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Kidney Week

Abstract: TH-PO213

Unusual Case of Lupus Nephritis with Negative Serology

Session Information

Category: Nephrology Education

  • 1302 Fellows and Residents Case Reports

Authors

  • De jesus, Eddy J, Bronx Lebanon Hospital, Bronx, New York, United States
  • Goel, Bindu, Bronx Lebanon Hospital, Bronx, New York, United States
  • Nasr, Rabih, Bronx Lebanon Hospital, Bronx, New York, United States
Background

Lupus Nephritis (LN) is a common complication of Systemic Lupus Erythematous (SLE) and occurs in 43-55% of the cases,usually in first year of the disease. It is one of the highest predictors of morbidity and mortality, and thus requires prompt diagnosis and treatment

Methods

We report a case of a 25 year old hispanic female presenting to the nephrology clinic referred due to episodes of gross hematuria for 2 months followed by persistent asymptomatic microhematuria and proteinuria of 2g/day, In the initial workup she was found to have proteinuria up to 2 grams/day and further autoimmune workup revealed positive anti-myeloperoxidase of 115. She did not have any history of diabetes or hypertension, no edema, serum creatinine of 0.8 mg/dl, urinalysis 1-2+ protein microhematuria, no RBC cast, urine total protein/creatinine ratio 2.0 gm, serum albumin 3.6 g/dl, with positive anti-MPO ANCA, negative ANA, Hepatitis B Virus(HBV) and Hepatitis C. Initially she was suspected to have microscopic polyangiitis however kidney biopsy was done and revealed membranous and focal segmental endocapillary and extra capillary proliferative and sclerosis glomerulonephritis, immune complex type, suggestive of class V and III of moderate activity and moderate chronicity, and mild tubulointerstitial atrophy with P-ANCA/Anti MPO antibody associated/clinical. Since biopsy was suggestive of Lupus Nephritis, comprehensive serologies were sent once again and reported as negative for ANA, anti-ds DNA, Sm, RNP, SSA-SSB, cardiolipin and included normal complements. Patient was started on Mycophenolate Mophetil and prednisone ( tapering ) One year after diagnosis patient still denies any constitutional symptoms, rashes, photosensitivity, oral ulcers, joint pain/swelling or weight loss

Conclusion

Lupus Erythematous (SLE), is a heterogeneous autoimmune disease that affects multiple organs and particularly the kidneys. Fortunately anatomical and pathologic markers of Lupus nephritis are quite specific. Rare cases such as the one from Chi Young Park et al, where there is pathological confirmation of LN but negative serology have been reported. Nephrologists should still have high suspicion of Lupus nephritis in any patient that presents with proteinuria and hematuria and negative serology as limited data is available as it has shown poor prognosis.