TOPIC: Pulmonary Vascular Disease TYPE: Fellow Case Reports INTRODUCTION: Common Variable Immunodeficiency (CVID) is a disorder that is occasionally complicated by a rare form of interstitial lung disease: granulomatous lymphocytic interstitial lung disease (GLILD). Progression of GLILD may result in Group 3 pulmonary hypertension (PH). Group 1 PH is rare in CVID. We present a patient with CVID with group 1 PH treated with inhaled prostacyclin. CASE PRESENTATION: A 39-year-old woman with medical history of CVID treated with long-term intravenous immunoglobulin (IVIg) therapy, immune mediated thrombocytopenia (ITP) and chronic respiratory failure on 2 liters of supplemental oxygen presented with acute right ventricular (RV) failure and for evaluation of heart and lung transplant. She had worsening dyspnea. She was treated for pneumonia with sepsis. Computed Tomography Angiography (CTA) chest showed pulmonary artery dilation, septal thickening, bilateral reticulation and mild bilateral lower lobe traction bronchiectasis, with scattered pulmonary nodules. This was suggestive of GLILD. Echocardiogram showed a left ventricular ejection fraction of 60%, pulmonary arterial pressure of 78 mmHg, with trivial pericardial effusion and severe RV dysfunction. Right heart catheterization demonstrated, in mmHg, right atrial pressure of 2, mean pulmonary arterial pressure of 42, pulmonary capillary wedge pressure of 2, cardiac output and index of 2.83 and 1.71 respectively with pulmonary vascular resistance of 14 wood units, consistent with severe PAH. Pulmonary function tests showed severe restrictive disease. The degree of PVR elevation was higher than what was expected in the setting of diffuse parenchymal lung disease, suggesting a vasculopathic component beyond that attributable to group 3 disease. There was concern of potentially worsening ventilation perfusion (VQ) mismatch if treated with pulmonary vasodilators. The patient was deemed a poor transplant candidate due to ITP and was started on inhaled Treprostinil, and discharged home on tapering dose of steroids and supplemental oxygen. She was seen in PH clinic where she is was showing improved New York Heart Association functional class and was tolerating inhaled treprostinil with no worsening in hypoxia. DISCUSSION: CVID is a severe immunodeficiency that is caused by failure of antibody production. GLILD is the usual primary pulmonary parenchymal disease associated with CVID. Progression of GLILD can be further complicated by the development of PH which is usually classified as Group 3. Until recently, these patients did poorly. CONCLUSIONS: Group 3 PH is not treated with pulmonary vasodilator therapy. Our patient did well on inhaled prostacyclin, in the setting of parenchymal lung disease. This is attributed to a pulmonary vascular phenotype, similar to those with PAH. Treatment of Group 1 PH in this setting with inhaled prostacyclin could represent a novel frontier in the treatment of pulmonary vascular phenotype. REFERENCE #1: Huston J, Johnson J, Hemnes A, Pugh M. Evidence of pulmonary arterial hypertension in two patients with common variable immunodeficiency. Pulm Circ. 2020 May 1;10(2):2045894020922792. doi: 10.1177/2045894020922792. PMID: 32426112; PMCID: PMC7218961. REFERENCE #2: P501 Pulmonary hypertension rates in common variable immunodeficiency J. Farmer M, Ong S, Barmettler, J. WalterDOI:https://doi.org/10.1016/j.anai.2017.09.067 REFERENCE #3: Pulmonary hypertension in interstitial lung diseaseJ. Behr, J. H. RyuEuropean Respiratory Journal Jun 2008, 31 (6) 1357-1367; DOI: 10.1183/09031936.00171307 DISCLOSURES: No relevant relationships by ALAA ABU SAYF, source=Web Response No relevant relationships by Sara Hegab, source=Web Response Speaker/Speaker's Bureau relationship with Bayer Please note: 11/20/20-4/2021 Added 04/29/2021 by Reem Ismail, source=Web Response, value=Consulting fee No relevant relationships by Rajaninder Sharma, source=Web Response