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Abstract: TH-PO519

Leukocyte Chemotactic Factor 2 Amyloidosis with Concurrent Post-Infectious Glomerulonephritis and Diabetic Nephropathy

Session Information

  • Trainee Case Reports - I
    October 25, 2018 | Location: Exhibit Hall, San Diego Convention Center
    Abstract Time: 10:00 AM - 12:00 PM

Category: Trainee Case Reports

  • 1202 Glomerular Diseases: Immunology and Inflammation

Authors

  • Cruz Whitley, Jessica, University of California, Davis, Sacramento, California, United States
  • Sheth, Vishwa C., University of California, Davis, Sacramento, California, United States
  • Jen, Kuang-Yu, University of California, Davis, Sacramento, California, United States
  • Morfin, Jose A., University of California, Davis, Sacramento, California, United States
  • Madan, Niti, University of California, Davis, Sacramento, California, United States
Introduction


Leukocyte chemotactic factor 2 amyloidosis (ALECT2) is a form of amyloidosis that manifests with progressive renal failure. Biopsies typically exhibit congophilic deposits that are most prominent in the interstitium. ALECT2 is the third most common form of amyloidosis with strong ethnic bias, affecting mainly Hispanics with chronic renal insufficiency. Significant proteinuria is rarely associated with ALECT2 unless a second concurrent glomerulopathy is present. We report a case of ALECT2 with nephrotic syndrome due to concurrent post-infectious glomerulonephritis and diabetic nephropathy.

Case Description


A 50-year-old Hispanic female with no significant medical history admitted for anasarca, was found to have bilateral pleural effusions and newly diagnosed type 2 diabetes mellitus. Laboratory values were significant for HgbA1c of 13.2%, normal renal function, albumin of 2.5 g/dL and 4.5 grams of proteinuria. Serological workup showed positive ANA with decreased C3 and C4. HIV, hepatitis, SPEP and serum free light chains were negative. Although diabetic nephropathy (DN) can cause nephrotic range proteinuria, it rarely presents with nephrotic syndrome. Thus, renal biopsy was performed which showed multiple glomerular abnormalities including glomerulonephritis (GN) with dominant C3 staining along with predominat mesangial deposits and nodular diabetic glomerulosclerosis. Congo red was performed which showed scattered positive staining in the interstitium, consistent with amyloid. Mass spectroscopy further characterized the amyloid type as ALECT2. The patient was discharged with insulin therapy and started on an ACE-inhibitor. The differential diagnosis for her C3-dominant GN included post-infectious GN and C3 GN. At 6 weeks clinic follow up, her C3 level normalized favoring the diagnosis of post-infectious GN.

Discussion


ALECT2 is one of the more recently recognized amyloid proteins to affect the kidney and fairly prevalent however it is still under recognized. Concurrent glomerular disease is not uncommon in patients with ALECT2, with DN being most frequent. Coexistence of three disease processes (post-infectious GN, DN and ALECT2) makes our case exceptional. This case also illustrates the significance of renal biopsy in patients with atypical presentations of common renal diseases.