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3. Steroid therapy in acute exacerbation of fibrotic interstitial lung disease.

Author

Koshy, Kavya, Barnes, Hayley, Farrand, Erica, and Glaspole, Ian

Subjects
IDIOPATHIC pulmonary fibrosis, PULMONARY fibrosis, LOGISTIC regression analysis, DISEASE exacerbation, LUNG transplantation, INTERSTITIAL lung diseases
Abstract

Background and Objective: Evidence for the benefit of steroid therapy in acute exacerbations (AEs) of idiopathic pulmonary fibrosis (IPF) is limited; however, they remain a cornerstone of management in other fibrotic interstitial lung diseases. This retrospective observational study assesses the effect of steroid treatment on in‐hospital mortality in patients with acute exacerbation of fibrotic interstitial lung disease (AE‐FILD) including IPF and non‐IPF ILDs. Methods: AE‐FILD cases over a 10‐year period were filtered using a code‐based algorithm followed by individual case evaluation. Binary logistic regression analysis was used to assess the relationship between corticosteroid treatment (defined as ≥0.5 mg/kg/day of prednisolone‐equivalent for ≥3 days within the first 72 h of admission) and in‐hospital mortality or need for lung transplantation. Secondary outcomes included readmission, overall survival, requirement for domiciliary oxygen and rehabilitation. Results: Across two centres a total of 107 AE‐FILD subjects were included, of which 46 patients (43%) received acute steroid treatment. The steroid cohort was of younger age with fewer comorbidities but had higher oxygen requirements. Pre‐admission FVC and DLCO, distribution of diagnoses and smoking history were similar. The mean steroid treatment dose was 4.59 mg/kg/day. Steroid use appeared to be associated with increased risk of inpatient mortality or transplantation (OR 4.11; 95% CI 1.00–16.83; p = 0.049). In the steroid group, there appeared to be a reduced risk of all‐cause mortality in non‐IPF patients (HR 0.21; 95% CI 0.04–0.96; p = 0.04) compared to their IPF counterparts. Median survival was reduced in the steroid group (221 vs. 520.5 days) with increased risk of all‐cause mortality (HR 3.25; 95% CI 1.56–6.77; p < 0.01). Conclusion: In this two‐centre retrospective study of 107 patients, AE‐FILD demonstrates a high risk of mortality, at a level similar to that seen for AE‐IPF, despite steroid treatment. Clinicians should consider other precipitating factors for exacerbations and use steroids judiciously. Further prospective trials are needed to determine the role of corticosteroids in AE‐FILD. This is a multicentre retrospective observational study comparing the outcomes of acute exacerbation of fibrotic ILD between steroid and non‐steroid treatment. Those who were administered steroids were younger, had fewer comorbidities but greater oxygen requirement and lower physiologic reserve. When adjusted for these variables, there appeared no survival benefit and potential increased risk of death in those who were given steroids. See relatededitorial [ABSTRACT FROM AUTHOR]

Published
2024
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7. Contributors

