Symptoms Of Nsip Vs Ipf
Symptoms are not able to distinguish IPF from NSIP. In some cases, cough may be more prominent in IPF but this is not reliable. There is a broad overlap in ages for the two diseases. In general, both affect patients over 50 years of age. The physical exam may reveal prominent crackles in the lungs. IPF patients are more likely to have clubbing of the fingers.
Diagnosis Of Idiopathic Pulmonary Fibrosis
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High-resolution CT
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Sometimes surgical lung biopsy
Diagnosis requires HRCT and in some cases surgical lung biopsy.
Chest x-ray typically shows diffuse reticular opacities in the lower and peripheral lung zones. Small cystic lesions and dilated airways due to traction bronchiectasis are additional findings.
HRCT shows diffuse, patchy, subpleural, reticular opacities with irregularly thickened interlobular septa and intralobular lines subpleural honeycombing and traction bronchiectasis. This is referred to as the usual interstitial pneumonia pattern. Ground-glass opacities affecting > 30% of the lung suggest an alternative diagnosis.
Laboratory testing plays little role in diagnosis.
Citation Doi And Article Data
Citation:DOI:Dr Yuranga WeerakkodyRevisions:see full revision historySystem:
- UIP
Usual interstitial pneumonia is a histopathologic and radiologic pattern of interstitial lung disease, which is the hallmark pattern for idiopathic pulmonary fibrosis .
On imaging, usual interstitial pneumonia usually presents with a lung volume loss and a craniocaudal gradient of peripheral septal thickening, bronchiectasis, and honeycombing.
This article will focus solely on the usual interstitial pneumonia pattern as a radiological or histopathological descriptor, for further discussion in the clinical aspects, please refer to the parental article on the specific underlying clinical diagnosis .
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Clinical Features And Imaging
Usual interstitial pneumonia is more common in men than in women. Patients with UIP usually present in the sixth or seventh decade of life with slowly progressive dyspnea and nonproductive cough refractory to antitussive agents. Patients with IPF who are younger than 50 years of age are rare such patients may subsequently manifest features of an underlying connective tissue disease that was subclinical at the time of the IPF diagnosis or may be familial IPF. Most UIP patients are current or past smokers. Constitutional symptoms are unusual, and digital clubbing develops in 25%-50% of patients. Although not specific, velcro-type fine end-inspiratory crackles on chest auscultation are characteristic physical finding in nearly all patients. Features of right heart failure and peripheral edema may develop usually in later stage of disease.
Most patients display restrictive pulmonary function abnormalities, including reduced lung volumes with relative preservation of airflow, a reduction in the diffusion capacity for carbon monoxide with hypoxemia at rest and/or with exercise.
Some patients present with a more acute onset of respiratory symptoms that may mimic the clinical presentation of acute interstitial pneumonia . This syndrome has been termed acute exacerbation of idiopathic pulmonary fibrosis or accelerated UIP and occurs in as many as 14% of untreated patients observed for 2 years.
Etiology Of Idiopathic Pulmonary Fibrosis
A combination of environmental, genetic, and other unknown factors probably contribute to alveolar epithelial cell dysfunction or reprogramming, which leads to abnormal fibroproliferation in the lung. There is ongoing research into the contributions of genetics, environmental stimuli, inflammatory cells, the alveolar epithelium, mesenchyme, and matrix.
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Survival And Pulmonary Function Comparison Between Biopsy
MA-UIP with biopsy-proven UIP had a significantly better event-free survival compared with the matched IPF-UIP cohort after adjusting for age, gender, ethnicity and baseline FVC . However, cumulative survival was not significantly different, likely due to the high frequency of lung transplantation in the IPF-UIP patients. Similarly, pulmonary function decline was significantly better in biopsy-matched MA-UIP , after controlling for age, gender, ethnicity and baseline FVC. Smoking status had no influence on these results.
Prognosis For Idiopathic Pulmonary Fibrosis
Most patients have moderate to advanced clinical disease at the time of diagnosis and deteriorate despite treatment. Median survival is about 3 years from time of diagnosis. Several prognostic models have been proposed. Among the factors that portend a worse prognosis are older age, male sex, lower forced vital capacity, and lower diffusing capacity for carbon monoxide .
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3. Richeldi L, du Bois RM, Raghu G, et al: Efficacy and safety of nintedanib in idiopathic pulmonary fibrosis. N Engl J Med 370:2071â2082, 2014.
