BLOODSHED TREATED SUCCESSFULLY IN A HOUSE OF VILLAINS: A CHALLENGING AND UNUSUAL CASE OF HEMOPTYSIS IN A PATIENT WITH CONNECTIVE TISSUE DISEASE- PULMONARY ARTERIAL HYPERTENSION

Abstract

SESSION TITLE: Medical Student/Resident Chest Infections Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Pulmonary arterial hypertension (PAH) is a progressive disease caused by a remodeling of precapillary arterioles that leads to a progressive increase in pulmonary vascular resistance and right ventricular failure. PAH can complicate any connective tissue disease, most frequently SSc as discussed above, but also SLE, MCTD, RA, or other diseases such as Sjogren’s syndrome (SS) and dermatomysositis with often a poor prognosis. Our case illustrates a case of Hemoptysis under uniquely complex circumstances. CASE PRESENTATION: 53-year-old Hispanic female initially presented with features of severe Acute right heart failure, cardiogenic shock and Hypoxemia. Pulmonary arterial hypertension (PAH) diagnosis was made and was stabilized on emergent parenteral prostacyclin therapy. We also found connective tissue disease (CTD) with Sjogren’s features. She had history of recurrent Venous thromboembolism (VTE) and was on rivaroxaban. PAH therapy - SQ Treprostinil (@ 60ng/kg/min) + tadalafil 40mg qd. A few months later, she developed (CNPA). She responded well to voriconazole with clinical & radiographic improvement. A significant cutaneous allergic reaction to voriconazole led to its cessation and substitution with posaconazole. She presented with large volume hemoptysis with preceding minor episodes for several days. She was hemodynamically stable with no constitutional symptoms. CT chest revealed a stable appearing right upper lobe pulmonary mass measuring 5.5 x 3.0 cm. BNP was 67. Anticoagulation was held and an IVC filter was placed emergently. Bronchoscopy revealed necrotic debris, tan purulent mucous, and blood in the anterior segment of the right upper lobe. Interventional radiology performed bronchial and subclavian angiography with chemoembolization (small branch of the right subclavian artery & the distal portion of the right bronchial artery) of the Aspergilloma. Anticoagulation was resumed after with no further episodes of hemoptysis and she was successfully discharged. DISCUSSION: In a review of patients with SS & PAH, the mean age at diagnosis of PAH was 50 and all female with severe functional class (FC III and IV) and hemodynamic impairment (1), strikingly similar to our case. Survival rate was low (66% at 3 years) as standard therapy of PAH was typically ineffective despite an initial improvement. However, Significant clinical improvement was noted in our patient with Parenteral prostacyclin therapy and PDE5 inhibitors. CONCLUSIONS: This case illustrates a complex unique clinical situation that was challenging – Hemoptysis from CNPA with a background of the following active diagnoses: PAH, CTD (Sjogren’s features), Recurrent VTE. Chemoembolization of feeding arteries + IVC filter placement + temporary cessation of anticoagulation was used with a successful outcome. Reference #1: Sato T, Hatano M, Iwasaki Y, et al. Prevalence of primary Sjögren's syndrome in patients undergoing evaluation for pulmonary arterial hypertension. PLoS One. 2018;13(5):e0197297. Published 2018 May 15. doi:10.1371/journal.pone.0197297 DISCLOSURES: Clinical Research - PI relationship with United Therapeutics Please note: 3 years Added 05/08/2020 by Vijay Balasubramanian, source=Web Response, value=Grant/Research Support Speaker relationship with Bayer Please note: 3 years Added 05/08/2020 by Vijay Balasubramanian, source=Web Response, value=Honoraria Speaker/Speaker's Bureau relationship with Boehringer Ingelheim Please note: $1001 - $5000 Added 05/08/2020 by Vijay Balasubramanian, source=Web Response, value=Honoraria No relevant relationships by Eric Reid, source=Web Response No relevant relationships by James Yang, source=Web Response