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Abstract: FR-PO633

It's Not Always Lupus Nephritis

Session Information

Category: Glomerular Diseases

  • 1302 Glomerular Diseases: Immunology and Inflammation

Authors

  • Hardee, Maris, University of Mississippi School of Medicine, Jackson, Mississippi, United States
  • Schwartz, Carter V., The University of Mississippi Medical Center, Jackson, Mississippi, United States
  • Dossabhoy, Neville R., The University of Mississippi Medical Center, Jackson, Mississippi, United States
  • Caza, Tiffany, Arkana Laboratories, Little Rock, Arkansas, United States
  • Atari, Mohammad, The University of Mississippi Medical Center, Jackson, Mississippi, United States
Introduction

Systemic lupus erythematosus (SLE) is a complex immunological disease, with a plethora of manifestations. While lupus nephritis commonly affects patients with SLE, other renal diseases may also involve the kidney in SLE patients. Here, we describe a rare case of chronic sclerosing IgA nephropathy in a patient with SLE.

Case Description

A 31-year-old African American female with a history of SLE and secondary Sjogren syndrome presented with dyspnea and pleuritic chest pain. On exam, she had severe hypertension and peripheral edema. Labs were significant for pancytopenia, and creatinine elevated to 2.4 mg/dL (up from a baseline of 1 mg/dL). The urine protein-to-creatinine ratio was 6.57 g/g. Antinuclear antibodies, SSA antibodies, and chromatin antibodies were positive, with a low C3 complement but normal anti-double-stranded DNA antibodies. Kidney biopsy revealed chronic sclerosing IgA nephropathy and arterionephrosclerosis. Global (15/31) and segmental glomerulosclerosis with collapsing features were present. Immunofluorescence showed granular mesangial staining for IgA (+3), IgG (+1), IgM (+2), C3 (+3), kappa and lambda (+2), and negative C1q. Electron microscopy showed focal subendothelial and numerous mesangial electron-dense deposits. The patient declined testing for APOL-1.

Discussion

The incidence of IgA nephropathy in the setting of SLE is unknown. However, secondary IgA nephropathy has been well described in the setting of Sjogren syndrome. The patient described here presented with nephrotic range proteinuria, elevated creatinine and active systemic lupus, raising concern for lupus nephritis at the time. Kidney biopsy revealed an unexpected glomerular pathology of IgA nephropathy. This case emphasizes the importance of consideration of other etiologies as a cause of renal impairment in SLE patients even with active systemic lupus.