ALECT2 Amyloid Concurrent with IgAN

Introduction: Amyloidosis is a disorder of improper confirmational changes of proteins leading to deposition in multiple organs causing disease. In this case series we present 2 patients with IgA nephropathy (IgAN) and amyloidogenic leukocyte chemotactic factor 2 (ALECT2). Case Description: A 49-year old Hispanic female diagnosed with IgAN had been managed on conservative therapy with enalapril and spironolactone with stable serum creatinine (Cr) of 1.1-1.2mg/dL and proteinuria (UPCR) of ~1g/g. 17-years renal function and proteinuria started to rise, Cr 1.4-1.7mg/dL and UPCR 2-3g/g. A renal biopsy was performed and showed IgAN with focal sclerosis glomerulonephritis and ALECT2 with glomerular and interstitial invasion. Patient was treated with conservative measures and maintained stable renal function. A 45-year old Hispanic female diagnosed with IgAN had been managed on losartan with stable serum Cr at 1.7mg/dL. 18-years later she had rising Cr & proteinuria, 2.6mg/dL and 4g/g respectively. She underwent a renal biopsy which found IgAN with ALECT2 amyloid deposition. Patient was treated with conservative measures and had stable renal function, but was lost to follow-up. Discussion: Although new to the game of amyloidosis, ALECT2 is now just as common as AA amyloidosis. It has a strong predominance for Hispanics especially those living in the southwest United States, though other ethnicities have been implicated. It predominately affects the kidneys and liver, but other organs have been found to be affected, except the heart. Patients will have some degree of renal insufficiency with variable urine sediments. Prognosis is good compared to other types of amyloidosis, with trajectory of renal function dependent on concurrent disease on biopsy, serum Cr at diagnosis, and degree of glomerulosclerosis. Treatment is supportive as no current therapies exist. These cases demonstrate the need for consideration of other pathologies that could be contributing to worsening renal function especially in Hispanic patients with IgAN as it could impact future management.