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Pulmonary Hypertension As A Complicating Factor
Pulmonary hypertension has been postulated to be a factor that complicates and affects the prognosis of IPF. Therapeutic agents for PH might be effective for those who have PH with IPF. Early diagnosis of PH in patients with IPF is difficult lack of specific clinical symptoms often leads to delayed diagnosis of PH in patients with IPF. The reported prevalence of PH in patients with IPF ranges from 20%-84% when evaluated by pulmonary arterial enlargement on chest radiography, right ventricular systolic pressure using transthoracic echocardiography, or mean pulmonary artery pressure using right-heart catheterization.
Biomarkers such as B-type natriuretic peptide and N-terminal prohormone BNP are potentially helpful tools in identifying PH. One study has shown that iron deposition and increased alveolar septal capillary density in nonfibrotic areas of the UIP lung tissue are associated with right ventricular systolic pressure, seemingly independent of the degree of fibrosis, suggesting that these features are possible morphologic predictors of PH in UIP.
Histopathology In The Uip Group
At low magnification, the lesions varied in severity and were distributed erratically. Chronic and acute lesions of interstitial inflammation, fibrosis, and honeycombing were interspersed among the normal lung tissue, and were primarily within the subpleural lung parenchyma. The interstitial inflammation was typically patchy with alveolar septum infiltrates comprising leukomonocytes and plasmacytes accompanied by type II alveolar cell proliferation. Diffuse hyperplastic fibrous tissue with collagen deposition formed the alveolar structure. In the areas of inflammation, fibrosis, and honeycomb changes, foci in a light-blue myxoid stroma background were observed. These foci comprised proliferative fibroblast and myoblast cells were identified as myofibroblast cell foci. The honeycombed lung formed by a cystic fiber chamber was often covered by bronchial epithelial cells and contained mucus. In the fibrotic and honeycomb areas, smooth muscle proliferation was observed, which was patchy and at times with myogelosis. In two cases, mixed diffuse alveolar damage, cell proliferation, and the loss of the alveolar epithelial cells were also present. The lung interstitium also showed fibroblast proliferation and a macrophagocyte mass along with serous effusion from the alveolar space. The incidences of each particular lesion in both patient groups are summarized in .
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General Characteristics And Demographics
A total of 263 patients from both institutional sites met inclusion criteria, including 169 cases at Mayo Clinic in Scottsdale, Arizona, and 94 cases at Mayo Clinic in Rochester, Minnesota. General characteristics and patient demographics for each time cohort are summarized in Table 1, and characteristics of the subset of patients 50 years and older are summarized in Table 2. Fewer biopsies were obtained after 2011, with the total number of patients declining after 2011 , and the number of patients at least 50 years old fell after 2011 . A significant difference in patient age was observed between the pre-2011 and post-2011 cohorts, with the mean age at the time of diagnosis being 3.3 years younger after 2011 . Interestingly, a significant increase was observed in the number of available slides per patient after 2011 , and a similar increase in the number of available slides was also observed in the subset of patients 50 years and older . No significant differences were observed in the proportion of cases obtained across participating sites, patient gender, or number of lobes sampled before versus after 2011.
Etiology Prevalence And Epidemiology
Idiopathic pulmonary fibrosis has been defined as a specific form of chronic fibrosing interstitial pneumonia limited to the lung and associated with the histologic and/or CT appearance of usual interstitial pneumonia . IPF is the most common idiopathic interstitial lung disease. Data from the late 1980s estimated a prevalence of 20 per 100,000 for men and 13 per 100,000 for women, and an incidence of 11 per 100,000 per year in men and 7 per 100,000 per year in women. However, more recent data has shown that prevalence and incidence of IPF have increased, with a prevalence of 42.7 to 63 per 100,000 and an incidence of 16.3 to 17.4 per 100,000 per year, which may reflect the average increased life expectancy in that span of time. Most patients with IPF are older than 50 years, and approximately 80% are older than 65 years. Indeed, age older than 75 years has been shown to have an extremely high positive predictive value in IPF diagnosis. IPF has been reported worldwide in rural and urban settings. IPF is likely more common in Caucasians, although this has not been definitively proven. IPF is more common in smokers the odds ratio of IPF in smokers compared with lifelong nonsmokers in various studies ranges from 1.6 to 2.9. Occasionally, clusters of IPF may occur in families. Although the prevalence of familial IPF is unknown, it has been estimated to account for 0.5% to 2.2% of patients with IPF.
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Prognosis And Predictive Factors
The prognosis of interstitial pneumonia is grim, with short-term mortality rates in excess of 50% in most reported series. In most patients, UIP follows a progressive course, with median survivals from the time of diagnosis of about 3 years.
