September is Pulmonary Fibrosis Awareness Month — and for the estimated 200,000 Americans living with this diagnosis, awareness has never mattered more. New treatments approved in the past year are giving patients options that didn't exist before, and early recognition remains the single most important factor in preserving lung function.
Pulmonary fibrosis is a condition in which the lungs become progressively scarred, making it harder and harder to breathe. It belongs to a broader family of diseases called interstitial lung disease (ILD) — a group of more than 200 conditions that cause inflammation or scarring of the lung tissue between the air sacs. Of these, idiopathic pulmonary fibrosis (IPF) is the most common and most serious form.
The Challenge of Early Diagnosis
One of the most difficult aspects of pulmonary fibrosis is how gradually it develops. Most patients experience a slow, progressive worsening of breathlessness over months to years — and because it comes on so gradually, it's easy to attribute to aging, deconditioning, or weight gain.
The two hallmark symptoms are progressive shortness of breath — initially with exertion, later at rest — and a persistent dry, nonproductive cough that doesn't respond to typical cough remedies. Other signs include fatigue, unexplained weight loss, and in some patients, clubbing of the fingertips (a rounding and widening of the fingernails).
When to seek evaluation: If you've had a dry cough or increasing breathlessness for more than 8 weeks — especially if you're over 50, have a history of smoking, environmental exposures (asbestos, silica, coal dust, bird droppings), or autoimmune disease — ask your doctor about a referral to a pulmonologist for further workup.
How Pulmonary Fibrosis Is Diagnosed
Diagnosis starts with a careful history and physical exam. A characteristic finding on auscultation is "velcro crackles" — fine, dry crackles heard at the lung bases that sound like separating Velcro strips. This sound is one of the earliest physical examination clues.
The most important imaging tool is high-resolution CT (HRCT) of the chest. In IPF, the scan typically shows a pattern called usual interstitial pneumonia (UIP) — characterized by honeycombing, traction bronchiectasis, and a peripheral, basal-predominant distribution of fibrosis. When the HRCT pattern is definitive for UIP in the right clinical context, a lung biopsy is usually not needed.
Pulmonary function tests (PFTs) help quantify the degree of impairment. The hallmark pattern is a restrictive defect with reduced lung volumes and a disproportionately low diffusion capacity (DLCO), which reflects the thickened, scarred barrier between the air sacs and blood vessels.
The Multidisciplinary Approach
Current guidelines strongly recommend that ILD diagnosis be made through a multidisciplinary discussion (MDD) involving pulmonologists, radiologists, and pathologists. This team-based approach has been shown to improve diagnostic accuracy and is considered the gold standard. At PulmoCrit, our physicians work closely with specialized radiology and pathology colleagues to ensure every patient receives a precise diagnosis.
Types of Interstitial Lung Disease
While IPF gets the most attention, several other forms of ILD are important to recognize because their causes and treatments differ:
- Idiopathic pulmonary fibrosis (IPF) — the most common idiopathic interstitial pneumonia, typically affecting adults over 50, more common in men and former smokers. Cause is unknown by definition.
- Connective tissue disease–associated ILD (CTD-ILD) — occurs in patients with rheumatoid arthritis, scleroderma, Sjögren syndrome, or other autoimmune conditions. May follow or precede the diagnosis of the underlying disease.
- Hypersensitivity pneumonitis (HP) — caused by inhaling organic antigens such as mold, bird proteins, or certain chemicals. Identifying and removing the exposure is critical.
- Occupational ILD — including asbestosis, silicosis, and coal workers' pneumoconiosis. A detailed occupational history is essential.
- Drug-induced ILD — certain medications, including some chemotherapy agents, amiodarone, and nitrofurantoin, can cause lung fibrosis.
- Sarcoidosis — a granulomatous disease that can affect the lungs and other organs. Unlike IPF, sarcoidosis often improves spontaneously or with steroids.
Distinguishing between these conditions matters because treatment varies dramatically. IPF, for instance, does not respond to steroids or immunosuppressants — treatments that can actually be harmful — while CTD-ILD often does.
Treatment: Three Antifibrotic Options in 2026
There is currently no cure for IPF, but treatment has advanced significantly. The goal of therapy is to slow the rate of lung function decline, reduce symptoms, and preserve quality of life for as long as possible.
Pirfenidone (Esbriet)
FDA-approved in 2014, pirfenidone was one of the first antifibrotic medications shown to slow FVC decline in IPF. It works through anti-inflammatory and antifibrotic mechanisms, though the exact pathway is not fully understood. Common side effects include nausea, rash, and photosensitivity.
Nintedanib (Ofev)
Also approved in 2014, nintedanib is a tyrosine kinase inhibitor that blocks growth factor receptors involved in fibrosis. It has been shown to reduce FVC decline by approximately 50% over 52 weeks. In 2020, its indication was expanded to include other forms of progressive pulmonary fibrosis (PPF) beyond IPF. The most common side effect is diarrhea.