Author

Abdelazeem, Mohamed A., Abelman, Rebecca A., Kar, Sarah Abu, Afari, Maxwell Eyram, Ahmad, Ali, Ahmed, Hammood, Ahn, Benjamin J., Ahn-Horst, Rosa, Akoto, Shanice, AlAbdulhadi, Abrar, Aldras, Yoseph A., Ali, Fahad, Ali, Tanya, Alipanah-Lechner, Narges, Altunel, Erdem, Alzoebie, Lama, Anderson, Chelsie, Andrews, Christine B., Taylor, Kathryn Maire, Anthony, Anngene, Asaro, Joseph A., Asiedu, Daniel K., Astsaturov, Artem, Aulakh, Sudeep K., Authement, Matthew, Avasare, Rupali S., Babar, Tania B., Bachman, Emelia Argyropoulos, Baig, Muhammad, Bains, Ashika, Balaguera, Pedro, Balda, Javier, Bank, T. Caroline, Banwell, Brenda, Basman, Craig L., Bass, Crystal T., Bayefsky, Sarah D., Beausang, Jasjit, Belcher, Ren, Bell, Jennifer, Bellinghausen, Amy L., Bennett, Colleen E., Benninger, John W., Bergen, Michael A., Berger, Agatha, Berkowitz, Carol D., Berkowitz, Julia, Betageri, Omkar, Bhagat, Vicky H., Bharmal, Murtaza, Bhatt, Harikrashna B., Bhuptani, Prachi H., Bido-Medina, Richard O., Bielak, Kendall M.H., Bilodeau, Courtney Clark, Bird, Lisa, Bishop, Erin, Bitar, Ghamar, Blakeney, Craig, Block, Brian, Bochner, Risa, Bortz, Christina M., Bosse, Kristopher R., Bouton, Tara C., Bove, Mary E., Bowlby, Lynn A., Brady, Mark F., Bratman, Russell E., Brennan, Keith, Brown, Austin K., Brown, Emily C.B., Bui, Eric, Burke, Christine, Butler, William, Byanova, Katerina L., Byra, Marcin, Callanan, Tucker C., Campbell, Taylor, Cantos, Adriana, Canty, Ethan, Canty, Katherine W., Caraganis, Andrew, Carpenter, Randy S., Carragee, Eugene, Carrel-Lammert, Meredith, Carter, Ashlie Sewdass, Casillas, Ernesto, Jr., Castaneda-Guarderas, Ana, Castillo, Jorge J., Chait, Daniel, Chan, Philip A., Chang, Kenny, Chapman, Arlene B., Chatterjee-Paer, Sudeshna, Chaudhary, Richard S., Cheng, Vicky, Chilcote, Daniel, Chofay, Dana Ricci, Chou, Jonathan, Chou, Stella T., Choudhury, Natasha, Choxi, Shivali, Chua, Winona, Chubb, Rhiannon E., Cinelli, Michael P., Claman, David, Clark, Grace V., Clark, Seth, Clarke, Nathan, Clebak, Karl T., Cleland, Derrick, Co, Michael Lawrenz, Cohen, Debbie L., Cohen, Lisa, Congdon, Morgan, Constantinou, Maria, Corley, James Earl, III, Cosgrove, Abigail, Craine, Rebecca, Cristofaro, Patricia, Cruz, Aristides I., Cunningham, Karlene, Curtin, Alicia J., Cusano, Joseph, D’Alessandro, Frank B., Dado, Lynn, Dalela, Deepansh, Das, Abhijit, DaSilva, Manuel F., Davis, Mackenzie, De La Garza, Carlos, DeDent, Alison, Deeb, Maya, DeJong, Tyler, Desrochers, Colette, Diaz, Joseph A., Dickherber, Jason L., Do, Toan, Doo, Kathleen, Dove, James H., Dubanchet, Alexandre, Duh-Leong, Carol, Dutta, Roop, Dworkin, Myles, Dyster, Timothy G., Ebert, Eric, Edmund, Joseph, Eggers, Erica, Eisenhower, Christine, Ellsworth, Pamela, Escamilla-Ocañas, César