An Atypical Case of IgAN with Chronic Daily Macroscopic Hematuria for 10 Years

Introduction: While IgA nephropathy is the most common primary glomerular disease worldwide, its presentation is highly variable, with episodic gross hematuria and microscopic hematuria among the most common clinical manifestations. Chronic daily hematuria leading to recurrent symptomatic, transfusion-dependent anemia is unusual. Case Description: A 25-year-old male was hospitalized with severe anemia. He endorsed chronic, painless, and daily gross hematuria for which he had been extensively evaluated without any diagnosis or resolution. On numerous occasions, he had developed iron deficiency anemia requiring transfusions/IV iron repletion. Onset of hematuria was without a known inciting event like a upper respiratory or gastrointestinal illness. He did not have hearing loss and or use NSAIDs or anticoagulants. He has no known family history of kidney disease. Hemoglobin electrophoresis was unremarkable. He had undergone extensive imaging due to concern for nutcracker syndrome which returned negative, but instead demonstrated variant venous anatomy in the left lower pole accessory renal vein with drainage into the lumbar vein at the level of L5. He underwent balloon angioplasty of this aberrant renal vein followed by embolization of an accessory left renal artery, without resolution of hematuria. Cystoscopy was unremarkable. Further renal imaging was without cysts or stones. He was found to have a UACR of 1180 mg/g and UPCR of 2.1 g/g, with normal complements and serologies. Renal biopsy revealed mild mesangial matrix increase and irregular mesangial proliferation with minimal interstitial fibrosis and tubular atrophy. Immunofluorescence showed mesangial staining with IgA and a final diagnosis of IgA nephropathy was made. He was initiated on a steroid taper as well as an ARB with unabated daily hematuria and albuminuria. He is in the process of obtaining sparsentan for further therapy. Discussion: IgA nephropathy is a known cause of episodic hematuria but may rarely be a cause of persistent hematuria. It has been established that microscopic hematuria is associated with a higher risk of disease progression. It is presently not directly implemented in the risk score but is said to correlate with the Oxford MEST system. Most treatments are tailored towards proteinuria management and data is sparse on the best approach to treatment in patients with persistent hematuria.