Nerandomilast (Jascayd) — Newly Approved
The biggest news in ILD treatment is the FDA approval of nerandomilast (brand name Jascayd), a first-in-class PDE4B inhibitor developed by Boehringer Ingelheim. Approved for IPF in October 2025 and expanded to progressive pulmonary fibrosis in December 2025, nerandomilast represents the first new mechanism of action for pulmonary fibrosis in over a decade.
About the FIBRONEER-ILD trial: Nerandomilast's approval was based on the FIBRONEER-ILD trial, which enrolled 1,178 adults with progressive pulmonary fibrosis. The study demonstrated a statistically significant reduction in the rate of FVC decline compared to placebo, with a tolerability profile that may offer advantages for patients who cannot tolerate existing antifibrotics.
The availability of three antifibrotic medications gives pulmonologists more flexibility to match treatment to each patient's tolerance and response — an important development given that side effects are the most common reason patients discontinue therapy.
Beyond Medication: Comprehensive ILD Care
Antifibrotic drugs are only one part of a comprehensive management plan. Other critical elements include:
- Supplemental oxygen — prescribed when blood oxygen levels fall below a certain threshold, initially with exertion and later at rest. Oxygen therapy improves exercise tolerance, reduces right heart strain, and can meaningfully improve daily functioning.
- Pulmonary rehabilitation — a structured exercise and education program that has been shown to improve exercise capacity, reduce breathlessness, and enhance quality of life in ILD patients. Benefits are comparable to what's seen in COPD rehabilitation.
- Vaccination — influenza, pneumococcal, COVID-19, and RSV vaccines are strongly recommended, as respiratory infections can trigger acute exacerbations that cause rapid, sometimes irreversible decline.
- Screening for comorbidities — pulmonary fibrosis frequently coexists with obstructive sleep apnea, pulmonary hypertension, gastroesophageal reflux, and depression. Identifying and treating these conditions improves overall outcomes.
- Lung transplant evaluation — for patients with progressive disease despite medical therapy, early referral for transplant evaluation is recommended. IPF is the most common indication for lung transplantation in the United States.
The Sleep Apnea Connection
An often-overlooked comorbidity is obstructive sleep apnea (OSA), which occurs in an estimated 60–90% of IPF patients. The combination is particularly harmful: OSA causes intermittent drops in blood oxygen levels during sleep, which may accelerate the fibrotic process and worsen pulmonary hypertension.
At PulmoCrit, our dual expertise in pulmonary and sleep medicine allows us to screen for and treat both conditions simultaneously — an integrated approach that most pulmonary practices don't offer. Our PC Sleep Center provides in-lab polysomnography and our physicians manage both the ILD and any coexisting sleep disorder.
Living with Pulmonary Fibrosis
A diagnosis of pulmonary fibrosis is life-changing, but it doesn't mean life stops. While median survival statistics (often cited as 2–5 years for IPF from diagnosis) are important for planning, they're averages — and 20–25% of IPF patients survive beyond 10 years. Individual trajectories vary widely, and early treatment with antifibrotics has been shown to shift the curve in the right direction.
Patients who do best tend to share several characteristics: they started treatment early, they stay physically active with pulmonary rehabilitation, they maintain close follow-up with their pulmonologist for medication adjustments and comorbidity management, and they have a strong support system.
Pulmonary Fibrosis Awareness Month: This September, the Pulmonary Fibrosis Foundation and advocacy groups nationwide are working to raise awareness about the importance of early diagnosis and the expanding treatment landscape. If you or a loved one are experiencing unexplained breathlessness or a persistent dry cough, don't wait. Early evaluation by a pulmonologist is the most important step you can take.
When Should You See a Pulmonologist?
Consider requesting an evaluation if you experience any of the following:
- Progressive shortness of breath, especially if it's worsening over weeks to months
- A persistent dry cough lasting more than 8 weeks
- An incidental CT finding of "interstitial changes," "ground-glass opacities," or "fibrosis"
- A family history of pulmonary fibrosis (familial IPF accounts for up to 20% of cases)
- Known exposure to asbestos, silica, mold, or birds
- An autoimmune condition with new respiratory symptoms
PulmoCrit's physicians have extensive experience diagnosing and managing the full spectrum of interstitial lung disease. We offer in-house pulmonary function testing at our PC Physiology & Metabolic Center, coordinate HRCT imaging with specialized thoracic radiologists, and provide ongoing management including antifibrotic prescribing, oxygen assessment, and pulmonary rehabilitation referrals.
Concerned About Pulmonary Fibrosis?
Our board-certified pulmonologists specialize in diagnosing and managing interstitial lung disease, including IPF. We offer comprehensive evaluation with in-house PFTs, HRCT coordination, and access to all three FDA-approved antifibrotic medications. Offices in Granada Hills, Encino, and Thousand Oaks.
Request an Appointment Call (844) 428-5864