Pulmonary Fibrosis Quiz
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Shortness of breath
Dry cough
Persistent cough
Extreme fatigue
Dry tickly cough that won't go away
Difficulty breathing
Finger clubbing
Dry cough at night
Unexplained weight loss
Paroxysmal cough
Cough worse at night
Cough after exertion
Not seeing your symptoms? No worries!
What is Pulmonary Fibrosis?
A disease where the lungs become stiff from repeated damage. Causes include autoimmune disease (where the immune system attacks the body), medications, genetic abnormalities, and fine particle dust. There may not be symptoms initially, but most patients develop a dry cough and breathlessness as the condition worsens.
Typical Symptoms of Pulmonary Fibrosis
- Cough worsens after exercise
- Previously diagnosed with pulmonary hypertension
- Difficulty breathing
- Sudden, violent bursts of coughing
- Waking up at night struggling to breathe
- Dry cough
- History of connective tissue, autoimmune or rheumatic disease
- Cough
Diagnostic Questions for Pulmonary Fibrosis
Your doctor may ask these questions to check for this disease:
- Does your cough worsen after exercise?
- Have you been diagnosed with pulmonary hypertension?
- Are you experiencing difficulty breathing?
- Do you have sudden, violent bursts of coughing?
- Do you wake up at night struggling to breathe?
Treatment of Pulmonary Fibrosis
Unfortunately, this condition cannot be reversed. Treatment focuses on slowing lung scarring and improving lung function through exercises. In severe cases, breathing oxygen at home or lung transplants may be advised.
Reviewed By:
Phillip Aguila, MD, MBA (Pulmonology, Critical Care)
Dr. Aguila graduated from West Virginia University School of Medicine. He has trained in Pulmonary and Critical Care Medicine at The University of North Carolina in Chapel Hill and Internal Medicine at Medical College of Pennsylvania/Hahnemann University at Allegheny General Hospital in Pittsburgh Pennsylvania. He has served as Assistant Professor since 2010.
Eisaku Kamakura, MD (Pulmonology)
Dr. Kamakura graduated from the Tokyo Medical and Dental University, School of Dentistry, and the Niigata University School of Medicine. He trained at Yokosuka Kyosai Hospital and held positions in the Respiratory Medicine departments at Yokosuka Kyosai Hospital, Tokyo Medical and Dental University, Ome City General Hospital, and Musashino Red Cross Hospital. In 2021, he became the specially appointed assistant professor at the Department of General Medicine, Niigata University School of Medicine.
Content updated on Jul 29, 2024
Following the Medical Content Editorial Policy
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People with similar symptoms also use Ubie's symptom checker to find possible causes
- Shortness of breath when lying down
- Breathing out is easy but breathing in is difficult
- Wake up in the middle of the night with shortness of breath
- Feeling that I am suffocating and will die from the breathlessness
- Shortness of breath in the morning
- Short of breath because of rapid breathing
- Shallow breathing
- Breathing in is easy and breathing out is difficult
- Abnormal breathing and difficulty taking in deep breths
- Accelerated breathing
- Moving shoulders up and down to breathe
See full list
Symptoms Related to Pulmonary Fibrosis
Diseases Related to Pulmonary Fibrosis
FAQs
Q.
Is ECMO Enough? Why Your Lungs Are Failing & Medically Approved Next Steps
A.
ECMO can be life saving by taking over oxygenation while your lungs rest, but it is a bridge, not a cure; whether it is enough depends on whether the cause is reversible, such as ARDS, severe pneumonia, fibrosis, blood clots, or severe asthma, and on your overall organ function and response to treatment. There are several factors to consider, and medically approved next steps include targeted treatment of the cause, lung‑protective ventilation and proning, close monitoring for recovery, and timely consideration of transplant or palliative support if progress stalls. See below for crucial details on warning signs, timelines, and the exact questions to ask your care team that can shape your next steps.
