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1072: PROLONGED NSIP-INDUCED RESPIRATORY FAILURE WITH RAPID IMPROVEMENT FOLLOWING CYCLOPHOSPHAMIDE

Introduction: Nonspecific interstitial pneumonia (NSIP) is characterized radiologically by ground glass opacification with a clinical course ranging from dyspnea to rapidly progressive hypoxemia and death. NSIP is associated with rheumatologic disease, exposure, HIV or can be idiopathic.Treatment is with corticosteroids though immunosuppressants, such as cyclophosphamide (CYC), are detailed in retrospective studies and case reports. We present a case of rapid improvement of recurrent refractory NSIP after IV CYC. Description: A 67 year old female with NSIP diagnosed on CT scan in 2017 was admitted to the ICU for acute hypoxemic respiratory failure (AHRF). One month prior to admission she was hospitalized with a CT scan showing exacerbation of her NSIP. She required tracheostomy and 25 days of extracorporeal membranous oxygenation. Treatment with high dose pulse steroids and mycophenolate mofetil yielded slow recovery and discharge to LTAC after completing a 24 hour trach mask trial. Seven days post LTAC admission she was transferred to our ICU for AHRF. Vital signs were within normal limits with vent setting of VT 450, FiO2 of 55%, and PEEP of 10. CTA chest showed interstitial thickening and ground glass consistent with NSIP progression. ABG was 7.34/68/72 with PaO2/FiO2 (p/f) ratio of 138. She was ventilated per ARDSnet protocol and placed on empiric HAP coverage. Given concern for NSIP flare steroids were increased from methylprednisone 30mg to 60mg IV BID. On Hospital day (HD) 2 diuresis resulted in p/f of 120 and a salvage ventilatory strategy with Volumetric Diffusive Respiration (VDR) was attempted without improvement. On HD 6 p/f increased to 235 however, she remained ventilator dependent on 40% FiO2 and 10 PEEP with SBT failure. She was started on 750mg IV CYC on HD 7. On HD 8, her ABG improved to 7.27/70/125 with p/f of 312 on stable vent settings. Four days post IV cyclophosphamide, HD 12, she was breathing spontaneously with FiO2 28, pressure support of 10, and PEEP of 8. ABG showed 7.37, 53, 80 and p/f of 285. She was transferred to the floor and weaned from the vent with tracheostomy removal 22 days later. Discussion: This case illustrates a uniquely robust response to IV CYC as a treatment of refractory respiratory failure due to idiopathic NSIP.