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Abstract: PO1943

Renal Failure of the Non-Crisis Variety in Scleroderma

Session Information

Category: Pathology and Lab Medicine

  • 1600 Pathology and Lab Medicine

Authors

  • Vondenberg, Jaime (James) A., University of New Mexico School of Medicine, Albuquerque, New Mexico, United States
  • Teixeira, J Pedro, University of New Mexico School of Medicine, Albuquerque, New Mexico, United States
  • Shaffi, Saeed Kamran, University of New Mexico School of Medicine, Albuquerque, New Mexico, United States
  • Shieh, Michelle, University of New Mexico School of Medicine, Albuquerque, New Mexico, United States
Introduction

Renal disease from systemic sclerosis (SS) classically presents as scleroderma renal crisis (SRC). In contrast, amyloidosis in SS is rare.

Case Description

A 73-year-old woman with untreated limited SS for >20 years (with ANA 1:160 centromere pattern, negative Scl-70, sclerodactyly, perioral scleroderma, telangiectasias, Raynaud’s, and mild pulmonary hypertension), CKD with baseline creatinine of (Cr) ~1.5 mg/dL, Barrett's esophagus, and recent right below-knee amputation (for chronic wound osteomyelitis and invasive squamous cell carcinoma) is readmitted 2 weeks later for a pseudomonal stump infection. She develops AKI (Cr peak of 6) requiring HD. Renal biopsy reveals amyloidosis [figure]. Immunofluorescence and immunohistochemistry are negative for deposition of kappa, lambda, AA, or leukocyte chemotactic factor 2 (LECT2), but subsequent liquid chromatography-tandem mass spectrometry (LC-TMS) is positive for LECT2.

Discussion

SRC occurs in 5-10% of SS cases but is no longer the primary cause of death in SS with the advent of ACE inhibitors (ACEi). Unlike other autoimmune diseases, amyloidosis, especially renal amyloidosis, is rare in SS. While recovery from dialysis can occur in SRC with ongoing ACEi therapy, AA amyloidosis requiring dialysis carries a poor renal prognosis. LECT2 is a recently discovered amyloid protein that can deposit in multiple organs, with the most significant clinical feature of LECT2 amyloidosis (ALECT2) being slowly progressive renal failure. The pathogenesis is poorly understood. Diagnosis is by renal biopsy, with advanced methods like LC-TMS often needed for LECT2 detection. Though the reason is unclear, ALECT2 seems to have a predilection for Mexican Americans and Native Americans and is likely underdiagnosed in the Southwest U.S. However, it is not usually associated with autoimmune disease, with this being the first known case of ALECT2 occurring in the context of SS.

Biopsy shows amyloid deposits, primarily in the cortical interstitium and vessels, by Congo red stain [A] with green birefringence under polarized light [B], with global sclerosis of 22/35 glomeruli and 50% interstitial fibrosis.