References:
* Schmidt M, Combes A, Lebreton G, et al. Extracorporeal Membrane Oxygenation for Acute Respiratory Distress Syndrome: Current Evidence and Future Directions. J Clin Med. 2021 May 2;10(9):1969. PMID: 33925761.
* Gattinoni L, Camporota L, Marini JJ, et al. Advanced Therapies for Severe Acute Respiratory Failure Beyond ECMO. J Clin Med. 2021 May 26;10(11):2311. PMID: 34072381.
* Jobe NP, Mazza S, Oommen J, et al. Salvage strategies for refractory hypoxemia on veno-venous extracorporeal membrane oxygenation. Expert Rev Respir Med. 2021 Apr;15(4):447-456. PMID: 33261642.
* Tonna JE, Johnson LE, Sanchez PG. Weaning from Veno-Venous Extracorporeal Membrane Oxygenation: From Physiology to Practice. J Clin Med. 2021 Sep 1;10(17):3961. PMID: 34501306.
* Combes A, Schmidt M, Palmer E, et al. Extracorporeal membrane oxygenation for severe acute respiratory distress syndrome. Intensive Care Med. 2020 Dec;46(12):2422-2432. PMID: 33052309.
Q.
Struggling to Breathe? Why ARDS Fills Lungs With Fluid & Medical Next Steps
A.
ARDS is a life-threatening lung injury where intense inflammation makes the tiny blood vessels leaky, flooding the air sacs with fluid and blocking oxygen, so any sudden severe shortness of breath requires emergency care. In the hospital, treatment centers on oxygen support such as high-flow oxygen or a ventilator with lung-protective settings, rapid treatment of the cause like infection, cautious fluid management, and sometimes prone positioning; there are several factors that can change the right next steps for you, so see the complete details below.
References:
* Aschenbrenner, D. S., & O'Toole, L. P. (2018). Pathogenesis and Management of Acute Respiratory Distress Syndrome. *Mayo Clinic Proceedings*, *93*(8), 1145–1153. pubmed.ncbi.nlm.nih.gov/30089421/
* Matthay, M. A., Zemans, L. R., Zimmerman, G. A., Arabi, Y. M., Bos, L. D. J., Calfee, C. S., … Ware, L. B. (2019). Acute Respiratory Distress Syndrome. *Nature Reviews Disease Primers*, *5*(1), 18. pubmed.ncbi.nlm.nih.gov/30814472/
* Thompson, B. T., Moss, M., Raghavendran, K., Schoenfeld, D., & Ware, L. B. (2017). Acute Respiratory Distress Syndrome: Mechanisms and Novel Therapies. *Critical Care Clinics*, *33*(1), 1–11. pubmed.ncbi.nlm.nih.gov/27894498/
* Gattinoni, L., & Quintel, M. (2018). Fluid Management in Acute Respiratory Distress Syndrome. *Anesthesiology*, *129*(2), 332–341. pubmed.ncbi.nlm.nih.gov/29505417/
* Lewis, M. A., & Calfee, C. S. (2020). Acute Respiratory Distress Syndrome: A Clinical Update. *American Journal of Respiratory and Critical Care Medicine*, *202*(2), 183–193. pubmed.ncbi.nlm.nih.gov/32011928/
Q.
Short of Breath? Why Lungs Scar (Pulmonary Fibrosis) & Medical Steps
A.
Persistent shortness of breath can signal pulmonary fibrosis, where lung tissue scars and stiffens, reducing oxygen transfer; risks include long term dust or mold exposure, autoimmune disease, certain drugs or radiation, infections, smoking, and genetics. Diagnosis often involves a pulmonologist using history, exam, high resolution CT, lung function and blood tests, sometimes biopsy, and treatment aims to slow decline with antifibrotics such as pirfenidone or nintedanib, oxygen, pulmonary rehab, trigger removal, and occasionally transplant. There are several factors to consider and urgent warning signs to know, so see the complete guidance below for details that could change your next steps.
