OUTCOMES OF PRIMARY PULMONARY HYPERTENSION WITH CONNECTIVE TISSUE DISORDERS VS OTHER PRIMARY PULMONARY HYPERTENSION: AN ANALYSIS OF A NATIONAL INPATIENT SAMPLE

Abstract

SESSION TITLE: Pulmonary Manifestations of Systemic Diseases SESSION TYPE: Original Investigations PRESENTED ON: 10/18/2022 2:45 pm - 3:45 pm PURPOSE: Primary pulmonary arterial hypertension (PAH) serves as one of the serious complications of connective tissues disorders (CTDs). It has a detrimental effect on the CTD patients’ quality of life and mortality. Namely, the prevalence of PAH in systemic sclerosis (SSc) patients is as high as 12% and is associated with a less favorable outcome compared to idiopathic PAH. There has also been growing evidence that suggests an early treatment of PAH in CTDs may prevent irreversible pathologic changes in pulmonary vessels. However, the morbidity and mortality of PAH in non-SSc CTDs remains largely unknown due to the lack of large cohort studies. The aims of this study were to compare the outcomes of patients primarily admitted for primary PAH with and without CTDs. METHODS: We used the National Inpatient Sample (NIS) database for the years 2015 to 2018 to analyze all hospital admissions for primary PAH (ICD-10 CM code I27.0) with versus without CTDs. We selected CTDs most commonly associated with primary pulmonary hypertension: systemic sclerosis, systemic lupus erythematosus, mixed CTD, rheumatoid arthritis, dermatomyositis and sjögren’s syndrome. We compared baseline characteristics, outcomes and healthcare utilization between the two groups. RESULTS: From 2015 - 2018, a total of 3,010,994 patients were included in the study group. 10.9% (5479) of the study population had the CTDs. The cohort with CTDs had slightly older mean age (59.61 vs 56.42, p=0.04), more women (91.2% vs 75.5%, p<0.001), higher number of Charlson comorbidity index of more than 4 (20.2% vs 16.3%, p<0.001), but less diagnosis of diabetes (13.5% vs 31.2%, p<0.001). After adjusting for age, race, hospital regions and other comorbid conditions, the odds ratio (aOR) for mortality in patients with CTDs compared to those without CTDs was slightly increased to 1.09, albeit not significantly (95% CI: 0.46 - 2.59, p=0.838). Similarly, there existed no statistically significant differences in length of stay (LOS) (9.2 vs 8.1 days; p=0.831) or total hospital charges ($137,212 vs $117,221; p= 0.858) between the two groups. CONCLUSIONS: Hospitalizations for primary PAH with CTDs had comparable mortality, LOS, total hospital charges compared to those without CTDs. CLINICAL IMPLICATIONS: The results of the study suggest non-significant mortality differences between primary PAH associated with CTDs and those without CTDs. A large cohort study of CTD-associated PAH would be essential as the management and outcomes may differ from those of other pulmonary hypertension. DISCLOSURES: No relevant relationships by Iriagbonse Asemota No relevant relationships by Cyra-Yoonsun Kang No relevant relationships by Sandhya shri Kannayiram No relevant relationships by parnia khamooshi No relevant relationships by Viviana Reyes Pinzon