Administrative claims studies do not adequately distinguish pulmonary arterial hypertension (PAH) from other forms of pulmonary hypertension (PH). Our aim is to develop and validate a set of algorithms using International Classification of Diseases, Ninth Revision, Clinical Modification (ICD-9-CM) codes and electronic medical records (EMR), to identify patients with PAH. From January 2012 to August 2015, the EMRs of patients with ICD-9-CM codes for PH with an outpatient visit at the University of Texas Medical Branch were reviewed. Patients were divided into PAH or non-PAH groups according to EMR encounter diagnosis. Patient demographics, echocardiography, right heart catheterization (RHC) results, and PAH-specific therapies were assessed. RHC measurements were reviewed to categorize cases as hemodynamically determined PAH or not PAH. Weighted sensitivity, specificity, and positive and negative predictive values were calculated for the developed algorithms. A logistic regression analysis was conducted to determine how well the algorithms performed. External validation was performed at the University of Virginia Health System. The cohort for the development algorithms consisted of 683 patients with PH, PAH group (n = 191) and non-PAH group (n = 492). A hemodynamic diagnosis of PAH determined by RHC was recorded in the PAH (26%) and non-PAH (3%) groups. The positive predictive value for the algorithm that included ICD-9-CM and PAH-specific medications was 66.9% and sensitivity was 28.2% with a c-statistic of 0.66. The positive predictive value for the EMR-based algorithm that included ICD-9-CM, EMR encounter diagnosis, echocardiography, RHC, and PAH-specific medication was 69.4% and a c-statistic of 0.87. A validation cohort of 177 patients with PH examined from August 2015 to August 2016 using EMR-based algorithms yielded a similar positive predictive value of 62.5%. In conclusion, claims-based algorithms that included ICD-9-CM codes, EMR encounter diagnosis, echocardiography, RHC, and PAH-specific medications better-identified patients with PAH than ICD-9-CM codes alone.
BackgroundIsolated cases of pulmonary arterial hypertension (PAH) with interferon α or β therapy have been reported, but no population-based estimates of the incidence of the disease after interferon exposure are available. The aim of this study was to determine the incidence of PAH after initiation of interferon therapy, using a large commercial insurance database.MethodsUsing National Drug Codes (NDCs) and Healthcare Common Procedure Coding System (HCPCS) codes, we utilized the Clinformatics™ Data Mart (CDM) database to identify subjects between 20 and 65 years old who received α or β interferon therapy between April 2001 and December 2012. Patients were followed from one year prior to the first medication claim for interferon to the first diagnosis of pulmonary hypertension using ICD-9-CM codes 416.0 and 416.8, or disenrollment. In those subjects diagnosed with pulmonary hypertension, a prescription for PAH-specific medications was used as a surrogate endpoint.ResultsWe identified 20,113 subjects who received interferon therapy during the study period. The median follow-up was 20 months. Pulmonary hypertension occurred in 71 subjects, and PAH-specific medications were prescribed to 7 of these subjects.ConclusionAlthough our analysis showed that the development of PAH is a rare event with interferon therapy, the risk of developing the disease is several fold higher than that for the general population.
Background: Isolated cases of pulmonary arterial hypertension (PAH) with interferon α or β therapy have been reported, but no population-based estimates of the incidence of the disease after interferon exposure are available. The aim of this study was to determine the incidence of PAH after initiation of interferon therapy, using a large commercial insurance database.
Methods: Using National Drug Codes (NDCs) and Healthcare Common Procedure Coding System (HCPCS) codes, we utilized the Clinformatics™ Data Mart (CDM) database to identify subjects between 20 and 65 years old who received α or β interferon therapy between April 2001 and December 2012. Patients were followed from one year prior to the first medication claim for interferon to the first diagnosis of pulmonary hypertension using ICD-9-CM codes 416.0 and 416.8, or disenrollment. In those subjects diagnosed with pulmonary hypertension, a prescription for PAH-specific medications was used as a surrogate endpoint.
Results: We identified 20,113 subjects who received interferon therapy during the study period. The median follow-up was 20 months. Pulmonary hypertension occurred in 71 subjects, and PAH-specific medications were prescribed to 7 of these subjects.
Conclusion: Although our analysis showed that the development of PAH is a rare event with
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