References:
* George PM, Wells AU, Jenkins RG. Pulmonary fibrosis: where are we and where are we going? Lancet Respir Med. 2020 Oct;8(10):1038-1049. doi: 10.1016/S2213-2600(20)30349-4. Epub 2020 Sep 10. PMID: 32919532.
* Richeldi L, Caminati A, Puxeddu E, De Lauretis A, Luppi F. Evolving concepts in the pathogenesis and treatment of idiopathic pulmonary fibrosis. J Intern Med. 2022 Nov;292(5):713-728. doi: 10.1111/joim.13575. Epub 2022 Sep 2. PMID: 36040180.
* Maher TM, Ofek O, Thannickal VJ. Idiopathic pulmonary fibrosis. Lancet. 2021 Apr 10;397(10283):1417-1431. doi: 10.1016/S0140-6736(21)00305-6. PMID: 33838392.
* Joshi A, Taniya T, Sun X. Molecular mechanisms and therapeutic targets for pulmonary fibrosis. Signal Transduct Target Ther. 2022 Feb 10;7(1):47. doi: 10.1038/s41392-022-00898-3. PMID: 35145022.
* Collard HR, Richeldi L, Ryerson CJ, Lederer DJ, Tsitouras K, Guler SA, Tanase AM, Stauffer JL, De Lauretis A, Valenzuela C, Volpi A, Martinez FJ. Defining and treating progressive fibrosing interstitial lung diseases: a European Respiratory Society/American Thoracic Society international research statement. Eur Respir J. 2020 Feb 27;55(2):1901069. doi: 10.1183/13993003.01069-2019. Print 2020 Feb. PMID: 31836615.
Q.
Short of Breath? Why Your Lungs Fill With Fluid & Medically Approved Next Steps
A.
Shortness of breath from fluid in the lungs is often pulmonary edema, most commonly from heart problems but sometimes from infections, lung injury, toxins, kidney disease, high altitude, or certain medicines. Watch for emergency red flags severe breathlessness, chest pain, blue lips, or pink frothy sputum that require immediate care, and know that treatment ranges from oxygen and diuretics to longer term heart failure care and prevention; see below for many more details on symptoms, diagnosis, and exactly which next steps to take now.
References:
* van den Heuvel JGFC, van Lier RJT, Visschers SJW, van den Heuvel AMH. Acute pulmonary oedema. Lancet. 2021 May 15;397(10287):1901. doi: 10.1016/S0140-6736(21)00388-3. PMID: 33994017.
* Teixidor IAJ, Sanchez JLR, Sanchez DMMRMN. Acute pulmonary edema. Curr Opin Crit Care. 2017 Aug;23(4):304-309. doi: 10.1097/MCC.0000000000000424. PMID: 28537966.
* Heidenreich PA, Bozkurt B, Aguilar D, Allen LA, Byun JM, Cico SM, Collins SP, Cowie MB, Doherty JU, Fonarow GC, Franke B, Hurwitz JL, Jackson SL, Lindenfeld J, Peterson PN, Rodriguez F, Schooley A, Stevenson LW, Westlake C, Cannon CP; American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines. 2022 AHA/ACC/HFSA Guideline for the Management of Heart Failure: A Report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines. J Am Coll Cardiol. 2022 May 3;79(17):e263-e421. doi: 10.1016/j.jacc.2021.12.012. Epub 2022 Apr 1. PMID: 35368936.
* Bhadra S, Dushyant DJ, Chakravarthy D, Jyoti D, Singh D, Hadagali P, Ram R, Jasuja K, Parikh J, Singh S, Desh BD, Patel ND, Rout AK, Behera RB, Prasad A, Thapa H, Babu M. Acute respiratory distress syndrome: epidemiology, pathophysiology, diagnosis, and management. Eur Respir Rev. 2019 Jun 30;28(152):190001. doi: 10.1183/16000617.0001-2019. PMID: 31249005.