E., Espinosa, Patricio Sebastian, Esqueda, Marisol, Everly, Eileen, Faber, Mark D., Faber, Rachel, Facer, Evan, Fadale, Paul D., Farahvash, Benyamin, Farias, Michael J., Farkas, Jon, Farrand, Erica, Fay, Kevin, Hernandez, Samantha Fernandez, Fernandez-Robles, Carlos, Ferreira, Jason D., Ferri, Fred F., Ferri, Heather, Fischer, Staci A., Fiske, Hannah, Velez Florez, Maria Camila, Font, Cristina, Forbes, Kellie C., Forcier, Michelle, Ford, Sydney, Fort, Frank G., Fort, Glenn G., Freeman, Nancy, Frisch, Daniel R., Fru, Relindis Azenwi, Fullenkamp, Lynn C., Gaffney, Michael, Garcia, Paloma Jolin, Ge, Connie, Gesthalter, Yaron B., Ghimire, Anil, Gibson, Christopher, Gidwani, Nisha H., Gil, Joseph, Gold, Raphaela, Goldburt, Alla, Goldsmith, Corey Elam, Gangadhara Rao Golla, Maheswara Satya, Gomez, Alexander, Gomez Slagle, Helen B., Greenfield, Morgan, Grisham, Lindsey Anne, Gu, Joey, Guardino, Cara E., Gugerty, Lindsay, Gul, Fahad, Gultawatvichai, Patan, Gunning, Samantha, Guo, Averill, Guo, David, Gupta, Simran, Guttadauria, Brittany, Guzman, Herodes, Gwinn, Meghan, Hadden, Ryan, Hafeez, Muhammad Ubaid, Hall, Hayden, Hall, Sarah, Handa, Sajeev, Handler, Stacy N., Hanna, James, Hanna, John, Harbord, Nikolas, Hardacker, Kyle, Hardesty, Anna, Haribhakti, Nirav, Harris, Daniel J., Hartnett, Davis A., Hartvigsen, Nathan, Hawkins, Brian, Heinle, Rachel Wright, Helm, Leesha A., Helm, Matthew F., Herlevich, Thomas, Hermez, Klodia M., Hilliard, Ross W., Hladky, Katherine, Hodges, Ashley, Hoffman, Ryan, Hoffman, R. Scott, Hogan, Dawn, Holiman, Jennifer J., Holland, N. Wilson, Holton, Siri M., Homer, Benjamin J., Hook, Benjamin E., Howell, Cody, Hsu, Raymond, Huang, Laurence, Hull, Mariam, Hume, Anne L., Hussain, Zilla, Huynh, Donny V., Hyder, Sarah, Ibrahim, Zainab, Ingraham, Caitlin, Inman, Nicholas J., Insalaco, Louis F., Jain, Rishubh, Jain, Vanita B.D., Jain, Vipul V., Jamal, Fariha, Jani, Kaushal, Janigian, Robert H., Jr., Jasuja, Sonia, Javier, Noelle Marie, Jetty, Vybhav, Johnson, Jennie E., Johnson, Michael P., Johnson, Steven D., Jolly, Angad, Jones, Amanda, Joshi, Shyam, Joudi, Kareem, Justice, Joshua, Kaddis, Timothy, Kalligeros, Markos, Karim, Omar, Karthikeyan, Vanji, Kartub, Benjamin, Kasper, Vania, Kass, Joseph S., Kass, Naomi R., Kasthuri, Viknesh S., Katz, Luca, Kaul, Bhavika, Kaur, Arpanjeet, Keim, Jennifer L., Keleshian, Vasken, Keogh, Abigail, Keselman, Dennis, Khan, Adeeba, Khare, Sarthak, Khawaja, Hussain R., Khetpal, Vishal, Khurana, Saranya S., Kifle, Betelhem, Kyung (Ellen) Kim, Eun, Kim, Jinseong, Kleitsch, Julianne, Knudson-Fitzpatrick, Tess, Kohrman, Samuel I., Kokkirala, Aravind Rao, Konstantino, Yuval, Kopyt, Nelson, Kothadia, Savan, Koti, Ajay, Koulouridis, Ioannis, Kreider, Timothy