Paricalcitol as an Antiproteinuric Agent Can Result in the Deterioration of Renal and Heart Function in a Patient with Fabry Disease

Abstract

Patient: Female, 44Final Diagnosis: Deterioration of renal and heart functionSymptoms: HypercalcemiaMedication: —Clinical Procedure: LaboratorySpecialty: NephrologyObjective:Rare diseaseBackground:Fabry disease is a rare and progressive X-linked inherited disorder of glycosphingolipid metabolism that is due to deficient or absent lysosomal α-galactosidase A activity. Among its other associated signs and symptoms, patients present with renal failure and proteinuria, which are markers of disease progression. Renin-angiotensin-aldosterone system (RAAS) blockers can slow the progression of chronic renal failure and proteinuria. In fact, some studies have shown the beneficial effects of paricalcitol on proteinuria.Case Report:We present a case of a female patient with the classic variant of Fabry disease. She was treated with a high dose of paricalcitol as an antiproteinuric agent due to unsatisfactory double-RAAS blockage, which resulted in transient worsening of cardiac and renal function.Conclusions:Despite the positive effects of paricalcitol as an antiproteinuric agent, as previously shown by some authors, our case highlights the possible serious adverse effects associated with the use of high doses of this drug.

Treatment of Fabry Nephropathy: A Literature Review

“…Pisani et al showed that add-on 1-mg paricalcitol treatment reduced proteinuria from 2.1 g/14 h to 0.4 g/day over a 6-month period in patients with Fabry nephropathy who used an ACEi and/or ARB at the maximum tolerated dosage [ 53 ]. Although the ability of paricalcitol to control proteinuria is known, transient worsening of cardiac and renal functions associated with the use of paricalcitol has been described [ 54 ]. Trimarchi et al [ 55 ] proposed the potassium-sparing diuretic amiloride as a candidate adjuvant antiproteinuric agent in Fabry disease.…”