Leukocyte Chemotactic Factor 2 Amyloidosis with Proteinase 3-Antineutrophil Cytoplasmic Antibody Positivity Manifesting as Rapidly Progressive Glomerulonephritis

A BSTRACT This report describes a rare case of leukocyte chemotactic factor 2 (LECT2) amyloidosis associated with positive proteinase 3 antineutrophil cytoplasmic antibody (PR3-ANCA), presenting as rapidly progressive glomerulonephritis (RPGN) in a 55-year-old woman. On admission, the patient exhibited anuric renal failure, bilateral edema, and respiratory distress. Laboratory results revealed severe renal dysfunction with proteinuria and microscopic haematuria. High titre of anti proteinase -3 anti-neutrophil cytoplasmic antibody was noted. Kidney biopsy showed amyloid deposits with staining pattern suggestive of LECT2-related amyloidosis. The patient was managed with hemodialysis and immunosuppressive therapy, which led to significant improvement in renal function. This case highlights the importance of considering unusual forms of amyloidosis in RPGN presentations.