R., Krueger, Van S., Krumm, Ilana, Kumbar, Lalathaksha, Kuris, Eren O., Kurss, David I., L’Etoile, Nathan, Lam, Uyen T., Lam, William, Larijani, Mary, Le, Peter, Leard, Lorriana E., Leavitt, David A., Leba, Christopher T., Lee, Kachiu C., Lemme, Nicholas J., Li, Jian, Lieberman, Leora, Lim, Carol, Lin, Jason, Lippincott, Jordan, Liu, Jonathan, Logue, Teresa C., López, Gabriela, Lord, Kito Akin, Lucas, Mathew J., Lucier, David J., Jr., Ma’ayeh, Marwan, Maclean, Rachel L., MacMurdo, Maeve G., Madva, Elizabeth N., Mahani, Sahar, Makai, Gretchen, Malpica, Luis, Marcaccio, Stephen E., Marques Pinheiro, Ana Paula, Mazzaglia, Peter J., Mbuyi, Nadine N., McCulloh, Russell J., McDonald, Christopher L., McDonald-McGinn, Donna M., McGowen, Alexandra, McGraw, Katherine Elizabeth, McKenna, Jaclyn, McNamara, Kelly, Mead, Kelly C., Meehan, Caroline P., Meghani, Ozair K., Meleveedu, Kapil S., Mendez, Dianelys, Mendez-Miller, Megan, Mercado, Jorge, Mercado, Osvaldo, Merrill, Scott J., Merritt, Rory, Michaels, Marian G., Michel, Jeremy, Mikolasko, Brian D., Milner, John D., Min, May, Minami, Taro, Mitsuyama, Rei, Miyares, Laura, Mogla, Ankur, Mojarrab, Javad Najjar, Montgrain, Philippe, Moore, Michael Z., Moore, Shawn D., Morelli, Leah, Morrissey, Patrick J., Mughal, Aleem I., Mukkamalla, Shiva Kumar R., Mullersman, Katherine M., Mumtaz, Ahmad, Muraina, Fadeke, Mustafa, Ahmad, Myers, Ellen, Mylhousen, Emily, Nadella, Akash, Nadella, Mohnish, Nagpal, Nikita, Nandi, Minesh, Nasir, Uzma, Nasr, George H., Naveed, Asim, Neff, Robert, Neithardt, Adrienne B., Nelin, Timothy, Nestlerode, Christina, Ni, Samantha, Niazi, Azfar K., Nichols, Jonathan, Nokoff, Natalie, Noorbhai, Ibrahim Z.D., Novak, James E., Novarra, Austin, Nuss, Emily E., Oliver, Rebecca, Olszewski, Adam J., Onyeaka, Henry K., Orchowski, Lindsay M., Outlaw, Jasmine, Owens, Brett D., Oyelakin, Ayotomide, Pace, Paolo G., Palma-Zamora, Isaac, Pandya, Rachita, Pappas-Taffer, Lisa, Paranandi, Sneha, Parikh, Mihir, Park, Sara, Partin, Michael T., Patel, Birju B., Patel, Melissa D., Patel, Minta, Patel, Nima R., Patel, Pranav M., Patel, Vidhi, Patrick, Brett, Paudel, Shreedhar, Paxton, E. Scott, Peay, Celeste, Perazella, Mark A., Perez, David L., Hermina Perez, Jose J., Devon, Erin Pete, Pfeuti, Courtney, Pham, Lily C., Phelps-Polirer, Kendall, Phillips, Katharine A., Phillips, Oliver W., Phillips, Tara M., Piana, Lauren E., Portnoff, Brandon, Powell, Mitchell, Powers, Alexander, Prashar, Rohini, Premyodhin, Ned, Price, Dana, Priestley, Margaret, Provenza, Christian, Pulice, Christine, Punko, Diana, Puthawala, Imran, Qawi, Imrana, Qu, David, Quesada, Adrian, Quinn, Matthew, Rachu, Gregory S., Rajagopal, Amulya, Rathore, Bharti, Rathore, Ritesh, Raukar, Neha