Background/Purpose: Therapeutic plasma exchange (TPE) is used in anti-neutrophil cytoplasmic antibody associated vasculitis (AAV) as adjunct treatment for severe disease. There is paucity of data as to the effectiveness and safety of TPE in children with ANCA associated vasculitis. There are different recommendations regarding the role of TPE in AAV and most studies are based on adult data. It is presumed that TPE procedure in pediatric patients can be technically challenging. The focus of this review is to study the safety of the TPE procedure when it is recommended for severe AAV with rapidly progressive glomerulonephritis (RPGN) and pulmonary hemorrhage patients in pediatric patients.

Methods: In this retrospective study, we reviewed a total of 93 TPE procedures that were performed on 18 patients during a 10-year period (2011-2021). 78% were females. 50% were Hispanic, 44% were Caucasian and 5% were African American.

Inpatient age ranged from 2 to 18 years with median age range of 13.1 years. Two patients at 2 years and 3 years of age with remaining patients between 9 and 18 years of age.

Indications for TPE were as follows: 5 patients with RPGN + diffuse alveolar hemorrhage (DAH), 11 patients with DAH, 1 patient with RPGN with no lung involvement and 1 patient was Antibody-mediated rejection (AMR) in transplant.

One patient received four TPE treatments in tandem to ECMO. 28% of total TPE procedures used only 25% albumin as replacement fluid.

88% of patients were critical in the ICU and remainder were on the pediatric floor. All patients received corticosteroids with additional immunomodulatory treatment including cyclophosphamide, rituximab, and IVIG.

Regarding adverse effects, hypocalcemia was defined by ionized calcium concentration below < 1 mm/L. Hypertension and hypotension criteria were defined by percentiles based on age of patient.

Results: Among a total of 88 TPE procedures, 16 adverse events were noted (18%). 11 episodes were allergic reactions manifested as hives without anaphylaxis, itching and mild SOB (12.5%). Two TPE procedures were stopped due progressive hives/itching. Hypotension (1) and hypertension (2) occurred. Two episodes of catheter access issues occurred, one of which led to discontinuation of TPE due to occlusive thrombus surrounding tip catheter. No episodes of symptomatic hypocalcemia or bleeding due to hypofibrinogenemia occurred.

Median eGFR (measured in mL/min/1.73m2) pre-TPE was 82.9, post-TPE was 81, at 1-month was 79.5 and at 3-months was 78. Out of 18 patients, one patient died. No TPE-associated mortality was noted.

Conclusion: Our cohort showed that TPE was a well-tolerated and safe adjunctive treatment for severe AAV in pediatric patients. Further study on a large-scale pediatric population is needed to better define its role and safety.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/safety-of-plasmapheresis-as-an-adjuvant-therapy-in-severe-pediatric-anti-neutrophil-cytoplasmic-antibody-associated-vasculitis-a-single-center-cohort/