* Matthay MA, Lickstein N, Matalon S, Bhattacharya J. Noncardiogenic Pulmonary Edema. Chest. 2021 May;159(5):2075-2088. doi: 10.1016/j.chest.2020.12.001. Epub 2020 Dec 10. PMID: 33359005.
Q.
Short of Breath? Why Your Lungs Are Scarring & Popcorn Lung Medical Next Steps
A.
Shortness of breath and a persistent dry cough can point to lung scarring from pulmonary fibrosis or popcorn lung, a rare small airway injury often linked to chemical or vaping exposure that typically does not improve with standard asthma inhalers. There are several factors to consider. Next steps usually include pulmonary function tests, a high resolution CT scan, oxygen checks, and cause-directed treatment like removing exposures, corticosteroids or antifibrotics, oxygen, and pulmonary rehab, with urgent care for rapidly worsening breathing, chest pain, or bluish lips; see the complete guidance below for key details that could change your next steps.
References:
* Cárdenas-Garcia J, Uribe-Salas FJ, Cuatecontzi-Xolaltenco L, et al. Bronchiolitis Obliterans: Updates on Diagnosis and Management. *Lung*. 2022;200(3):289-301. doi:10.1007/s00408-022-00518-w
* George PM, Ryerson CJ, McCormack FX. Diagnosis and Management of Idiopathic Pulmonary Fibrosis. *BMJ*. 2020;370:m2815. doi:10.1136/bmj.m2815
* Martinez FJ, Collard HR, Pardo A, et al. Interstitial Lung Disease: Diagnosis and Management. *Am J Respir Crit Care Med*. 2021;203(5):543-560. doi:10.1164/rccm.202009-3552CI
* Wang Y, Chen T, He M, et al. Environmental factors and the risk of interstitial lung disease. *Respir Med*. 2023;208:107129. doi:10.1016/j.rmed.2023.107129
* Maher TM, Chambers RC. Future directions in the treatment of fibrotic lung disease. *Eur Respir Rev*. 2022;31(165):220131. doi:10.1183/16000617.0131-2022
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Link to full study:
https://www.medrxiv.org/content/10.1101/2024.08.29.24312810v1References
Thannickal VJ, Toews GB, White ES, Lynch JP 3rd, Martinez FJ. Mechanisms of pulmonary fibrosis. Annu Rev Med. 2004;55:395-417. doi: 10.1146/annurev.med.55.091902.103810. PMID: 14746528.
https://www.annualreviews.org/doi/10.1146/annurev.med.55.091902.103810Meyer KC. Pulmonary fibrosis, part I: epidemiology, pathogenesis, and diagnosis. Expert Rev Respir Med. 2017 May;11(5):343-359. doi: 10.1080/17476348.2017.1312346. Epub 2017 Apr 10. PMID: 28345383.
https://www.tandfonline.com/doi/full/10.1080/17476348.2017.1312346King TE Jr, Pardo A, Selman M. Idiopathic pulmonary fibrosis. Lancet. 2011 Dec 3;378(9807):1949-61. doi: 10.1016/S0140-6736(11)60052-4. Epub 2011 Jun 28. PMID: 21719092.
https://www.thelancet.com/journals/lancet/article/PIIS0140-6736(11)60052-4/fulltextNoble PW, Barkauskas CE, Jiang D. Pulmonary fibrosis: patterns and perpetrators. J Clin Invest. 2012 Aug;122(8):2756-62. doi: 10.1172/JCI60323. Epub 2012 Aug 1. PMID: 22850886; PMCID: PMC3408732.
https://www.jci.org/articles/view/60323Chioma OS, Drake WP. Role of Microbial Agents in Pulmonary Fibrosis. Yale J Biol Med. 2017 Jun 23;90(2):219-227. PMID: 28656009; PMCID: PMC5482299.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5482299/