Pulmonary fibrosis can be difficult to identify because its symptoms often resemble other lung diseases. When a chronic cough or shortness of breath doesn’t improve, we need to look deeper to find the cause.
We diagnose pulmonary fibrosis by combining detailed medical history, physical exams, imaging, and lung function tests to understand how well the lungs are working and what may be causing the scarring.
At Gwinnett Pulmonary, we rely on proven diagnostic tools to get clear answers. Pulmonary function tests measure how much air the lungs can hold and how efficiently they move oxygen.
High-resolution CT scans give detailed images of lung tissue, helping us see patterns of damage that might confirm pulmonary fibrosis. In some cases, blood tests or a lung biopsy provide more information about the type and cause of the disease.
We begin the diagnosis by identifying early signs that suggest scarring or stiffness in the lungs. Careful attention to breathing changes, medical background, and environmental exposures helps us decide when specialized testing is needed to confirm pulmonary fibrosis or another interstitial lung disease.
Pulmonary fibrosis often develops gradually. Shortness of breath during activity is usually the first symptom people notice.
Over time, even mild exertion can cause breathlessness. A dry, persistent cough is also common and may not improve with usual treatments.
Many patients experience fatigue, unexplained weight loss, or a general decline in stamina. These symptoms reflect how lung damage limits oxygen delivery to the body.
During a physical exam, we may detect crackling sounds in the lower lungs when listening with a stethoscope. Some people develop clubbing—a widening and rounding of the fingertips—caused by chronic low oxygen levels.
| Common Signs | Possible Indication |
|---|---|
| Shortness of breath | Reduced lung capacity |
| Dry cough | Airway irritation or fibrosis |
| Crackles on exam | Scarring in the lower lungs |
| Clubbing of fingers | Long-term low oxygen |
A detailed medical and family history is key to understanding potential causes of lung scarring. We ask about autoimmune diseases, prior radiation therapy, and certain medications that can injure lung tissue.
Environmental and occupational exposures also matter. Long-term contact with dust, gases, or chemicals—such as silica, asbestos, or metal fumes—can lead to interstitial lung disease.
We review smoking history, past infections, and any previous diagnosis of another lung disease. If no clear cause is found, the condition may be labeled idiopathic pulmonary fibrosis (IPF), meaning the cause is unknown.
A pulmonologist specializes in evaluating complex lung diseases like pulmonary fibrosis. We coordinate the diagnostic process, beginning with a physical exam and lung function tests to measure breathing capacity.
We often order a high-resolution CT (HRCT) scan to view the pattern and extent of lung scarring. If results are unclear, blood tests or, in some cases, a lung biopsy may be needed to confirm the diagnosis.
Our role also includes ruling out other conditions that mimic pulmonary fibrosis, such as chronic obstructive pulmonary disease or sarcoidosis. Early referral to a pulmonologist improves accuracy and helps patients begin management sooner.
We use pulmonary function tests (PFTs) to measure how well the lungs move air and transfer gases. These tests help identify patterns of lung restriction or obstruction and track how pulmonary fibrosis or other lung diseases, such as COPD, affect breathing over time.
Spirometry is often the first pulmonary function test we perform. It measures how much air a person can exhale and how quickly.
Using a spirometer, we record two main values:
A reduced FVC with a normal or high FEV1/FVC ratio often suggests a restrictive pattern, which is common in pulmonary fibrosis. In contrast, a low ratio points toward obstructive diseases like COPD.
We repeat spirometry at follow-up visits to check for changes in lung function and response to treatment.
Lung volume testing measures the total amount of air the lungs can hold and what remains after exhalation. The two key values are:
| Measurement | Description | Normal Range |
|---|---|---|
| Total Lung Capacity (TLC) | The maximum volume of air in the lungs after a full inhalation | Varies by age, sex, and height |
| Residual Volume (RV) | The air left in the lungs after full exhalation | About 20–25% of TLC |
In pulmonary fibrosis, both TLC and RV are usually reduced because scarring limits lung expansion.
We often measure these values using body plethysmography, a chamber test that detects pressure and volume changes. These measurements help us confirm the restrictive nature of lung disease and rule out other causes of breathlessness.
The DLCO test evaluates how well gases move from the lungs into the blood. During the test, patients inhale a small, safe amount of carbon monoxide mixed with air and hold their breath for a few seconds.
We then measure how much gas is absorbed. A low DLCO indicates that the lung tissue or blood vessels are impaired, as seen in pulmonary fibrosis.
This test helps us gauge the severity of scarring and monitor disease progression. It also helps differentiate pulmonary fibrosis from other conditions, such as emphysema, where DLCO may also be reduced but for different reasons.
The six-minute walk test (6MWT) measures functional exercise capacity and oxygen levels during activity. Patients walk at their own pace for six minutes while we track distance and oxygen saturation using a pulse oximeter.
A drop in oxygen levels or reduced walking distance signals limited lung function and lower exercise capacity.
