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Abstract: TH-PO514

Membranous Nephropathy in the Setting of Adult-Onset Still Disease: A Rare Association

Session Information

Category: Glomerular Diseases

  • 1303 Glomerular Diseases: Clinical‚ Outcomes‚ and Trials

Authors

  • Shettigar, Shruti, Cleveland Clinic Florida, Weston, Florida, United States
  • Gebreselassie, Surafel K., Cleveland Clinic Florida, Weston, Florida, United States
  • Bobart, Shane A., Cleveland Clinic Florida, Weston, Florida, United States
Introduction

Adult onset still’s disease (AOSD), is a multi-system, autoimmune, inflammatory disorder.
Typically, if a patient with AOSD presents with proteinuria, the initial concern is secondary renal amyloidosis given the severity and persistence of inflammation. We present a case of a patient with AOSD and proteinuria with biopsy proven membranous nephropathy (MN). The association of AOSD with MN is rare and to our knowledge, there is only one other published case of AOSD subsequently being diagnosed with MN.

Case Description

A 19 year old African American female presented to the Nephrology clinic for evaluation of non-nephrotic range proteinuria. Approximately 2 years prior at another institution, she presented with rash, arthralgia, and nightly fevers with severely elevated inflammatory markers and low positive ANA (1:80). She was diagnosed with Still’s disease and treated initially with prednisone followed by canakinumab an IL-1β inhibitor for 1 year. Symptoms persisted with development of lymphadenopathy, hepatomegaly, ferritin as high as 15797 ng/ml and CRP 137 mg/dL. She was then treated with three other biologics including anakinra (IL-1R antagonist), tocilizumab (IL-6R antagonist) and then maintained on sarilumab (IL-6R antagonist).

At Nephrology presentation, urinalysis showed no hematuira but 3+ proteinuria, confirmed to be 1.4 grams/24 hr. Serum albumin was 3.2 g/dL and serum creatinine 0.45 mg/dL. She was normotensive and had no edema on examination. Additional serological work up was unrevealing with negative dsDNA, normal C3 and C4, negative PLA2R, extractable nuclear antigens, ANCA, cryoglobulin, no infectious causes and HbA1c 5.8. Given persistent proteinuria, a kidney biopsy was done with unrevealing light microscopy, mild foot process effacement and subepithelial immune-complex type deposits on electron microscopy. On immunoflurescence, 3+ IgG, kappa and lambda within the distribution of subepithelial deposits confirmed the diagnosis of an early membranous nephropathy. PLA2R and Congo red stain were both negative. She was started on losartan with improvement in proteinuria to 0.6 grams.

Discussion

While secondary renal amyloidosis remains a clinical concern in AOSD presenting with proteinuria, clinicians should perform a kidney biopsy as other lesions such as membranous nephropathy may be rarely associated.