Case Presentation: 59 years old Hispanic male with history of alcoholic cirrhosis, esophageal varices that presented with 1 day history of abdominal pain and worsening renal function. Patient gets therapeutic paracentesis every 2 weeks, and the last one was one day prior his presentation. He was seen by his PCP who instructed him to present to the ED due to worsening kidney function and hyponatremia. On initial evaluation patient was tachycardic HR: 128 bpm, BP of 109/61 and with mild abdominal distension. Initial labs were notable for leukocytosis, 17.6 x10E3/uL indirect hyperbilirubinemia, 1.6 mg/dL, elevated AST at 74 Unit/L, ALT at 58 unit/L, elevated creatinine of 2.2 mg/dL, hypoalbuminemia of 2.4 g/dL and hyponatremia with a sodium of 124mmol/L. A diagnostic paracentesis was performed reporting 253 PMNs/mm3 (RBC corrected count is 249 PMNs/mm3) antibiotic coverage was started with Ceftriaxone for initial concerns of spontaneous bacterial peritonitis SBP. During admission patient had worsening AKI, and hypotension due to intravascular depletion, he was treated with daily albumin. Initial diagnostic impression of his AKI was hepatorenal syndrome vs acute tubular necrosis. A complete workup for nephropathy was ordered. Patient developed hematuria and low levels of C3 and C4 prompted renal biopsy. Patient was transferred to the MICU due to hypotension non responsive to intravenous fluids and requirement of vasopressors. During the ICU he developed worsening AKI and acute left perinephric hematoma post biopsy, requiring transfusion of RBC unit. Left popliteal vein thrombosis, was another complication requiring IVC filter placement, due to anticoagulation contraindication. Renal biopsy reported IgA nephropathy, moderate interstitial fibrosis, with overlay of acute tubular injury, amyloidosis LECT-2 type.
Discussion: Amyloidosis is caused by an abnormal deposition and accumulation of insoluble protein fibrils in multiple organs, often leading to diverse clinical presentations, and possible organ failure. There is a recently described protein amyloidogenic leucocyte chemotactic factor 2 (LECT2), recognized as a new type of renal and liver amyloidosis. There is also evidence that patients with LECT2 renal amyloidosis have high frequency of concurrent glomerular disease, including diabetic nephropathy, IgA nephropathy, membranous nephropathy. This case illustrates the coexistence of both LECT-2 renal amyloidosis and IgA nephropathy in the setting of a patient with chronic liver disease. The diagnosis of LECT2 amyloidosis is usually made by kidney or liver biopsy, LECT2 accounted for up to 25% of hepatic amyloid cases. In the United States, LECT2 protein has been found to be especially prevalent among people of Hispanic ethnicity.
Conclusions: LECT2 renal amyloidosis and IgA nephropathy coexistence is recently described and chronic inflammatory conditions as chronic liver disease are hypothesized to be related with both. However, there is not enough evidence that supports that there is a pathologic correlation between the two entities and further research to characterized it should be performed. Clinicians should be aware of its potential role as a cause of hepatic and renal disease especially in Hispanics population.