An extremely rare masquerader – A case of chronic allograft dysfunction

47 year old lady who was detected to have hypertension at the age of 25 years, with renal dysfunction (Creatinine – 2.5 mg/dl), progressed to End stage renal disease requiring hemodialysis at the age of 31 years. She was on maintenance hemodialysis for the next 18 months and underwent a deceased donor renal transplant in 2009. Her post transplant period was uneventful and her creatinine was 1.2 until September 2021. Her creatinine showed a gradual rise to 1.9 mg/dl with increased requirement of anti hypertensives and increasing proteinuria with protein estimation showing 3950 mg/day. She underwent an allograft biopsy which showed duplication of the glomerular basement membranes and glomerulitis with c4d negative on immunofluorescence. She was treated with intravenous immunoglobulin and Rituximab for the same with not much improvement. However, an ultrasound of the transplant kidney showed bulky and echogenic transplant kidney with mild hydronephrosis and prominent renal sinus fat. MR Urogram showed extensive lymphangiectasia around the transplant kidney and ureter with markedly enlarged and edematous kidney. The collecting system and ureter are encased by the lymphangiectasia. After the treatment of CABMR her creatinine improved to 1.7 mg/dl and proteinuria reduced to 2100 mg/day. However her lymphangiectasia persisted and is being managed conservatively with no intervention planned. There are very few reported cases of Lymphangiectasia post renal transplant.