Idiopathic Pulmonary Fibrosis Associated with Pulmonary Hypertension: A Case Report on Diagnostic Challenges and Follow-Up Recommendations
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Background:
Idiopathic Pulmonary Fibrosis (IPF) is an insidious continuous interstitial lung disease presenting histologically with fibrous deposition, and the linings of alveolar sac in the lungs leading to respiratory failure. Pulmonary hypertension (PH) is one complication of IPF that often exacerbates symptoms and worsens prognosis. The association between IPF and PH still necessitates improved diagnostic approaches and tailored treatment approaches.
Case Presentation:
A 60-year-old male presented with complaints of severe dyspnea of severe dyspnea, tiredness, and orthopnea. Objective examination showed acrocyanosis, JVP elevation, and loud P2. On the chest radiograph and HRCT of the lungs, showed interstitial infiltrates with honeycombing and traction bronchiolectasis, which confirmed IPF. Echocardiogram and right heart catheterization confirmed PH, with the mean pulmonary arterial pressure recording at 45mmHg, while lung function tests showed decreased FVC, TLC and DLCO.
Outcome:
The patient was initiated on antifibrotic therapy, pirfenidone and pulmonary vasodilator (sildenafil). Over a six-month period, patient showed improvements in exercise capacity, dyspnea, and oxygenation. Prompt recognition and individualized treatment approach is needed in patient with pulmonary hypertension (PH) and idiopathic pulmonary fibrosis (IPF) progress.
Conclusion:
The patient herein highlights the importance of early identification and intervention of PH in IPF patients. A multidisciplinary approach with vasodilators and antifibrotic therapy are needed to improve symptoms, slow disease progression and improving on quality of life.
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1. Lynch, J. P. III, Huynh, R. H., & Fishbein, M. C. (2016). Idiopathic pulmonary fibrosis: Epidemiology, clinical features, prognosis, and management. Thieme Medical Publishers.
Retrieved from https://thieme-connect.com
2. Ryu, J. H., Colby, T. V., & Hartman, T. E. (1998). Idiopathic pulmonary fibrosis: Current concepts. Mayo Clinic Proceedings, 73(5), 451-457.
https://doi.org/10.1016/S0025-6196(11)63448-1
3. Noble, P. W., & Homer, R. J. (2004). Idiopathic pulmonary fibrosis: New insights into pathogenesis. Clinics in Chest Medicine, 25(4), 737-750.
https://doi.org/10.1016/j.ccm.2004.08.006
4. Lederer, D. J., & Martinez, F. J. (2018). Idiopathic pulmonary fibrosis. New England Journal of Medicine, 378(19), 1811-1823.
https://doi.org/10.1056/NEJMra1616348
5. Zisman, D. A., Keane, M. P., Belperio, J. A., Strieter, R. M., & Fishman, J. A. (2005). Pulmonary fibrosis. Research: Methods and Reviews, 41(5), 491-503.
https://doi.org/10.1007/978-1-4020-3065-0_31
6. Poletti, V., Ravaglia, C., Buccioli, M., Tantalocco, P., et al. (2013). Idiopathic pulmonary fibrosis: Diagnosis and prognostic evaluation. Respiration, 86(3), 180-191.
https://doi.org/10.1159/000350257
7. Lynch, J. P., & Mahidhara, R. (2016). Idiopathic pulmonary fibrosis. Interstitial Pulmonary Disease: A Textbook of Interstitial Lung Disease, 6, 302-318.
https://doi.org/10.1201/b16032-17
8. Coultas, D. B., & Hubbard, R. (2003). Epidemiology of idiopathic pulmonary fibrosis. Idiopathic Pulmonary Fibrosis, 14(1), 3-9.
https://doi.org/10.3109/00122130309029588
9. Munchel, J. K., & Shea, B. S. (2021). Diagnosis and management of idiopathic pulmonary fibrosis. Rhode Island Medical Journal, 104(6), 233-237.
