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Article ; Online: Do we really need cyclophosphamide for lupus nephritis?

Wenderfer, Scott E / Cooper, Jennifer C

2024

Abstract: A 14-year-old patient presents with hematuria and proteinuria. Clinical evaluation reveals a positive anti-nuclear antibody titer, positive anti-double stranded DNA antibody and hypocomplementemia. Systemic lupus erythematosus (SLE) is diagnosed based on ...

Abstract	A 14-year-old patient presents with hematuria and proteinuria. Clinical evaluation reveals a positive anti-nuclear antibody titer, positive anti-double stranded DNA antibody and hypocomplementemia. Systemic lupus erythematosus (SLE) is diagnosed based on the 2019 EULAR/ACR (European League Against Rheumatism/American College of Rheumatology) classification criteria (Aringer et al. Arthritis Rheumatol 71:1400-1412, 2019). A kidney biopsy is performed that confirms the presence of immune complex glomerulonephritis, ISN-RPS (International Society of Nephrology/Renal Pathology Society) class IV (Bajema et al. Kidney Int 93:789-796, 2018). According to the latest clinical practice guidelines (Rovin et al. Kidney Int 100:753-779, 2021; Fanouriakis et al. Ann Rheum Dis 83:15-29, 2023), there are alternatives to treating this patient with cyclophosphamide. But what if this patient also presented with oliguria and volume overload requiring intensive care and dialysis? What if this patient also presented with altered mental status and seizures, and was diagnosed with neuropsychiatric lupus? What if this patient was also diagnosed with a pulmonary hemorrhage and respiratory failure? The clinical practice guidelines do not address these scenarios that are not uncommon in patients with SLE. Moreover, in some countries worldwide, patients do not have the privilege of access to biologics or more expensive alternatives. The purpose of this review is to evaluate the contemporary options for initial treatment of nephritis in patients with SLE.
Language	English
Publishing date	2024-04-12
Publishing country	Germany
Document type	Journal Article
ZDB-ID	631932-4
ISSN	1432-198X ; 0931-041X
ISSN (online)	1432-198X
ISSN	0931-041X
DOI	10.1007/s00467-024-06367-9
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Article ; Online: How common is chronic kidney disease in children with lupus nephritis?

Wenderfer, Scott E / Orjuela, Alvaro / Dionne, Janis

Pediatric nephrology (Berlin, Germany)

2022 Volume 38, Issue 6, Page(s) 1701–1705

MeSH term(s)	Humans ; Child ; Lupus Nephritis/complications ; Lupus Nephritis/diagnosis ; Renal Insufficiency, Chronic/complications ; Renal Insufficiency, Chronic/diagnosis ; Kidney ; Kidney Failure, Chronic ; Lupus Erythematosus, Systemic
Language	English
Publishing date	2022-12-16
Publishing country	Germany
Document type	Editorial
ZDB-ID	631932-4
ISSN	1432-198X ; 0931-041X
ISSN (online)	1432-198X
ISSN	0931-041X
DOI	10.1007/s00467-022-05848-z
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Zs.A 2196: Show issues

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Article ; Online: Viral-associated glomerulopathies in children.

Wenderfer, Scott E

Pediatric nephrology (Berlin, Germany)

2015 Volume 30, Issue 11, Page(s) 1929–1938

Abstract: Viral infections associate temporally with the onset of many glomerular diseases, particularly in children. In other cases of glomerulonephritis, when infection is clinically silent, viral syndromes can still be implicated as a trigger. However, strong ... ...

Abstract	Viral infections associate temporally with the onset of many glomerular diseases, particularly in children. In other cases of glomerulonephritis, when infection is clinically silent, viral syndromes can still be implicated as a trigger. However, strong evidence for viral causality in most glomerular disease is still lacking. While numerous case reports in children document the occurrence of specific forms of glomerular disease after seroconversion to a wide range of viruses, relatively few reports provide pathologic evidence of viral infection associated with glomerular lesions on kidney biopsy. Strong associations between hepatitis viruses and glomerular injury have been acknowledged in adults, but hepatitis C virus appears not to be an etiology in children. In the context of treating glomerular diseases, when diagnosed, the treatment of hepatitis B virus, cytomegalovirus and human immunodeficiency virus in children with membranoproliferative, membranous and collapsing glomerulopathy plays an important role. Otherwise, there is no evidence suggesting that the identification of a viral infection in a child with glomerulopathy should change the management of the infection or the glomerulonephritis. Therefore, additional research into this topic is very much needed.
MeSH term(s)	Child ; Glomerulonephritis/virology ; Humans ; Virus Diseases/complications
Keywords	covid19
Language	English
Publishing date	2015-11
Publishing country	Germany
Document type	Journal Article ; Review
ZDB-ID	631932-4
ISSN	1432-198X ; 0931-041X
ISSN (online)	1432-198X
ISSN	0931-041X
DOI	10.1007/s00467-015-3057-y
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Article ; Online: Evolving Epidemiology of Pediatric Glomerular Disease.

