TOPIC: Pulmonary Vascular Disease TYPE: Medical Student/Resident Case Reports INTRODUCTION: Pulmonary involvement in setting of common variable immunodeficiency (CVID) usually manifests as an airway disease and/or ILD. Development of severe pre-capillary PH is unusual. Herein, we present a case of severe PH associated with CVID ILD that was managed with combination therapy including inhaled treprostinil and was followed for 8 years. CASE PRESENTATION: A 34-year-old female with a diagnosis of CVID, on immunoglobulin therapy since the age of 10 years, and bronchiectasis presented in 2013 with worsening dyspnea, hypoxia, and lower extremities edema. Pro-BNP was elevated at 11,000 pg/mL and cardiac echocardiogram showed EF of 56%, severe RV dilation with severe hypokinesis, RVSP >120mm Hg, and moderate pericardial effusion. Chest CT with contrast showed bilateral areas of fibrosis with traction bronchiectasis without evidence of acute or chronic pulmonary emboli. After diuresis, RHC showed mRAP 4 mmHg, mPAP 54mmHg, PAWP 12 mmHg, CO 4.22L/min and PVR 9.95 WU. PFTs showed severe restriction, air trapping, and Dlco of 13%. The patients was referred for lung transplant evaluation but she was hesitant about transplantation. She was started on tadalafil 40 mg po daily and inhaled treprostinil that was titrated to 10 breaths QID. The patient noted subjective improvement in her dyspnea and at 1 year follow up the 6MWD improved from 303m to 465m with normalization of BNP to 27pg/ml and resolution of the pericardial effusion. Repeat RHC showed mRAP 1 mmHg, mPAP 32 mmHg, PAWP 5mmHg, CO 4.51 L/min, and PVR 5.9 WU. Over the last 8 years the patients continued vasodilator therapy with dose escalation of inhaled treprostinil (current dose of 16 breaths QID). Her last cardiac echo in 2021 showed normal RV size and function (TAPSE 28mm, and FAC 36%) and no pericardial effusion and the last RHC showed PVR 4.6 WU. The patient continues to be followed closely by transplant, ILD, and PH teams. DISCUSSION: Severe pre-capillary PH associated with CVID is not well described. We reported before, a patient with CVID without ILD who presented with severe PAH that required parenteral therapy which resulted in RV reverse remodeling and near normalization of hemodynamics. The case we are presenting here had severe pre-capillary PH associated with CVID but in setting of significant ILD with good response to pulmonary artery vasodilators including high dose inhaled treprostinil. Recently, the INCREASE trial provided the needed evidence to treat PH-ILD with inhaled treprostinil with doses up to 12 breaths QID. Here we present the unusual association between CVID and severe pre-capillary PH, describe the hemodynamic response to combination therapy that included high dose inhaled treprostinil in setting of ILD, and provide 8 years of follow up for this unusual case. CONCLUSIONS: CVID can be associated with severe pre-capillary PH. This association needs to be better characterized in the literature. REFERENCE #1: C. Salmon, K. El-Kersh, R. Kim, M. Taylor. Severe PAH Associated with CVID: Rapid Normalization with Upfront Triple Combination Therapy. American Journal of Respiratory and Critical Care Medicine 2020;201:A3755 DISCLOSURES: Speaker/Speaker's Bureau relationship with United therapeutics Please note: $5001 - $20000 by Karim El-Kersh, source=Web Response, value=Honoraria Removed 04/21/2021 by Karim El-Kersh, source=Web Response Advisory Committee Member relationship with United therapeutics Please note: $1001 - $5000 by Karim El-Kersh, source=Web Response, value=Consulting fee Removed 04/21/2021 by Karim El-Kersh, source=Web Response Advisory Committee Member relationship with Actelion Please note: $5001 - $20000 by Karim El-Kersh, source=Web Response, value=Consulting fee Removed 04/21/2021 by Karim El-Kersh, source=Web Response Advisory Committee Member relationship with United Therapeutics Please note: Current Added 04/23/2021 by Karim El-Kersh, source=Web Response, value=Consulting fee Advisory Committee Member relationship with Actelion Please note: 2019 and 2020 Added 04/23/2021 by Karim El-Kersh, source=Web Response, value=Consulting fee Speaker/Speaker's Bureau relationship with United Therapeutics Please note: Current Added 04/29/2021 by Karim El-Kersh, source=Web Response, value=Honoraria Advisory Committee Member relationship with United Therapeutics Please note: Current Added 04/29/2021 by Karim El-Kersh, source=Web Response, value=Honoraria Advisory Committee Member relationship with Actelion Please note: 2019 and 2020 Added 04/29/2021 by Karim El-Kersh, source=Web Response, value=Honoraria Consultant relationship with Acceleron Pharma Please note: Current Added 04/29/2021 by Karim El-Kersh, source=Web Response, value=Consulting fee No relevant relationships by Cristina Salmon, source=Web Response No relevant relationships by Nadine Sbaih, source=Web Response