Posterior Reversible Encephalopathy Syndrome While On Achtar Gel Therapy for Primary FSGS
Michael Filoramo1, Renish Contractor1, Deepak Chandramohan2, Prabhat Singh3, Ujjwala Murari4, Nihar Jena5, Krishna Kishore Bingi6, Pradeep Vaitla7, Prathap Kumar Simhadri1
1Florida State University College of Medicine, 2UAB School of Medicine, 3Christus Spohn Hospital, 4West Virginia University, 5Wayne State University, 6Mon Health, 7University of Mississippi Medical Center
Objective:
NA
Background:

Primary Focal Segmental Glomerulosclerosis (FSGS) is a type of podocytopathy and a common cause of nephrotic syndrome in adults. Achtar gel, an adrenocorticotropic hormone analog, has been used in resistant cases of FSGS with some success. Posterior reversible encephalopathy syndrome (PRES) has never been reported in association with Achtar gel. We present a primary FSGS patient with a history of well-controlled hypertension and chronic kidney disease who presented with generalized tonic-clonic seizures, encephalopathy, and PRES.

 

Design/Methods:

A 49-year-old Caucasian female with a history of biopsy-proven, otherwise unspecified type of primary FSGS with nephrotic syndrome, CKD Stage IIIB with baseline creatinine of 1.5-1.7 mg/dl and hypoalbuminemia had a partial response to prednisone therapy. She developed worsening proteinuria and progressive kidney disease on Tacrolimus therapy and sub-optimal response to rituximab. She was started on Achtar gel 80 units twice weekly on top of the standard of care. She improved proteinuria from 9 grams/day to less than 4 grams/day in 3 months, and serum albumin has improved to 3.5 gr/dl.  

She was brought to the emergency department for altered mentation and generalized tonic-clonic seizures. Initial blood pressure readings in the emergency department showed systolic blood pressure in the 160s and diastolic blood pressure in the 90-110 range. MRI of the brain done during hospitalization established bilateral occipital and parietal hypoattenuation consistent with PRES syndrome.  
Results:

Our patient has stable chronic kidney disease and well-controlled hypertension at baseline with nephrotic syndrome in remission while on Achtar therapy. We believe PRES syndrome is likely contributed to by the cerebral vascular endothelial dysfunction caused by Achtar gel therapy on top of her comorbidities.  

Conclusions:
Patients on Achtar gel may be at increased risk for developing PRES syndrome, and the clinicians should be wary of this uncommon adverse event and its management.
10.1212/WNL.0000000000208437
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