We use this test to assess how pulmonary fibrosis affects daily activity and to guide oxygen therapy needs. In some cases, we combine it with an exercise stress test on a treadmill or stationary bike to evaluate heart and lung performance under controlled conditions.
We use imaging to detect lung scarring, measure its extent, and distinguish pulmonary fibrosis from other lung diseases such as pneumonia or chronic inflammation.
Imaging also helps us monitor how the condition changes over time and decide whether other tests, like a biopsy, are needed.
High-resolution computed tomography (HRCT) is the most important imaging tool for diagnosing pulmonary fibrosis. It produces thin, detailed cross-sectional images of the lungs that reveal patterns of scarring and tissue damage.
HRCT helps us identify the distribution and severity of fibrosis. It can show subtle changes that a standard CT scan might miss, such as early interstitial thickening or small cystic spaces.
Radiologists look for specific patterns that suggest idiopathic pulmonary fibrosis (IPF), including reticular markings, ground-glass opacities, and honeycombing. These patterns help classify the disease and guide treatment decisions.
Because HRCT is noninvasive and highly sensitive, it often reduces the need for a surgical lung biopsy. It also allows us to track disease progression and evaluate how well treatments are working.
A standard CT scan provides broader images of the chest but with less detail than HRCT. It can still show areas of lung scarring, thickened tissue, or signs of inflammation.
Physicians often use it when HRCT is unavailable or to assess other chest structures. A chest X-ray is usually one of the first imaging tests performed.
It can reveal general patterns of lung abnormality, such as increased opacity or reduced lung volume. However, it cannot detect early or mild fibrosis.
| Imaging Test | Key Use | Level of Detail | Typical Role |
|---|---|---|---|
| Chest X-Ray | Initial screening | Low | Detects advanced scarring |
| Standard CT | Broader chest view | Moderate | Evaluates the lung and surrounding areas |
| HRCT | Focused lung detail | High | Confirms and monitors fibrosis |
Honeycombing refers to clusters of small, cyst-like air spaces that form when lung tissue becomes permanently scarred. On HRCT, these appear as stacked, rounded spaces near the lung edges.
Their presence strongly supports a diagnosis of pulmonary fibrosis. Traction bronchiectasis occurs when fibrotic tissue pulls the airways open, causing them to appear widened or irregular.
This finding often appears alongside honeycombing and indicates advanced structural damage. Recognizing both features helps us distinguish pulmonary fibrosis from other lung conditions, such as pneumonia or chronic inflammatory diseases.
Accurate identification of these patterns ensures that we select appropriate treatment and avoid unnecessary procedures.
We use lung biopsy techniques to obtain small samples of lung tissue that help confirm or clarify the cause of interstitial lung disease (ILD), including idiopathic pulmonary fibrosis (IPF). Each method differs in how tissue is collected, the amount of tissue obtained, and the level of risk involved.
A surgical lung biopsy (SLB) provides the largest and most detailed tissue samples. We usually perform SLB when imaging and noninvasive tests cannot confirm a diagnosis.
The procedure is done in the operating room under general anesthesia, often using video-assisted thoracoscopic surgery (VATS) rather than open surgery. VATS allows us to take samples from two or more lobes through small incisions.
This approach lowers pain, shortens recovery, and reduces complications compared to open thoracotomy.
Key considerations:
Pathologists examine the tissue for patterns such as usual interstitial pneumonia (UIP), which supports an IPF diagnosis.
A transbronchial lung biopsy (TBLB) uses a flexible bronchoscope inserted through the mouth or nose to collect small tissue samples from the lung. We perform this procedure under sedation rather than general anesthesia.
TBLB is less invasive than surgery but retrieves smaller samples, which may limit accuracy in conditions like IPF. It is more useful for diagnosing sarcoidosis, hypersensitivity pneumonitis, or infection.
Advantages:
Limitations:
A transbronchial lung cryobiopsy (TBLC) uses a cryoprobe to freeze and remove larger tissue pieces than standard TBLB. We often use this method for patients with suspected ILD who are not ideal candidates for surgical biopsy.
The frozen tissue preserves lung architecture, allowing better histopathologic evaluation. Studies show TBLC can approach the diagnostic accuracy of SLB when performed by experienced teams.
Benefits:
Risks:
TBLC results are most reliable when interpreted alongside high-resolution CT (HRCT) findings and clinical data in a multidisciplinary discussion.
We use specific tests and imaging tools to separate pulmonary fibrosis from other causes of lung damage. Accurate identification helps us choose the right treatment and predict how the disease may progress.
Pulmonary fibrosis belongs to a larger group of disorders known as interstitial lung diseases (ILDs). These conditions involve inflammation and scarring of the lung’s supporting tissue, which affects oxygen exchange.
We classify ILDs by their cause or pattern of injury. Some result from autoimmune diseases, environmental exposures (like asbestos or silica), or medications.
