Thank you to our volunteer Dr. Mohamed Elnaggar for contributing to this blog:
Dr. Mohamed Elnaggar is a physician in the field of Pulmonary and Critical Care Medicine. He serves as an Associate Clinical Professor of Medicine at the University of California, Irvine (UCI). Dr. Elnaggar is also the Associate Program Director for the Pulmonary/Critical Care Fellowship at UCI. He is also a Staff Physician within the Pulmonary/Critical Care Division at Long Beach VA medical center.
This month, Dr. Elnaggar met with our nonprofit’s Director, Neha Shah, PhD to discuss the community’s frequently asked questions about PF diagnosis, treatment options, and hospitalizations. Together, they created the list below of questions and answers. Please consult with your own medical team if you have specific questions about your personal treatment plan.
PF NOW!’s list of commonly asked community questions for a pulmonologist:
Diagnosis & Early Evaluation
How is pulmonary fibrosis (PF) diagnosed? What tests are necessary?
To diagnose pulmonary fibrosis we need to look at the lung anatomy/structure with a high resolution CT (HRCT) of the Chest and assess lung function, by performing Pulmonary Function Tests (PFT). We also do blood work screening for any auto-immune disease that might be causing the fibrosis.
Regarding blood work at diagnosis: this can include (1) HP panel (hypersensitivity pneumonitis panel); (2) various autoimmune panels to test for conditions such as lupus, RA, systemic sclerosis, connective tissue disease, scleroderma, myositis and (3) inflammatory markers such as ESR (erythrocyte sedimentation rate), CRP (C-reactive protein).
We also obtain a detailed exposure history in an attempt to identify the cause of PF. Sometimes, we need to do lung biopsy as well.
What conditions should first be ruled out in cases of idiopathic pulmonary fibrosis (IPF)?
IPF can be diagnosed with CT, and a lot of times we don’t need to do biopsy for IPF, if we have the typical radiological findings.
There are other causes of pulmonary fibrosis that can look like IPF, that we need to rule out mainly by obtaining history and checking labs. Examples of those causes are: Hypersensitivity pneumonitis (typically related to bird exposure), medication induced (known medication to cause this is Amiodarone used for heart rate control), radiation to the chest (to treat lung cancer) and PF related to Rheumatoid arthritis.
What factors contribute to delayed diagnosis, and what can patients do to self-advocate and expedite the process?
Symptoms are non-specific (like shortness of breath, cough and fatigue which could be present in other lung diseases). If you have unresolving symptoms – especially, if you have “risk for pulmonary fibrosis” as discussed here – you should talk to your doctor and inquire about fibrosis as a possible cause of your symptoms.
What are the risks of misdiagnosis?
Misdiagnosis can delay treatment options. Treatment might differ based on the known causes of pulmonary fibrosis.
Why do some people have symptoms similar to that of PF, even if nothing shows on an X-ray?
In the early stages of PF, lung scarring may be too mild to be visible on an X-ray which is not very sensitive for detecting mild fibrosis or subtle interstitial changes even though a patient may have symptoms of PF. Also, PF often starts in the lower lobes or periphery of the lungs and x-rays do not provide detailed images of these areas, hence HRCT is recommended for evaluation of lung tissue.
People with non-fibrotic lung disease such as hypersensitivity pneumonitis, early ILD, sarcoidosis or sjogren’s syndrome related lung disease have symptoms that mimic PF but may not show fibrosis on an x-ray. Some respiratory conditions such as asthma and chronic bronchitis can cause similar symptoms but affect the airways rather than lung tissue, leading to normal x-rays.
Finally, symptoms such as shortness of breath and fatigue can also be caused by heart disease, muscle weakness – which is common in autoimmune disease – or anemia, which are non-lung causes and will not show up on a chest x-ray.
Treatment & Management Options
What are the standard treatments for PF?
Medications: Treatment with medication depends on the cause of PF. If the PF is caused by auto-immune disease, treatment usually involves anti-inflammatory medications. We also have medications to slow down the fibrosis process (anti-fibrotic medications).
Lifestyle modifications: This can include improvements to your nutrition and exercise, as well as resources such as pulmonary rehabilitation and supplemental oxygen. Pulmonary rehab and exercise have been proven to improve quality of life in lung diseases.
