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Do we really need cyclophosphamide for lupus nephritis?

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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.

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Authors and Affiliations

  1. Department of Pediatrics, The University of British Columbia, Vancouver, BC, Canada

    Scott E. Wenderfer

  2. Pediatric Nephrology, BC Children’s Hospital, Vancouver, BC, Canada

    Scott E. Wenderfer

  3. Department of Pediatrics, University of Colorado, Denver, CO, USA

    Jennifer C. Cooper

  4. Pediatric Rheumatology, Children’s Hospital Colorado, Aurora, CO, USA

    Jennifer C. Cooper

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SEW serves on the data monitoring committee overseeing AstraZeneca and Novartis-sponsored clinical trials. JCC, no conflicts to declare.

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Answers: 1. b; 2. d; 3. e; 4. a; 5. e

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Wenderfer, S.E., Cooper, J.C. Do we really need cyclophosphamide for lupus nephritis?. Pediatr Nephrol (2024). https://doi.org/10.1007/s00467-024-06367-9

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