Case 1: A 29-year-old female with kidney disease A 29-year-old female presents with a scaly malar rash, nocturia, puffy eyes, and swollen feet of 4 weeks duration. Her medications include ibuprofen for headaches x 6 weeks. Her physical exam is notable for high blood pressure 150/90, pulse 80, periorbital swelling, erythema with scale in malar distribution, normal heart and lung exam, 2+ bilateral pedal edema. Her labs are notable for WBC 8.8 x 10⁹/L, Hb 9.0 g/dL, platelets. 150 x 10⁹/L, ESR 100 mm/hr, eGFR 55 mL/min/1.73m², ANA 1:320 speckled, anti-dsDNA 142 IU/mL, + lupus anticoagulant, C3 48 mg/dL, UA: +5–10 RBCs, +5–10 WBCs, spot UPCR 5.4.

She undergoes a diagnostic kidney biopsy that shows Class IV lupus nephritis (LN), AI 9/24, CI 2/12, no vascular abnormalities.

After discussing her new diagnosis of systemic lupus erythematosus and LN, you discuss therapeutic options. She reveals that she is very worried about her kidneys and is interested in future childbearing. Also, due to her busy work schedule, she prefers to NOT use an intravenous medication.

Discussion Points

• What best describes the pathogenesis of her kidney disease?

• In addition to photoprotection, hydroxychloroquine, and glucocorticoids, what do you recommend as initial therapy for this patient?

Case 1: (continued) She is initiated on a regimen of mycophenolate mofetil (MMF) 2 g/d and voclosporin 23.7 mg bd in addition to hydroxychloroquine (HCQ) and glucocorticoids. After 14 months, she achieves a complete renal response with UPCR 0.4, eGFR 85 mL/min/1.73 m², anti-dsDNA 30 IU/mL, C3 72 mg/dL. Her prednisone dose is down to 5 mg/d.

One year later, she presents with arthralgias/stiffness of her bilateral wrists, malar rash, fatigue, and foamy urine after a trip to Hawaii with her family. UPCR 2.2, eGFR 60 mL/min/1.73 m², anti-dsDNA 53 IU/mL, C3 60 mg/dL.

You ask her kindly about medication adherence, and she admits that it has been very difficult for her to remember to take her medications because of her busy schedule. In addition, she has been experiencing nausea and loose stools with the MMF and prefers to avoid it. She is not interested in trying enteric-coated mycophenolate sodium.

She undergoes a repeat kidney biopsy that shows: Class III + V LN; AI 4/24, CI 5/12.

Learning Objectives At the end of this workshop participants will be able to:

• Discuss the pathogenesis of LN including the ISN/RPS Classification

• Describe the evidence-based management of the initial treatment of LN

• Compare the use of belimumab and voclosporin for the treatment of LN

• Discuss the role of a repeat kidney biopsy in the management of LN