REFRACTORY GRANULOMATOSIS WITH POLYANGIITIS FLARE? HOW ABOUT PLASMA EXCHANGE?

Abstract

SESSION TITLE: Medical Student/Resident Lung Pathology SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: ANCA-negative granulomatosis with polyangiitis (GPA) patients often have limited disease, but they may evolve to have life-threatening renal and pulmonary complications. In general, patients are treated with glucocorticoids and immunosuppression. We present a patient with GPA flare-up refractory to initial therapy who improved after undergoing plasma exchange. CASE PRESENTATION: A 78-year-old male with a past medical history of ANCA-negative GPA and chronic kidney disease (baseline Cr 2.5 mg/dL) presented to the hospital with dyspnea. He was discharged from the hospital one week prior where he was managed for a GPA flare-up (i.e., sudden onset bilateral lower extremity nonpalpable purpura) after he stopped methotrexate due to transaminitis. At that time, he was treated with pulse IV methylprednisolone and discharged home to begin rituximab. When he returned to the hospital with worsening dyspnea, vital signs were: BP 106/47, HR 83, RR 18, 93% O2 saturation on room air. His hospital course was complicated by copious amount of hemoptysis. Labs revealed: Hgb 5.2 g/dL (which dropped from 8.5 g/dL a week ago) and worsening renal function with BUN >150 mg/dL and Cr 5.1 mg/dL. Chest x-ray showed diffuse bilateral alveolar opacities. He had respiratory failure secondary to pulmonary hemorrhage, which led to intubation and transfusion of blood products. A decision was made to start the patient on seven sessions of plasma exchanges. The patient responded well to the therapy and was eventually extubated with pulmonary and renal function returning to baseline. DISCUSSION: In the past, plasma exchange in GPA was based upon the efficacy of removing autoantibodies in Goodpasture's syndrome. More recently, the PEXIVAS clinical trial demonstrated that plasma exchange neither reduced the risk of end-stage renal disease nor had a treatment effect on pulmonary hemorrhage in ANCA-associated vasculitis. Some statistical analyses regarding pulmonary hemorrhage had wide confidence intervals, suggesting that plasma exchange could still be useful. In addition, a kidney biopsy was not required to enter the PEXIVAS trial, making it difficult to delineate if mild or severe disease was present (as vasculitides patients often have a relapsing and remitting course). Those with minimal tubulointerstitial and glomerular scarring could theoretically benefit from plasma exchange. The patient in this case underwent plasma exchange as he was thought to be among a select subgroup whose benefits outweighed risks. CONCLUSIONS: The use of plasma exchange in vasculitides should be tailored according to the characteristics of each patient and the previous treatments that each patient received. Reference #1: 1. Pagnoux C. Updates in ANCA-associated vasculitis. Eur J Rheumatol. 2016; 3(3):122-133. DOI:10.5152/eurjrheum.2015.0043 Reference #2: 2. Walsh M et al. Plasma Exchange and Glucocorticoids in Severe ANCA-Associated Vasculitis. N Engl J Med. 2020; 382(7):622-631. DOI: 10.1056/NEJMoa1803537 Reference #3: 3. Jayne D.R.W et al. Randomized Trial of Plasma Exchange or High-Dosage Methylprednisolone as Adjunctive Therapy for Severe Renal Vasculitis. JASN. 2007; 18(7):2180-2188; DOI: 10.1681/ASN.20070100904. Derebail V, Falk R. ANCA-Associated Vasculitis -- Refining Therapy with Plasma Exchange and Glucocorticoids. N Engl J Med. 2020; 382:671-673. DOI: 10.1056/NEJMe1917490