Abstract: TH-PO528
Antinuclear Antibody Negative Lupus Presenting as Nephritis
Session Information
- Glomerular Diseases: Clinical, Outcomes, Trials - I
November 03, 2022 | Location: Exhibit Hall, Orange County Convention Center‚ West Building
Abstract Time: 10:00 AM - 12:00 PM
Category: Glomerular Diseases
- 1303 Glomerular Diseases: Clinical‚ Outcomes‚ and Trials
Author
- Vangapalli, Ananthalaxmi, Albert Einstein Medical Center, Philadelphia, Pennsylvania, United States
Introduction
Lupus nephritis (LN) is a serious and common complication of systemic lupus erythematosus (SLE) that predisposes to significant morbidity and mortality. Studies show that prompt diagnosis and treatment improve patient survival.
Case Description
24-year-old Asian-American female with no known past medical history presented to ED for 4 days of fatigue, shortness of breath, and rash. She reports symptoms were there for one year with arthralgias involving bilateral knees, hands and fingers and toes with morning stiffness without significant joint swelling. She noticed episodes of skin rash on extremities, worsened with scratching with an exaggerated pathergy. She has been taking Advil for joint pains. SH: She was born in America. She works as a designer for Pfizer. Labs showed creatinine 2.11 from normal baseline a year ago, with Urine protein to creatinine ratio of 2 grams. Hemoglobin 7 with normal MCV. Platelets, PT/INR, PTT, LDH, and haptoglobin are normal. D-dimer elevated to 2600. Her ANA was negative. CT abdomen pelvis showed hepatosplenomegaly and multiple enlarged lymph nodes. On exam vital signs are normal. Dry mucous membranes and diffuse popular rash in the lower extremities. She underwent a renal biopsy which revealed lupus-like glomerulonephritis, class v/class IV, mild acute tubular necrosis, moderate interstitial inflammation, and mild to moderate interstitial fibrosis. It was called lupus-like nephritis given immunofluorescence negative and multiple deposits on EM. Lymph node biopsy which showed reactive hyperplasia. She was started on Cellcept 1500 mg twice daily and prednisone 60mg daily.
Discussion
Literature review on antinuclear antibody (ANA)-negative and seronegative LN revealed the following patient presentations: (1) renal-limited or renal and extra-renal manifestations of SLE with negative serologies and (2) renal and extra-renal manifestations of SLE with negative serologies at presentation who develop positive serologies later in follow-up. Both groups represent a unique and challenging cohort of patients who may require longer follow-up and further testing to rule out other glomerular diseases that may mimic LN on renal biopsy. The absence of SLE-related serologies should be weighed against a high pre-test probability of ANA-negative or seronegative LN. If highly suspected, the patient should be treated promptly with close monitoring.