndrome (SRNS) at disease onset and her histological findings were consistent with LP. LP represents 8.93% of renal pathology in our patients, which is consistent with other reports [6,[17][18][19].…”

Section: Discussionmentioning

confidence: 99%

Is podocytopathy another image of renal affection in p-SLE?

Abdelnabi

2021

Pediatr Rheumatol

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Background Lupus podocytopathy (LP) is a renal affection described in systemic lupus erythematosus (SLE) patients with nephrotic range proteinuria, characterized by diffuse foot process effacement without immune deposits and glomerular proliferation. This study describes LP, its pathological features and outcomes of pediatric (p-SLE) patients in comparison to the usual lupus nephritis (LN) cases. Methodology A retrospective cohort study conducted on a 10-year registration (2010–2019) of 140 p-SLE patients at the Pediatric Department, Tanta University. Histopathological analysis with light microscopy (LM) and immunofluorescence (IF) of all renal biopsies were evaluated according to the International Society of Nephrology Renal Pathology Society (ISN/RPS) grading system. In addition, some biopsies were examined with electron microscopy (EM). Results Eighty-six p-SLE cases (61.4%) had renal involvement; seventy-nine biopsies (91.86%) of them met the classification criteria of LN as defined by ISN/RPS system. Five biopsies were normal (MCD) and two showed focal segmental sclerosis (FSGN) that did not meet any known classification of LN. Hence, they were reevaluated using EM that revealed diffuse effaced podocytes without glomerular sub-epithelial, endocapillary or basement membrane immune deposits, and were classified as having lupus podocytopathy, representing (8.14%) of all LN biopsies. Those seven cases showed good response to steroids with a complete remission duration of 3.40 ± 1.95 weeks. However, some case had 1–3 relapses during the duration of follow up. Conclusions LP is a spectrum of p-SLE, not an association as it is related to disease activity and its initial presentation.

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“…Nephrotic syndrome is usually associated with diffuse or membranous. LN [ 9 ]. However, membranous LN or mesangial proliferative LN is rare in children.…”

Section: Discussionmentioning

confidence: 99%

Atrial thrombus as a complication of SLE and APS in an 8-year-old child

Yan

2020

Pediatr Rheumatol

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Background Systemic lupus erythematosus (SLE) is an autoimmune disease involving multiple systems with various clinical manifestations. Renal involvement is common, but intracardiac thrombus is rarely reported as a complication of antiphospholipid syndrome (APS, also known as anticardiolipin syndrome). Anticoagulant therapy is the first-line treatment, and surgery is performed in severe cases. We report a case to improve clinicians’ understanding of disease diagnosis. Case presentation An 8-year-old girl was admitted to our hospital because of left costal pain, hematuria and fever. She had obvious edema occult blood 3+, urinary protein 3.2 g/24 h, albumin 17.6 g/L, and total cholesterol 7.21 mmol/L, consistent with a diagnosis of nephrotic syndrome. We continued to track the etiology of nephrotic syndrome and performed a renal biopsy, showing dsDNA 1:10 positivity, low C3, low platelets and hemoglobin, anticardiolipin IgM 12 U/ml, anti-β2-glycoprotein I (β2GPI) 223 U/ml; renal pathology suggested lupus nephritis (LN), and the patient was ultimately diagnosed with SLE, secondary APS and LN. The patient was treated with hormones and immunosuppressants. Sixteen weeks later, her urinary protein was 1+, and the quantity of urine protein was less than 0.5 g/d. Echocardiography showed that the mass in the right atrium was thrombotic. Heparin anticoagulant therapy was effective. Conclusion SLE can involve multiple systems and various complications. Thrombus in the right atrium is a rare complication of APS. Early diagnosis and treatment are key to improving the prognosis of children.

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A case of steroid-resistant nephrotic syndrome associated with systemic lupus erythematosus

Cited by 13 publications

References 10 publications

Glucocorticoid with or without additional immunosuppressant therapy for patients with lupus podocytopathy: a retrospective single-center study

Glucocorticoid with or without additional immunosuppressant therapy for patients with lupus podocytopathy: a retrospective single-center study

Is podocytopathy another image of renal affection in p-SLE?

Atrial thrombus as a complication of SLE and APS in an 8-year-old child

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