Rheault, Michelle N / Wenderfer, Scott E

Clinical journal of the American Society of Nephrology : CJASN

2018 Volume 13, Issue 7, Page(s) 977–978

MeSH term(s)	Biopsy ; Child ; China ; Cross-Sectional Studies ; Humans ; Kidney Glomerulus ; Surveys and Questionnaires
Language	English
Publishing date	2018-06-18
Publishing country	United States
Document type	Editorial ; Comment
ZDB-ID	2226665-3
ISSN	1555-905X ; 1555-9041
ISSN (online)	1555-905X
ISSN	1555-9041
DOI	10.2215/CJN.06220518
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Zs.A 6584: Show issues

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Article ; Online: 50 Years Ago in The Journal of Pediatrics: The Hemolytic-Uremic Syndrome: Renal Status of 76 Patients at Long-Term Follow-Up.

Alge, Joseph L / Wenderfer, Scott E

The Journal of pediatrics

2018 Volume 197, Page(s) 185

MeSH term(s)	Hemolytic-Uremic Syndrome/complications ; Hemolytic-Uremic Syndrome/history ; History, 20th Century ; Humans ; Kidney/physiopathology ; Pediatrics/history
Language	English
Publishing date	2018-05-25
Publishing country	United States
Document type	Historical Article ; Journal Article
ZDB-ID	3102-1
ISSN	1097-6833 ; 0022-3476
ISSN (online)	1097-6833
ISSN	0022-3476
DOI	10.1016/j.jpeds.2017.12.030
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Um IV Zs.116: Show issues

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Article ; Online: Lupus Nephritis.

Wenderfer, Scott E / Eldin, Karen W

Pediatric clinics of North America

2018 Volume 66, Issue 1, Page(s) 87–99

Abstract: Childhood-onset systemic lupus erythematosus (SLE) is a subset of SLE with an onset before 18 years of age. Patients with early onset SLE tend to have a greater genetic component to their disease cause, more multisystemic involvement, and a more severe ... ...

Abstract	Childhood-onset systemic lupus erythematosus (SLE) is a subset of SLE with an onset before 18 years of age. Patients with early onset SLE tend to have a greater genetic component to their disease cause, more multisystemic involvement, and a more severe disease course, which includes greater risks for developing nephritis and end-stage kidney disease. Five- and 10-year mortality is lower than in adult-onset SLE. Although patient and renal survival have improved with advances in induction and maintenance immunosuppression, accumulation of irreversible damage is common. Cardiovascular and infectious complications are frequent, as are relapses during adolescence and the transition to adulthood.
MeSH term(s)	Adrenal Cortex Hormones/therapeutic use ; Biopsy ; Child ; Diagnosis, Differential ; Humans ; Immunosuppressive Agents/therapeutic use ; Lupus Nephritis/diagnosis ; Lupus Nephritis/drug therapy ; Lupus Nephritis/pathology
Chemical Substances	Adrenal Cortex Hormones ; Immunosuppressive Agents
Language	English
Publishing date	2018-09-08
Publishing country	United States
Document type	Journal Article ; Review
ZDB-ID	215711-1
ISSN	1557-8240 ; 0031-3955
ISSN (online)	1557-8240
ISSN	0031-3955
DOI	10.1016/j.pcl.2018.08.007
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Um IV Zs.147: Show issues

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Article ; Online: Real-world use and outcomes of belimumab in childhood-onset lupus: A single-center retrospective study.

Roberts, Jordan E / Burn, Cordelia / Sadun, Rebecca E / Smitherman, Emily A / Wenderfer, Scott E / Son, Mary Beth F

Lupus

2023 Volume 32, Issue 9, Page(s) 1111–1116

Abstract: Background: Studies of real-world effectiveness of belimumab in adults with systemic lupus erythematosus have shown improved disease control and decreased oral glucocorticoid use. However, belimumab use outside of clinical trial settings has not been ... ...

