Impact of pharmacy services on time to elexacaftor-tezacaftor-ivacaftor initiation.

BACKGROUND: The approval of elexacaftor-tezacaftor-ivacaftor (ELX/TEZ/IVA) expanded highly effective cystic fibrosis transmembrane receptor modulator therapy to approximately 90% of persons aged 12 years and older with cystic fibrosis. Clinical pharmacists and pharmacy technicians played a key role in planning for ELX/TEZ/IVA initiation prior to US Food and Drug Administration approval as well as initiating therapy after approval. OBJECTIVE: To evaluate the impact of pharmacy services on time to ELX/TEZ/IVA initiation. METHODS: A retrospective chart review evaluated 146 patients aged at least 12 years with cystic fibrosis qualifying for ELX/TEZ/IVA at a single health system between October 21, 2019, and April 1, 2020. RESULTS: Patients filling ELX/TEZ/IVA at an integrated health system specialty pharmacy (HSSP) vs an outside specialty pharmacy (SP) started on therapy an average of 10.8 days sooner (10.8 days ± 14.0 vs 21.6 days ± 18.8, respectively; P = 0.006). More patients filling at an HSSP received ELX/TEZ/IVA within 14 days of the prescription being written compared with outside SPs (82.0% vs 41.4%, respectively; P = 0.001). Before ELX/TEZ/IVA initiation, patients were hospitalized for a cystic fibrosis-related complication for an average of 6.26 days (range = 0-183) compared with 1.16 days (range = 0-91) after ELX/TEZ/IVA initiation. Lastly, an estimated $134,810 was saved in hospitalization dollars in the 105 patients that were able to fill ELX/TEZ/IVA at an HSSP by initiating the drug an average of 10.8 days sooner than outside SPs. CONCLUSIONS: The results of this study demonstrate the value of an integrated HSSP model. The ability to fill specialty medications at an integrated HSSP may optimize medication access, control costs, and improve patient outcomes for patients receiving care within a health system. DISCLOSURES: Dr Loucks has accepted payment for reviewing content of Lexicomp through Wolters Kluwer Consulting and for presenting and attending the American Society of Health System Pharmacists (ASHP) Summer Meeting in June 2022. Dr Loucks is also a Workgroup Chair for the ASHP Pharmacist Section of Specialty Pharmacy Practitioners - Section Advisory Group on Outcomes and Value. Dr Simonsen was a participant in the Vertex Pharmaceuticals Advisory Board in April 2019 and accepted payment for travel and expenses. The remaining authors have no conflicts of interest or financial interests to disclose. This work is in part supported by the Statistical Expertise and Network (StatNet) Award of Cystic Fibrosis Foundation.

Rheumatoid arthritis (RA)-specific autoantibodies in patients with interstitial lung disease and absence of clinically apparent articular RA.

The purpose of this study was to identify rheumatoid arthritis (RA)-related autoantibodies in subjects with interstitial lung disease (ILD) and no articular findings of RA, supporting the hypothesis that RA-related autoimmunity may be generated in non-articular sites, such as the lung. This was a retrospective chart review utilizing clinic databases of patients with ILD to identify cases with lung disease, RA-related autoantibody positivity, and no clinical evidence of articular RA. Four patients with ILD, RF, and anti-CCP positivity and no articular findings of RA were identified. All four patients were male with a mean age at time of diagnosis of ILD of 70 years old. All had a history of smoking. Three patients died within 2 years of diagnosis of ILD and never developed articular symptoms consistent with RA; the final case met full criteria for articular RA several months after stopping immunosuppressive treatment for ILD. RF and anti-CCP can be present in smokers with ILD without clinical evidence of articular RA and in one case symptomatic ILD and autoantibody positivity preceded the development of articular RA. These findings suggest that RA-specific autoimmunity may be generated due to immunologic interactions in the lung and may be related to environmental factors such as smoking.