P., Ravindra, Lakshmi, Reagan, John L., Reardon, P.K., Reddy, Bharathi V., Reddy, Snigdha T., Reed-Weston, Anne, Reginato, Anthony M., Reichart, James P., Reus, Victor I., Rich, Harlan G., Rivera, Lauren Davis, Robertson, Melena J., Roby, Lauren, Roche, Meaghan, Morfin Rodriguez, Alejandra E., Romero Gómez, Perla M., Ron, Hayley A., Rosenbaum, Blake L., Rossi, Michael, Roussel, Breton, Rubin, David, Ruhstaller, Kelly, Rutstein, Beth H., Sadeghi, Noushine, Sahota, Ruchi Jalota, Saks, Emily, Sall, Jeffrey W., Salvi, Joshua D., Sanchez, Frank, Sanchez, Martha C., Sanka, Pranavi, Santhosh, Lekshmi, Santos, Linda Herrera, Sauck, Emily, Sayeed, Syeda M., Saylor, Leah, Schmitt, Phillip R., Sciscione, Anthony, Sederdahl, Bethany K., Seeber, Richard E., II, Sen, Aritra, Sewell, Rachel, Shaban, Hesham, Shah, Ankur, Shaikh, Aaron Ali, Sharma, Animesh, Sharma, Yuvraj, Sharp, Lydia, Sheikh, Wasiq, Sherman, Alexander, Shill, Jessica E., Shimanovsky, Alexei, Shlossman, Philip A., Siddiqui, Khawja A., Siegel, Alyssa, Sigman, Mark, Simon, Rebecca, Singh, Harinder P., Slingsby, Brett, Sloane, Amy, Smick, Alexandra H., Smith, Devon, Smith, Jeanette G., Smith, Matthew J., Sohaney, Ryann, Soi, Vivek, Soinski, Rebecca, Soler, Maria E., Soman, Sandeep, Song, Jeffrey S., Sorg, Emily, Sperati, C. John, Stanford, Nathan, Stennis, Seth Daly, Stephens, Michael S., Stern, Ella, Stockwell, Philip, Suh, Edward, Sun, Jennifer K., Sundaramoorthy, Anjali, Supanakorn, Varut, Suri, Priyasha, Sutter, Stephanie, Sweeney, Hannah, Takasaki, Kaoru, Takei, Ryosuke, Tan, Laren, Taylor, Alan, Taylor, Matthew, Taylor, S. Trevor, Tellioglu, Tahir, Testa, Edward J., Thomas, Anthony G., Thompson, Stefani, Tien, Alexandra Meyer, Tien, David Robbins, Tierney, Anna-Marie, Tiffany, Patrick, Toma, Helen, Triplett, Thomas M., Tryforos, Margaret, Tsaban, Gal, Tschoe, Christine, Tucci, Joseph R., Turock, Julia, Uduman, Junior, Uiterwyk, Sean H., Ukaegbu, Rebecca, Umanath, Kausik, Vakili, Babak, Van Kirk, Emily, Vemula, Ridhima, Venado, Aida, Vijayakumar, Shilpa, Vollum, Kathryn G., Vorel, Ethan, Walson, Ifeanyi, Wang, Angela, Wang, Jarey H., Weekley, Manuel Shayne, Weinberg, Marc S., Wendolowski, Stephen, Westreich, Roi, Whitaker, Colin, Wilhoite, Morgan, Wilks, Andrew, Wilson, Annise, Winikor, Jared M., Wininger, Bryce, Winschel, Julia M., Wolpaw, Adam J., Wolpert, Marlene Fishman, Wu, Perry, Wylie, John, Yaguchi, Grace K., Yang, Anna, Yao, Xuan, Yaseen, Muhammad, Yee, Jerry, Yesodharan, Gemini, Yeung, Jennifer, Yin, Chun H., Yip, Agustin G., Yu, Elizabeth An, Zambrano, Juliana, Zenlea, Talia, Zha, B. Shoshana, Zhang, Helen, Zhao, Leon, Zhou, Diana X., Ziaie, Navid, Zollman, Joshua W., Zorin, Danylo, and Zurek, Olivia