Abstract	Background: Studies of real-world effectiveness of belimumab in adults with systemic lupus erythematosus have shown improved disease control and decreased oral glucocorticoid use. However, belimumab use outside of clinical trial settings has not been well studied in childhood-onset systemic lupus erythematosus (cSLE). We aimed to characterize indications for belimumab use and evaluate oral glucocorticoid doses and disease activity scores in the year following belimumab initiation at a single, large pediatric rheumatology center. Methods: We included children and young adults with cSLE who received ≥ 1 dose of belimumab. Repeated measures one-way ANOVA was used to compare SLEDAI-2K scores and prednisone-equivalent daily oral glucocorticoid doses at baseline, 6 months, and 12 months after belimumab initiation for those who continued therapy for a year. Results: We identified 21 patients with cSLE who received ≥ 1 dose of belimumab. The median disease duration at belimumab initiation was 30.8 months [IQR 21.0-79.1]. At the time of belimumab initiation, 100% of patients were taking an antimalarial, 81% were on oral glucocorticoids, and 91% were on at least one conventional DMARD. Thirteen patients (62%) continued belimumab for ≥6 months and 11 (52%) for ≥12 months. Among those continuing belimumab for ≥12 months, median [IQR] oral prednisone daily doses in milligrams at baseline, 6 months, and 12 months were 12.5 [7.5-17.5], 9 [6.25-10], and 5 [5-9.5], Conclusions: In our cohort of pediatric patients with lupus and moderate disease activity treated with belimumab for ≥12 months, daily oral glucocorticoid doses were significantly lower 6 and 12 months after belimumab initiation than baseline. Use in patients with active nephritis was uncommon. Further research is needed in a large, multicenter cohort to determine the real-world effectiveness of belimumab in children and develop guidelines for use.
MeSH term(s)	Young Adult ; Humans ; Child ; Prednisone/therapeutic use ; Lupus Erythematosus, Systemic/drug therapy ; Lupus Erythematosus, Systemic/chemically induced ; Glucocorticoids/therapeutic use ; Immunosuppressive Agents/adverse effects ; Retrospective Studies ; Severity of Illness Index ; Treatment Outcome
Chemical Substances	Prednisone (VB0R961HZT) ; Glucocorticoids ; Immunosuppressive Agents ; belimumab (73B0K5S26A)
Language	English
Publishing date	2023-07-06
Publishing country	England
Document type	Multicenter Study ; Journal Article
ZDB-ID	1154407-7
ISSN	1477-0962 ; 0961-2033
ISSN (online)	1477-0962
ISSN	0961-2033
DOI	10.1177/09612033231187752
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Zs.A 3717: Show issues

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Article ; Online: Urinary CD163 is a marker of active kidney disease in childhood-onset lupus nephritis.

Inthavong, Haleigh / Vanarsa, Kamala / Castillo, Jessica / Hicks, M John / Mohan, Chandra / Wenderfer, Scott E

Rheumatology (Oxford, England)

2022 Volume 62, Issue 3, Page(s) 1335–1342

Abstract: Objective: The objective of this study was to evaluate the utility of urine CD163 for detecting disease activity in childhood-onset SLE (cSLE) patients.: Methods: Sixty consecutive pediatric patients fulfilling four or more ACR criteria for SLE and ... ...

Abstract	Objective: The objective of this study was to evaluate the utility of urine CD163 for detecting disease activity in childhood-onset SLE (cSLE) patients. Methods: Sixty consecutive pediatric patients fulfilling four or more ACR criteria for SLE and 20 healthy controls were recruited for testing of urinary CD163 using ELISA. SLE disease activity was assessed using the SLEDAI-2K. Results: Urine CD163 was significantly higher in patients with active LN than inactive SLE patients and healthy controls, with receiver operating characteristics area under the curve values ranging from 0.93 to 0.96. LN was ascertained by kidney biopsy. Levels of CD163 significantly correlated with the SLEDAI, renal SLEDAI, urinary protein excretion and C3 complement levels. Urine CD163 was also associated with high renal pathology activity index and chronicity index, correlating strongly with interstitial inflammation and interstitial fibrosis based on the examination of concurrent kidney biopsies. Conclusion: Urine CD163 emerges as a promising marker for identifying cSLE patients with active kidney disease. Longitudinal studies are warranted to validate the clinical utility of urine CD163 in tracking kidney disease activity in children with lupus.
MeSH term(s)	Child ; Humans ; Antigens, CD ; Biomarkers/urine ; Kidney/pathology ; Lupus Erythematosus, Systemic/pathology ; Lupus Nephritis/pathology
Chemical Substances	Antigens, CD ; Biomarkers ; CD163 antigen
Language	English
Publishing date	2022-08-12
Publishing country	England
Document type	Journal Article ; Research Support, N.I.H., Extramural
ZDB-ID	1464822-2
ISSN	1462-0332 ; 1462-0324
ISSN (online)	1462-0332
ISSN	1462-0324
DOI	10.1093/rheumatology/keac465
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Zs.B 240: Show issues			Location: Je nach Verfügbarkeit (siehe Angabe bei Bestand) bis Jg. 2021: Bestellungen von Artikeln über das Online-Bestellformular ab Jg. 2022: Lesesaal (EG)
Zs.MO 497: Show issues

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Article ; Online: Use of renin angiotensin aldosterone system inhibitors in children with lupus and time to glucocorticoid discontinuation.

