Background/Purpose:  
It is well known that patients with juvenile Systemic Lupus Erythematosus (jSLE) are at much higher risk of developing Lupus Nephritis (LN) compared to their adult counterparts. Many patients with jSLE present with LN at onset disease. A less severe jSLE course, without organ threatening disease such as LN, is uncommon.

Methods:  
In this case series, we review 3 jSLE patients, all meeting 2019 ACR/EULAR classification criteria for SLE, that presented with jSLE without LN at time of diagnosis. We compare case demographics, disease characteristics at diagnosis, initial treatment course, time to development of LN, LN histopathologic classification and treatment changes after development of LN.

Results:
The patients varied in age at time of diagnosis from 9 to 12. They are of 3 different ethnicities that are at higher risk of SLE (Hispanic, Punjabi and Black). All three patients presented with polyarthritis of varying severity. At diagnosis, patients 1 and 2 had rash while patient 3 had lymphadenopathy. Patient 2 also had oral and palatal ulcers. All 3 patients presented with varying degrees of cytopenia and patients 2 and 3 had transaminitis. All 3 patients had low complement and elevated dsDNA at time of diagnosis. Patient 1 had anti-Smith, anti-RNP and anti-Ro positivity and was initially treated with oral prednisone, azathioprine and hydroxychloroquine. Five years after initial diagnosis she developed proteinuria on monitoring labs and went on to have a renal biopsy confirming Class IV/V LN. She was given IV pulse steroid followed by oral steroid and switched from azathioprine (AZA) to Mycophenolate Mofetil (MMF). Patient 2 had anti-chromatin and anti-Scl-70 positivity and was initially treated with low dose prednisone, naproxen and hydroxychloroquine. Four months after initial diagnosis she developed proteinuria on 20 mg of prednisone and her biopsy found Class IV LN. She was maintained on oral prednisone and MMF was initiated. Patient 3 had anti-chromatin, anti-Ro and anti-ribosomal-P antibodies and was initiated on oral prednisone and AZA. Six months after initial diagnosis, arthritis flared after weaning prednisone and she was found to have new proteinuria. Biopsy showed Class LN. She started on oral prednisone and MMF.

Conclusion: These 3 cases of delayed onset LN in jSLE highlight the importance for ongoing renal surveillance, even in the absence of early renal symptoms. LN can develop quickly and often with few symptoms, and disease can be severe and renal threatening even with prompt recognition of LN.

« Back to 2026 Pediatric Rheumatology Symposium

ACR Meeting Abstracts - https://acrabstracts.org/abstract/the-quiet-beginning-early-pediatric-systemic-lupus-erythematosus-sle-without-nephritis-and-the-path-to-renal-involvement/