A Case of Diffuse Class 4 Lupus Nephritis With Normal Complement Levels

Document Type

Conference Proceeding

Publication Date

4-2024

Publication Title

American Journal of Kidney Diseases

Abstract

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that can affect any organ, but Lupus Nephritis is one of the most common with an incidence of 60%. Characteristics of lupus nephritis is low total complement hemolytic activity (CH50) as well as decreased C3 and C4 levels. These are found in about 75% of patients with focal nephritis and 90% in patients with diffuse nephritis. A 35-year-old female presented with gradually worsening lower extremity swelling. Past medical history significant for hypertension, Diabetes, hyperlipidemia, and lupus nephritis treated with cyclophosphamide. The patient was on a maintenance dose of mycophenolate mofetil 1000 bid and prednisone for the previous six months. EVitals were stable. Physical examination showed lower extremity edema. Laboratory evaluation showed sodium of 142, potassium of 4.7, bicarb of 22, BUN of 20, creatinine of 0.74. CBC showed hemoglobin 12.6, white count 5.8, platelets 234. Complements were normal at C3 119, C4 28. ANA was positive. C-ANCA and P-ANCA were negative (Proteinase- 3 antibody <0.2, myeloperoxidase antibody <0.2).Urinalysis showed protein 500 mg/dl, urine protein creatinine ratio of 3.6 g/g. Biopsy revealed widespread endocapillary hypercellularity, cellular and fibrocellular crescents in 6/25 glomeruli. Immunofluorescence showed granular deposits of C1q, C3, IgA, IgG and IgM along the capillary walls in the mesangium. The biopsy was diagnostic of diffuse class IV lupus nephritis. Patient received Methylprednisolone 500 mg IV for 3 days, rituximab, 1 gram twice a day and continued on mycophenolate mofetil 1500 mg twice daily. After initiating therapy, the patient showed improvement in proteinuria 0.5g/g We currently do not have good biomarkers of disease activity for Lupus and decreased complements are thought of as a reliable marker for disease activity, but here in patient with normal creatinine and normal complements, we found highly active disease necessitating therapy. Clinicians should be aware of this and be quick to biopsy seemingly stable patients with lupus.

Volume

83

Issue

4 Suppl 2

First Page

S114

Comments

National Kidney Foundation 2024 Spring Clinical Meetings, May 14-18, 2024, Long Beach, CA

DOI

10.1053/j.ajkd.2024.01.375

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