Posterior reversible encephalopathy syndrome (PRES) and hemolytic anemia: two severe and unusual complications of acute post-streptococcal glomerulonephritis

Abstract

The most frequent cause of nephritic syndrome in the pediatric population is acute post-infectious glomerulonephritis (PIGN). A rare complication is posterior reversible encephalopathy syndrome (PRES), characterized by subcortical vasogenic cerebral edema associated with variable neurological symptoms. The development of autoimmune hemolytic anemia is an atypical clinical presentation.

Objective: To report the coexistence of two unusual and serious extrarenal complications of PIGN and to discuss potential mechanisms involved in their development. 

Clinical Case: A 4-year-old male patient, with a 5-day history of hematuria and edema, headache, nausea, and vomiting. He was admitted in convulsive status and hypertensive crisis, laboratory showed C3 hypocomplementemia and high titers of Antistreptolysin O, which was interpreted as PIGN. Due to the presence of encephalopathy, PRES secondary to hypertensive emergency was suspected, which was confirmed by brain MRI. He also presented autoimmune hemolytic anemia, with hemoglobin up to 5 g/dL. The treatment was based on antihypertensive therapy, neuroprotective measures, and steroid treatment. He was discharged 31 days after hospitalization and remained asymptomatic 6 months after dischar- ge.

Conclusions: There must be a high suspicion index of PRES before the appearance of nonspecific neurological symptoms during the evolution of a PIGN. The existing anemia on occasion can be autoimmune.