Chang, Joyce C / Weiss, Pamela F / Xiao, Rui / Atkinson, Meredith A / Wenderfer, Scott E

Kidney international

2022 Volume 102, Issue 2, Page(s) 395–404

Abstract: There is little data to inform use of renin angiotensin aldosterone system (RAAS) inhibitors in pediatric patients with systemic lupus erythematosus (SLE). Here, we sought to characterize RAAS inhibitor use in pediatric SLE and determine whether early ... ...

Abstract	There is little data to inform use of renin angiotensin aldosterone system (RAAS) inhibitors in pediatric patients with systemic lupus erythematosus (SLE). Here, we sought to characterize RAAS inhibitor use in pediatric SLE and determine whether early RAAS inhibitor initiation among children with incident lupus nephritis is associated with decreased duration of chronic glucocorticoid exposure. A retrospective cohort study was performed of children (ages 5-18) with SLE and/or lupus nephritis in the Truven MarketScan™ Medicaid and Commercial databases (2013-2018) and estimated RAAS inhibitor use. Among incident nephritis cases, we used competing risk hazard models with inverse probability of treatment weighting to estimate the association between RAAS inhibitor initiation less than 180 days after diagnosis and time to glucocorticoid discontinuation with kidney failure as a competing event. Among 592 children with nephritis and 1407 children with non-kidney SLE, 67% and 15% ever received RAAS inhibitors, respectively. Median duration of RAAS inhibitor use among 323 incident users was 14 and 9 months in children with and without nephritis, respectively. Medicaid enrollment was independently associated with greater likelihood of RAAS inhibitor use, irrespective of nephritis. Among 158 incident nephritis cases, early RAAS inhibitor initiation was significantly associated with a faster rate of glucocorticoid discontinuation (adjusted sub-distribution hazard ratio 1.81, 95% confidence interval [1.09 - 3.00]). Thus, early initiation of RAAS inhibitors may have a role in children newly diagnosed with lupus nephritis; not only those with refractory proteinuria after induction therapy. Hence, integrated health systems data could be leveraged to confirm these findings and optimize adjunctive therapies in pediatric lupus.
MeSH term(s)	Adolescent ; Aldosterone ; Angiotensin-Converting Enzyme Inhibitors/adverse effects ; Antihypertensive Agents ; Child ; Child, Preschool ; Glucocorticoids/adverse effects ; Humans ; Lupus Erythematosus, Systemic/drug therapy ; Lupus Nephritis/drug therapy ; Renin-Angiotensin System ; Retrospective Studies
Chemical Substances	Angiotensin-Converting Enzyme Inhibitors ; Antihypertensive Agents ; Glucocorticoids ; Aldosterone (4964P6T9RB)
Language	English
Publishing date	2022-05-23
Publishing country	United States
Document type	Journal Article ; Research Support, N.I.H., Extramural
ZDB-ID	120573-0
ISSN	1523-1755 ; 0085-2538
ISSN (online)	1523-1755
ISSN	0085-2538
DOI	10.1016/j.kint.2022.04.023
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Zs.A 797: Show issues

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Article ; Online: Can inhibition of proteasomes or NF-kappaB help control idiopathic nephrotic syndrome?

Wenderfer, Scott E

Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association

2012 Volume 27, Issue 5, Page(s) 1698–1701

MeSH term(s)	Drug Resistance ; Female ; HIV Protease Inhibitors/therapeutic use ; Humans ; Male ; Nephrotic Syndrome/drug therapy ; Prednisone/therapeutic use ; Saquinavir/therapeutic use ; Steroids/therapeutic use
Chemical Substances	HIV Protease Inhibitors ; Steroids ; Saquinavir (L3JE09KZ2F) ; Prednisone (VB0R961HZT)
Language	English
Publishing date	2012-04-30
Publishing country	England
Document type	Editorial ; Research Support, N.I.H., Extramural ; Research Support, Non-U.S. Gov't ; Comment
ZDB-ID	90594-x
ISSN	1460-2385 ; 0931-0509
ISSN (online)	1460-2385
ISSN	0931-0509
DOI	10.1093/ndt/gfs079
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