ACR Meeting Abstracts

ACR Meeting Abstracts

Abstract Number: 463

Percentage of Glomerular Crescents Predicts Renal Outcomes in Childhood-Onset Lupus Nephritis

Pooja Patel1, Marietta M. De Guzman1, Joseph Maliakkal2, Michelle Rheault3, David Selewski4, Katherine Twombley5, Jason Misurac6, Cheryl Tran7, Alexandru Constantinescu8, Ali Mirza Onder9, Meredith Seamon10, Vaishali Singh11, Cynthia Pan11, Joseph Flynn12, Abiodun Omoloja13, William Smoyer14, Guillermo Hidalgo15 and Scott E. Wenderfer16, 1Pediatrics-Rheumatology, Baylor College of Medicine, Houston, TX, 2Pediatrics-Renal, Saint Louis University, St. Louis, MO, 3Pediatrics-Renal, University of Minnesota, Minneapolis, MN, 4Pediatrics-Renal, University of Michigan, Ann Arbor, MI, 5Pediatrics-Renal, Medical University of South Carolina, Charleston, SC, 6Pediatrics-Renal, University of Iowa, Iowa City, IA, 7Pediatrics-Renal, Mayo Clinic, Rochester, MN, 8Pediatrics-Renal, Joe DiMaggio Children’s Hospital, Hollywood, FL, 9Pediatrics-Renal, University of Tennessee, Memphis, TN, 10Pediatrics-Renal, The University of Utah, Salt Lake, UT, 11Pediatrics-Renal, Medical College of Wisconsin, Wauwatosa, WI, 12Pediatrics-Renal, Seattle Children's Hospital, Seattle, WA, 13Pediatrics-Renal, Wright State University, Dayton, OH, 14Pediatrics-Renal, Nationwide Children’s Hospital, Columbus, OH, 15Pediatrics-Renal, East Carolina University, Greenville, NC, 16Pediatrics-Renal, Baylor College of Medicine, Houston, TX

Meeting: 2018 ACR/ARHP Annual Meeting

Keywords: , , ,

Session Information

Date: Sunday, October 21, 2018

Title: Pediatric Rheumatology – Clinical Poster I: Lupus, Sjögren’s Disease, and Myositis

Session Type: ACR Poster Session A

Session Time: 9:00AM-11:00AM

Background/Purpose:

Outcomes for childhood-onset crescentic lupus nephritis are unclear. The revised classification system for lupus nephritis by the Renal Pathology Society clearly distinguishes between cellular, fibrocellular, and fibrous crescents. A revised activity index includes cellular and fibrocellular crescents and uses thresholds of 25% and 50% glomerular involvement to distinguish between mild, moderate, and severe. The revised chronicity index includes scores for <25%, 25-50% and >50% fibrous crescents.

Methods:

The objective was to test the validity of the 25% and 50% thresholds for crescentic involvement in childhood-onset crescentic LN. We identified 69 patients in the Midwest Pediatric Nephrology Consortium’s pediatric glomerulonephritis with crescents registry (21% of total). Enrollment in the retrospective IRB-approved registry includes patients <21 years old with >1 crescentic glomerulus on kidney biopsy from 15 centers from 2004-16 with at least 1 year of follow-up data. All biopsies sampled from >10 glomeruli. The primary outcome was end stage kidney disease (ESKD) at 1 year. Secondary outcomes included estimated glomerular filtration rate (eGFR) at 1 year and change in eGFR over time. Crescents were defined as cellular/fibrocellular or fibrous based on local pathologist.

Results:

The median age at time of biopsy was 14.0 years (range 6-20) and median follow up was 3.0 years (range 1-11.3). A median of 27 glomeruli were sampled per biopsy (IQR 19-35). The median percentage of cellular crescents was 16.4% (IQR 10-38%, max 96%) and fibrous crescents was 0% (IQR 0-5%, max 56%). The cumulative incidence of ESKD was 1.5% at one year and 9% (n=6 patients) at last follow up. Median time to ESKD was 27.6 months (range 5-131). Outcomes stratified well by both percentage of cellular glomerular crescents and by percentage of fibrous crescents (Table 1). Median change in eGFR was +18mL/1.73m2/min (IQR -7 to +42) at 1 year and -3.1 mL/min/1.73m2/year (IQR -16 to +6) at latest follow-up. There were only three subjects with >25% fibrous crescents and only one with >50%.

Cellular crescents # Renal survival eGFR at biopsy Δ eGFR/yr Follow-up (yr)
<25% 42 100% 67 (50-105) +2.8 3.2
25-50% 15 93% 58 (46-81) +4.5 2.7
>50% 11 82% 30 (19-51) +8.8 1.8
Fibrous crescents # Renal survival eGFR at 1year Δ eGFR/yr Follow-up (yr)
<25% 64 92% 92 (74-100) +3.2 3.0
<10% 57 93% 93 (78-102) +2.7 3.0
None 45 91% 92 (74-102) +3.2 3.0
>1 23 91% 92 (81-100) +2.8 2.8
>10% 10 80% 69 (28-91) -2.4 3.8
>25% 3 67% 52 (26-79) -15.4 2.3

Conclusion:

We show utility for the thresholds for cellular crescents used in the 2018 revised NIH activity index in children with lupus nephritis. For fibrous crescents, thresholds of 10% and 25% better discriminate renal outcomes. We propose inclusion of a pediatric arm for future studies intended to generate evidence-based definitions for classification of lupus nephritis.

Disclosure: P. Patel, None; M. M. De Guzman, None; J. Maliakkal, None; M. Rheault, Regulus Therapeutics, 2,Reata pharmaceuticals, 2,Retrophin pharmaceutcals, 2,Roche pharmaceuticals, 2; D. Selewski, None; K. Twombley, None; J. Misurac, None; C. Tran, None; A. Constantinescu, None; A. M. Onder, None; M. Seamon, None; V. Singh, None; C. Pan, None; J. Flynn, None; A. Omoloja, None; W. Smoyer, None; G. Hidalgo, None; S. E. Wenderfer, None.

To cite this abstract in AMA style:

Patel P, De Guzman MM, Maliakkal J, Rheault M, Selewski D, Twombley K, Misurac J, Tran C, Constantinescu A, Onder AM, Seamon M, Singh V, Pan C, Flynn J, Omoloja A, Smoyer W, Hidalgo G, Wenderfer SE. Percentage of Glomerular Crescents Predicts Renal Outcomes in Childhood-Onset Lupus Nephritis [abstract]. Arthritis Rheumatol. 2018; 70 (suppl 9). https://acrabstracts.org/abstract/percentage-of-glomerular-crescents-predicts-renal-outcomes-in-childhood-onset-lupus-nephritis/. Accessed .

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