WHAT'S LUPUS GOT TO DO WITH IT? A CASE OF ACUTE HYPERCAPNIC RESPIRATORY FAILURE IN A PATIENT UNDERGOING KIDNEY TRANSPLANTATION

Abstract

SESSION TITLE: Medical Student/Resident Pulmonary Vascular Disease Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease that is classically associated with constitutional symptoms, joint pain, and erythematous rash. However, patients with SLE can also have multiple cardiopulmonary comorbidities, making management difficult. In this report we present a patient with severe pulmonary arterial hypertension (PAH) and obstructive lung disease due to SLE who experienced a brief arrest during kidney transplantation. CASE PRESENTATION: A 41-year-old female with PAH and end-stage renal disease (ESRD) secondary to SLE presented in 2015 with the following right heart catheter (RHC) findings: RA 5, PAP 73/24 (40), PCWP of 9 mmHg, cardiac index of 1.93 L/min, and PVR of 9.7 Wood units. Alternative causes of PAH were assessed and ruled out via standard diagnostic testing. Treatment was initiated with sildenafil and inhaled treprostinil, but side effects eventually necessitated transition to ambrisentan and selexipag. A repeat RHC in 2017 showed the following: RA 5, PAP 30/17 (21), PCWP of 12 mmHg, cardiac output 3.12 L/min, PVR of 1.8. These improvements strongly suggested a component of Group 1 PAH that responded well to therapy. The patient was listed for renal transplant, and in Feb 2018 this was attempted but aborted due to intraoperative difficulty ventilating, yielding acute hypercapnic respiratory failure and brief cardiac arrest. After recovery and hospital discharge, repeat evaluation showed stable echocardiogram and RHC findings, but severe obstructive lung disease (FEV1/FVC 54, FEV 1 of .92 (40%), RV 161%, and DLCO 57%). Because the pathophysiology of acute hypercapnic respiratory failure does not correspond with acute PAH exacerbation or right ventricular failure, we suspected that severe air trapping secondary to SLE-induced bronchial inflammation led to her intraoperative respiratory arrest. Treatment of obstructive lung disease was then initiated with a fluticasone/salmeterol inhaler and advanced to 500/50 dosage, which led to significant improvement in subsequent FEV1. The patient was then relisted for renal transplant and underwent successful renal transplantation in Oct 2019. She did not require hemodialysis following the transplant, recovered quickly, and was discharged. At this time, 6 months after surgery, she remains functional class II and continues to do very well in the posttransplant setting. DISCUSSION: In this report, despite successful management of our patient's PAH, it was only after treatment of her concurrent SLE-related bronchitis that she was able to successfully receive a renal transplant. CONCLUSIONS: SLE is a systemic condition that can have multiple concurrent cardiopulmonary abnormalities, including obstructive lung disease due to bronchial inflammation and pulmonary arterial hypertension. Therefore, it is important for clinicians to look carefully at treatment options for each related cardiopulmonary condition. Reference #1: Hachulla E, Jais X, Cinquetti G, et al. Pulmonary Arterial Hypertension Associated With Systemic Lupus Erythematosus. Chest. 2018;153(1):143-151. https://doi.org/10.1016/j.chest.2017.08.014https://doi.org/10.1016/j.chest.2017.08.014 Reference #2: Kimoto M, Sharma TS, Manzi S. 35 - Lupus and the Cardiopulmonary System. In: Wallace DJ, Hahn BH, eds. Dubois' Lupus Erythematosus and Related Syndromes (Ninth Edition). Content Repository Only!; 2019:418-433. https://doi.org/10.1016/B978-0-323-47927-1.00035-9 Reference #3: Cojocaru M, Cojocaru IM, Silosi I, Vrabie CD. Manifestations of systemic lupus erythematosus. Maedica (Buchar). 2011;6(4):330-336. DISCLOSURES: No relevant relationships by dan grinnan, source=Web Response No relevant relationships by Meagan Shinbashi, source=Web Response