Published
2024
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8. Differences in Healthcare and Palliative Care Utilization at the End of Life: a Comparison Study Between Lung Cancer, COPD, and IPF

Author

Suen, Angela O., Bischoff, Kara, Iyer, Anand S., Radhakrishnan, Keerthana, Fenton, Cynthia, Singer, Jonathan P., Sudore, Rebecca L., Kotwal, Ashwin, and Farrand, Erica

Abstract

Patients with lung cancer, idiopathic pulmonary fibrosis (IPF), and chronic obstructive pulmonary disease (COPD) have high symptom burden, poor quality of life, and high healthcare utilization at the end of life. While proactive integration of palliative care in lung cancer can improve outcomes, it is unclear whether similar practices have been adopted in COPD and IPF care.

Published
2024
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9. Differences in Healthcare Utilization at the End of Life: a Comparison Study of Three Progressive Lung Diseases.

Author

Suen, Angela, Bischoff, Kara E., Kotwal, Ashwin, Sudore, Rebecca, and Farrand, Erica

Subjects
*LUNG diseases, *CHRONIC obstructive pulmonary disease, *SYMPTOM burden, *MEDICAL care, *INPATIENT care, *CANCER pain
Abstract

1. Understand differences in healthcare and palliative care utilization across lung cancer, COPD, and idiopathic pulmonary fibrosis. 2. Identify potential areas of unmet palliative care needs in patients with different non-cancer lung diseases. Patients dying of COPD and IPF have lower outpatient palliative care and opioid use and higher intensive care utilization when compared to those dying of lung cancer. Our findings suggest possible unmet palliative care needs in patients dying with COPD or IPF. Patients with lung cancer (LC), idiopathic pulmonary fibrosis (IPF), or chronic obstructive pulmonary disease (COPD) frequently report high symptom burden, poor quality of life, and have high healthcare utilization at the end of life. Palliative care integration in oncology has improved these outcomes in LC, however it is unclear whether similar practice has been adopted in COPD and IPF care. We compared rates of palliative care and healthcare utilization in the last six months of life in patients with LC, COPD, and IPF. We retrospectively identified 2,343 deceased patients with LC, COPD, or IPF with ≥1 outpatient or inpatient visit at UCSF. We used registry data or previously validated algorithms to identify patients with LC, COPD, or IPF. Outcomes included metrics of outpatient and inpatient healthcare utilization. We used multivariate logistic regression controlling for demographics/co-morbidities. Compared to the LC group, patients with COPD and IPF were more likely to be white, male, older at the time of death, with fewer comorbidities (p< 0.001). Patients with COPD and IPF compared to LC were less likely to receive outpatient palliative care (adjusted probability in LC: 30%, COPD: 13%, IPF: 19%, p< 0.05) or outpatient opioids (LC: 69%, COPD: 55%, IPF: 49%, p< 0.001). Patients with COPD and IPF were more likely to receive intensive care (LC: 29%, COPD: 52%, IPF: 59%, p< 0.001), mechanical ventilation (LC: 14%, COPD: 21%, IPF: 34%, p< 0.001), and have higher inpatient palliative care utilization (LC: 28%, COPD: 38%, IPF: 44%, p=0.002) in the last six months of life. Patients dying of COPD and IPF were less likely to receive outpatient palliative care and more likely to receive intensive care when compared to LC at the end of life. Our preliminary analysis suggests possible unmet outpatient palliative care needs in patients dying with COPD or IPF and should be further explored. Disease specific management / Patient Outcomes [ABSTRACT FROM AUTHOR]

Published
2024
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10. Phage Immunoprecipitation-Sequencing Reveals CDHR5 Autoantibodies in Select Patients With Interstitial Lung Disease.

Author

Upadhyay V, Yoon YM, Vazquez SE, Velez TE, Jones KD, Lee CT, Law CS, Wolters PJ, Lee S, Yang MM, Farrand E, Noth I, Strek ME, Anderson MS, DeRisi JL, Sperling AI, and Shum AK

Abstract

Objective: Interstitial lung diseases (ILDs) are a heterogeneous group of disorders that can develop in patients with connective tissue diseases. Establishing autoimmunity in ILD impacts prognosis and treatment. Patients with ILD are screened for autoimmunity by measuring antinuclear autoantibodies, rheumatoid factors, and other nonspecific tests. However, this approach may miss autoimmunity that manifests as autoantibodies to tissue antigens not previously defined in ILD., Methods: We use Phage Immunoprecipitation-Sequencing (PhIP-Seq) to conduct an autoantibody discovery screen of patients with ILD and controls. We screened for novel autoantigen candidates using PhIP-Seq. We next developed a radio-labeled binding assay and validated the leading candidate in 398 patients with ILD recruited from two academic medical centers and 138 blood bank individuals that formed our reference cohort., Results: PhIP-Seq identified 17 novel autoreactive targets, and machine learning classifiers derived from these targets discriminated ILD serum from controls. Among the 17 candidates, we validated CDHR5 and found CDHR5 autoantibodies in patients with rheumatologic disorders and importantly, patients not previously diagnosed with autoimmunity. Using survival and transplant free-survival data available from one of the two centers, patients with CDHR5 autoantibodies showed worse survival compared with other patients with connective tissue disease ILD., Conclusion: We used PhIP-Seq to define a novel CDHR5 autoantibody in a subset of select patients with ILD. Our data complement a recent study showing polymorphisms in the CDHR5-IRF7 gene locus strongly associated with titer of anticentromere antibodies in systemic sclerosis, creating a growing body of evidence suggesting a link between CDHR5 and autoimmunity., (© 2024 The Author(s). ACR Open Rheumatology published by Wiley Periodicals LLC on behalf of American College of Rheumatology.)

Published
2024
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