Others, such as idiopathic pulmonary fibrosis (IPF), have no known cause.
To tell one subtype from another, we rely on a combination of high-resolution CT (HRCT) scans, pulmonary function tests (PFTs), and sometimes lung biopsies. HRCT images show the distribution and pattern of scarring, while PFTs measure how well the lungs move air and transfer oxygen.
Typical ILD patterns we may identify include:
| ILD Subtype | Common Cause | Imaging Pattern |
|---|---|---|
| IPF | Unknown | Basal, peripheral scarring |
| Asbestosis | Asbestos exposure | Lower-lobe fibrosis |
| Hypersensitivity pneumonitis | Repeated antigen exposure | Patchy ground-glass changes |
Recognizing these patterns helps us confirm whether fibrosis is part of a broader ILD or a distinct process.
Chronic obstructive pulmonary disease (COPD) and pulmonary fibrosis can both cause shortness of breath and chronic cough, but they affect the lungs differently. COPD involves airway obstruction and air trapping, while pulmonary fibrosis causes stiffness and reduced lung expansion.
We use spirometry to measure airflow limitation typical of COPD, where the FEV₁/FVC ratio is reduced. In pulmonary fibrosis, lung volumes are decreased, but airflow ratios often remain normal.
Chest imaging provides further clues. COPD usually shows hyperinflated lungs and flattened diaphragms, while fibrosis appears as reticular markings and honeycombing on HRCT.
Blood oxygen levels also differ. People with fibrosis often desaturate quickly during activity due to poor gas exchange, while those with COPD may have a more gradual oxygen decline.
By combining these findings, we can distinguish scarring disorders like pulmonary fibrosis from obstructive diseases such as COPD.
We use advanced tools and team-based evaluation to confirm pulmonary fibrosis and identify its specific type. These methods improve accuracy, guide treatment decisions, and help us rule out other lung diseases.
Blood tests help us identify possible causes of lung scarring and exclude other conditions. We often measure autoimmune markers, such as antinuclear antibodies (ANA) or rheumatoid factor (RF), to check for connective tissue diseases.
Elevated results may suggest a secondary form of fibrosis rather than idiopathic pulmonary fibrosis (IPF). We also review oxygen levels and inflammatory markers to assess how the disease affects the body.
Although blood tests cannot confirm IPF alone, they provide valuable clues that complement imaging and lung function tests.
Additional assessments, such as exercise tests and pulse oximetry, help us understand how well oxygen moves through the lungs during activity. These results guide treatment plans and monitor disease progression.
| Test Type | Purpose | Example |
|---|---|---|
| Autoimmune panel | Detect immune-related lung disease | ANA, RF |
| Oxygen assessment | Measure oxygen saturation | Pulse oximetry |
| Inflammatory markers | Evaluate systemic inflammation | ESR, CRP |
A multidisciplinary discussion (MDD) brings together experts from different specialties to review all diagnostic data. The team usually includes a pulmonologist, radiologist, and pathologist, each contributing a unique perspective.
We compare high-resolution CT (HRCT) images, lung biopsy findings, and clinical history to reach a consensus. This process helps us distinguish IPF from other interstitial lung diseases that may appear similar on imaging.
When the CT pattern clearly shows usual interstitial pneumonia (UIP), we may confirm IPF without a surgical biopsy. This approach ensures patients receive timely and appropriate care.
The Pulmonary Fibrosis Foundation (PFF) plays an important role in connecting patients and clinicians with evidence-based resources. We often refer patients to the PFF for educational materials, support groups, and updates on clinical trials.
The foundation also promotes standardized diagnostic guidelines that align with recommendations from major respiratory societies. This consistency helps ensure that patients receive comparable evaluations across different care centers.
Through its Care Center Network, the PFF supports collaboration among pulmonologists and research teams. These centers share data and best practices, improving both diagnosis and management for people living with pulmonary fibrosis.
Diagnosing pulmonary fibrosis requires a careful, thorough approach because its symptoms often overlap with other lung conditions. By combining a detailed medical history, physical examination, imaging, and lung function testing, pulmonologists can identify the presence and extent of lung scarring with precision. Tools like high-resolution CT scans, spirometry, DLCO testing, and in some cases, biopsies provide critical insight into how the lungs are functioning and what is driving the disease. Early and accurate diagnosis not only helps clarify the underlying cause but also allows patients to begin treatment sooner, slow progression, and make informed decisions about their long-term lung health. With the right evaluations and ongoing care, patients gain a clearer path toward managing symptoms and protecting their quality of life.
Get the clarity and support your lungs deserve.
At Gwinnett Pulmonary & Sleep, our board-certified pulmonologists use advanced diagnostic tools to accurately identify pulmonary fibrosis and other interstitial lung diseases. From initial evaluation to long-term management, our team is committed to helping you breathe easier with personalized, compassionate care.
Book your appointment today at gwinnettlung.com or call 770-995-0630 to schedule your visit.
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