Systemic Lupus Erythematosus in a 24-Year-Old

AFFILIATION:
Nephrologist, Senior Vice President for Medical Affairs, and Chief Medical Officer, CentraState Medical Center, Freehold, New Jersey

CITATION:
Matera J. Systemic lupus erythematosus in a 24-year-old. Consultant. 2023;63(7):e1. doi:10.25270/con.2022.08.000004

Published online August 2, 2022.

DISCLOSURES:
The authors report no relevant financial relationships.

CORRESPONDENCE:
James Matera, MD, CentraState Medical Center, 901 W Main St, Freehold, NJ 07728 (JMatera@centrastate.com)

A 24-year-old woman presented to the emergency department (ED) with joint swelling, severe arthralgias, and myalgias. She works as a nurse in a busy ED. She was admitted to the hospital for further workup.

History. She had received a diagnosis of systemic lupus erythematosus (SLE) at age 18 years and had her first known positive antinuclear antibodies (ANA) test at age 16 years. She has a maternal family history of SLE and lupus nephritis, and her mother is status post cadaveric renal transplant.

At the time of diagnosis at age 18, the patient did not have proteinuria but did have joint swelling and fatigue. About 6 months later, she had developed a malar rash, significant joint tenderness, nephrotic range proteinuria, and gross hematuria. A renal biopsy was conducted in January 2016, at which time a diagnosis of Class IV lupus nephritis was made. Her condition progressed to oliguric renal failure over the next 3 days and needed temporary dialysis. When the renal failure resolved, the patient’s tests returned with a normal creatinine level of 0.8 to 0.9 mg/dL.

At that time, the patient had been treated with high-dose corticosteroids, which led to steroid-induced diabetes; mycophenolate mofetil, 1500 mg twice daily; and an angiotensin-converting enzyme inhibitor. Because of the steroid-induced diabetes, the corticosteroids were tapered off. Her condition improved but then recurred, leading to restarting the corticosteroid at a higher dose with a slower taper. During this time, she was doing very well with stable renal function and a proteinuria level of less than 300 mg/g. To control her diabetes, she was using an insulin pump and maintaining a hemoglobin A1c level of less than 8%. 

Physical examination. No malar rash was noted upon admission to the hospital. She was tachycardic with a heart rate of 110 beats/min, had a regular rhythm, and had no murmur or rub. There were aphthous ulcers on the soft palate without petechiae. Her lungs were clear upon auscultation. No abdominal tenderness or splenomegaly were noted. There was 2+ pitting edema of both lower extremities. The skin did not show rashes or ulcerations.

Diagnostic testing. A renal ultrasonography scan was conducted, results of which showed normal-sized kidneys and no evidence of hydronephrosis.

Results of laboratory testing showed leukopenia, with an elevated white blood cell count of 2.0/μL; a creatinine level of 0.64 mg/dL; an estimated glomerular filtration rate of 96 mL/min; an albumin level of 3.3 g/dL; a C3 complement level of less than 5 mg/dL; a C4 complement level of less than 20 mg/dL; and urine albumin, with a creatinine ratio of 864 mg/g. She then underwent a renal biopsy for an update on her diagnosis. 

Treatment and management. After the biopsy was performed, hydroxychloroquine, 200 mg/d; oral prednisone, 80 mg/d; and oral lisinopril, 20 mg/d were added to her treatment regimen. Her renal function remained stable with a creatinine level of 0.74 mg/dL, and a second spot urinalysis returned a creatinine level of 2845 mg/g. She was discharged home on hospital day 4 on the above medications.

Two days after discharge, the patient’s glucose levels were consistently greater than 400 mg/dL, and she reported polyuria and polydipsia. Her fatigue, weakness, and myalgias persisted, and she tapered her own dose of prednisone to 10 mg/d to help alleviate the hyperglycemia. At that time, she reported an increase in her joint swelling. The prednisone dose was increased to 60 mg every other day and mycophenolate mofetil was increased to 1500 mg orally twice daily. Because of hypotension, the patient’s lisinopril dose was reduced to 5 mg/d orally.

The renal biopsy identified 21 glomeruli and the following pathology:

• Focal endocapillary and membranous glomerulonephritis with 1-minute fibrocellular crescent, consistent with lupus nephritis Class III+V
• Mild tubular atrophy and interstitial fibrosis
• Mild arteriosclerosis
• An acuity index of 5 (scale, 0-24) and chronicity index of 3 (scale, 0-12)

What is the next step in managing this patient, who has progressed to lupus nephritis Class III+V (mild acuity and chronicity)?

1. Maintain mycophenolate mofetil and prednisone, 60 mg every other day for 3 to 6 months, following serial complement levels renal function, and amount of proteinuria
2. Add belimumab to mycophenolate mofetil and utilize a corticosteroid-sparing regimen
3. Change to rituximab therapy
4. Change to cyclophosphamide therapy
5. Continue prednisone 0.5 to 1.0 mg/kg every other day and add oral voclosporin
6. Consider other therapies

Answer and discussion on next page.