Retrieved from https://rimed.org
10. Swigris, J. J., Kuschner, W. G., Kelsey, J. L., Gould, M. K., & Stoller, J. K. (2005). Idiopathic pulmonary fibrosis: Clinical aspects and prognosis. Chest, 127(5), 2167-2175.
https://doi.org/10.1378/chest.127.5.2167
11. Sgalla, G., Biffi, A., & Richeldi, L. (2016). Idiopathic pulmonary fibrosis: Diagnosis, epidemiology, and natural history. Respirology, 21(5), 892-902.
https://doi.org/10.1111/resp.12752
12. Munchel, J. K., & Shea, B. S. (2021). Diagnosis and management of idiopathic pulmonary fibrosis: Implications for respiratory care. Respiratory Care, 66(1), 54-63.
https://doi.org/10.4187/respcare.07123
13. Du Bois, R. M. (2011). Idiopathic pulmonary fibrosis: Present understanding and future options. European Respiratory Review, 20(121), 166-171.
https://doi.org/10.1183/09059180.00007911
14. Oldham, J. M., & Noth, I. (2014). Idiopathic pulmonary fibrosis: Early detection and referral. Respiratory Medicine, 108(5), 696-705.
https://doi.org/10.1016/j.rmed.2013.12.020
15. Pérez, E. R. F., Daniels, C. E., Sauver, J. S., & Hartman, T. E. (2010). Incidence, prevalence, and clinical course of idiopathic pulmonary fibrosis: A population-based study. Chest, 138(3), 625-633.
https://doi.org/10.1378/chest.09-3177
16. Prasad, R., Gupta, N., Singh, A., & Gupta, P. (2015). Diagnosis of idiopathic pulmonary fibrosis: Current issues. Intractable & Rare Diseases Research, 4(2), 52-60.
https://doi.org/10.14341/irdr2015139
17. Dempsey, O. J., Kerr, K. M., & Gomersall, L. (2006). Idiopathic pulmonary fibrosis: An update. Journal of the Royal College of Physicians of Edinburgh, 36(4), 275-280.
https://doi.org/10.4997/JRCPE.2006.400
18. Meier-Sydow, J., Weiss, S. M., & Buhl, R. (1994). Idiopathic pulmonary fibrosis: Current clinical concepts and challenges in management. Journal of Respiratory and Critical Care Medicine, 149(4), 1056-1065.
https://doi.org/10.1164/ajrccm.149.4.7515649
19. Oldham, J. M., Moua, T., & Daniels, C. E. (2014). Idiopathic pulmonary fibrosis: Evolving concepts. Mayo Clinic Proceedings, 89(4), 516-528.
https://doi.org/10.1016/j.mayocp.2014.01.017
20. Raghu, G., & Chang, K. (2004). Idiopathic pulmonary fibrosis: Current trends in management. Clinics in Chest Medicine, 25(4), 627-643.
https://doi.org/10.1016/j.ccm.2004.08.004
21. Zisman, D. A., Schwarz, M., Anstrom, K. J., Collard, H. R., Flaherty, K. R., & Hunninghake, G. W. (2010b). A controlled trial of sildenafil in advanced idiopathic pulmonary fibrosis. New England Journal of Medicine, 363(7), 620–628. https://doi.org/10.1056/nejmoa1002110
22. Han, M. K., Bach, D. S., Hagan, P. G., Yow, E., Flaherty, K. R., Toews, G. B., . . . Martinez, F. J. (2013). Sildenafil preserves exercise capacity in patients with idiopathic pulmonary fibrosis and right-sided ventricular dysfunction. CHEST Journal, 143(6), 1699–1708. https://doi.org/10.1378/chest.12-1594
23. Harari, S., Elia, D., & Humbert, M. (2017). Pulmonary hypertension in parenchymal lung diseases. CHEST Journal, 153(1), 217–223. https://doi.org/10.1016/j.chest.2017.06.008
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