Supplemental oxygen can also play an important role as it resolves the low oxygen level from PF. It can prevent the development of complications from PF, namely pulmonary hypertension (which is elevated blood pressure of the pulmonary vessels) which can lead to right heart failure.
When is supplemental oxygen necessary?
We [at Dr. Elnaggar’s location] always send PF patients to get evaluated for home Oxygen. Some patients just need oxygen with exertion, others with more advanced disease, will require oxygen even at rest.
Does supplemental oxygen improve quality of life, or just prolong survival?
Oxygen has been shown to improve survival. It makes PF patients feel better and they can do more exercise as the oxygen level doesn’t drop that much. Oxygen also can prevent the development of pulmonary hypertension, which is a complication from PF. All this would improve the quality of life.
What about alternative & emerging therapies, such as the IV therapy Intravenous Immunoglobulin (also known as IVIG)?
IVIG has been tried as a therapy in acute PF flare requiring hospitalization. IVIG is used during acute exacerbations and in case of hospitalization for infections. It is also used in PF patients with autoimmune conditions. However, it is unclear if IVIG has anti-inflammatory action and is generally not used in PF patients due to lack of evidence. It is not recommended by many doctors, because we don’t have strong evidence to support doing it for patients with IPF.
Hospitalization
What happens during hospitalization? What tests and evaluations are typically done upon arrival?
Usually we do blood work, imaging, which might include CT chest as well as infection work-up to rule out infection/pneumonia as a trigger to the acute flare.
How do you determine if a patient needs high-flow oxygen, non-invasive ventilation (BiPAP), or mechanical ventilation?
That depends on the clinical status of the patient/severity of the flare and the level of oxygen and carbon dioxide in the blood. High flow oxygen is typically used when a patient is able to breathe on their own but are hypoxemic (have low oxygen levels). High flow can be up to 60L/min. It is also used post extubation (removal of a breathing tube which may have been inserted during surgery, injury or infection) . BIPAP and mechanical ventilation are used when patients are both hypoxemic and have carbon dioxide retention. These patients need oxygenation and ventilation support.
How are hospital-acquired infections (such as pneumonia) prevented in PF patients?
In hospitals, there are implemented protocols to prevent hospital acquired pneumonia. Examples of those methods are oral care, elevation of the head of bed to prevent aspiration events, as well as early mobilization.
What are the risks of steroid treatments, antibiotics, IVIG, or other interventions in hospitalized PF patients?
For acute flare of PF, we usually treat with steroids and antibiotics. IVIG is not widely used as a treatment option for PF flare given the lack of evidence (scroll up to the question about IVIG for more details).
Steroids can cause high blood pressure, high sugar levels, and could also increase the risk of infection. We monitor the patient closely for those side effects and treat them if they develop.
How long do most hospital stays last for PF exacerbations, and what determines when a patient can go home?
The average hospital stay is about 10 days. It depends on the severity of the disease; it could be shorter or longer. We would like to see that the patient is getting close to their baseline. Examples of things we look at are how the patient is feeling and the oxygen level. In the hospital, we assess if patients need any support at home (like oxygen therapy or breathing machine) and provide them with what they need.
Thank you once again to Dr. Elnaggar for collaborating with our Director to address these common community questions about PF diagnosis, treatment options, and hospitalization.
Left: Neha Shah, PhD, Director of Pulmonary Fibrosis NOW!
Right: Dr. Mohamed Elnaggar, MD
To read other educational blogs about the community’s frequently asked questions, visit:
- https://pulmonaryfibrosisnow.org/2021/07/31/faqs-about-pf/
- https://pulmonaryfibrosisnow.org/2021/08/25/faqs-about-pf-part-2/
- https://pulmonaryfibrosisnow.org/2024/01/29/faqs-about-pulmonary-function-tests-pfts/
- https://pulmonaryfibrosisnow.org/2024/12/02/faqs-about-pf-and-end-of-life-planning/
Be sure to comment below with any additional questions that might be of interest to you, and register for our support group meetups to have interactive conversations with others who can relate. Join our community forum on Facebook for 24/7 support too.
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