No single histologic finding consistently predicts prognosis in individual patients with UIP. Patients with more extensive fibroblast foci have experienced shorter mean survival in some studies while other investigators have failed to demonstrate the same relationship to survival.
The natural history of idiopathic pulmonary fibrosis has been known as a steady decline in lung function over time without a response to medical therapy. However, the clinical course of IPF is not always predictable despite its generally poor prognosis.
Some common genetic variants have also been associated with distinct clinical phenotypes. Clinical studies should be designed controlling for the genetic backgrounds of subjects, since clinical outcomes and therapeutic responses may differ by genotypes. Further understanding of these differences will allow the development of personalized approaches to IPF management.
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Can You Distinguish Idiopathic Pulmonary Fibrosis From Interstitial Lung Disease
You can distinguish the difference between Idiopathic Pulmonary Fibrosis and other interstitial lung diseases by examining lung tissue under a microscope. The tissue of IPF patients has a very specific pattern. This pattern is called UIP . This term is a bit confusing because there is no infection despite having the word pneumonia in the phrase. In some geographic areas UIP is used interchangeably with IPF. Fibrotic foci can be seen during microscopic evaluation of IPF lung tissue. These are small areas of active collagen deposition and are felt to be the engines of progressive fibrosis . The lesions are unevenly distributed in the lung tissue, more prominently at the bases and in the periphery areas. There is abnormal lung tissue adjacent to normal lung tissue. Honeycomb change is also typically seen and is felt to be an advanced stage of fibrosis.
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Why Is It Important To Distinguish Ipf From Nsip
The treatments are entirely different. For IPF we have compelling data that immunosuppression . In fact a large study showed that patients treated with prednisone and azathioprine did worse than those not treated at all. On the other, the treatment for NSIP is usually prednisone and other immunosuppressants.
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Interstitial Lung Disease Vs Idiopathic Pulmonary Fibrosis
It is critically important to distinguish IPF from other types of lung disease. There is a family of lung diseases that are termed interstitial lung diseases or ILD. This collection of diseases has different causes and treatments. Some are related to connective tissue diseases such as Lupus, while others may be related to viral infections.
Some doctors are lumpers and others are splitters. Being a splitter means that for some doctors it is important to categorize and then further sub categorize each diagnosis. Other doctors, the lumpers, are more comfortable with large groupings- such as diseases that are treated with one type of therapy or another. I am a lumper when it comes to ILD. The most important question is whether the lung process will respond to medicines that suppress the immune system . In the world of ILD, most respond to prednisone whereas IPF dose not. Thus it is important to separate Idiopathic Pulmonary Fibrosis from all of the other interstitial lung diseases.
Patient Demographics And Clinical Findings
The mean patient age in the UIP group was 60 years and ranged from 50-75 years the group comprised 12 men and 6 women. Thirteen patients had a history of smoking, and 6 patients had previous contact with inorganic or organic dust. Previous exposure ranged from 5-62 months and averaged 21.6 months. In the NSIP group, the mean patient age was 48 years and ranged from 28-70 years the group included 7 men and 14 women. Eight patients had a history of smoking, and four patients had previous contact with inorganic or organic dust. Previous exposure ranged from 1-24 months and averaged 7.8 months.
The clinical findings in the UIP and NSIP patients are summarized in . Bacterial culture of sputum yielded negative results in all patients. Anti-nuclear antibody and molecular reactions yielded negative results in all patients except one patient in the NSIP group, who had increased O antibody levels. In the UIP patients, the chest radiographs showed asymmetric bilateral reticular shadows in the basal and peripheral lungs, and decreased lung volume. The CT and HRCT showed flake- and net-like shadows, primarily in the basal lungs. In a few patients, ground-glass shadows and severe fibrosis were present and were accompanied by traction bronchiectasis, bronchiolectasis, or subpleural honeycomb-like lesions.
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Histopathology In The Nsip Group
All cases showed varying severities of chronic inflammation and interstitial fibrosis. The pathological changes were similar across the tissue samples. On histologic examination, the subtypes were distributed as follows: one case of cellular type, nine cases of mixed type, and eight cases of fibrous type lesions. Inflammatory cell infiltration and fibrous tissue proliferation were observed in the mixed type cases, which primarily comprised lymphocytes and a small number of plasmacytes. In the fibrous type cases, the inflammation was comparatively less, but the collagen deposition was significantly greater. The comparison between the pathologic characteristics in the NSIP and UIP patients is shown in .
Usual Interstitial Pneumonia In Contemporary Surgical Pathology Practice: Impact Of International Consensus Guidelines For Idiopathic Pulmonary Fibrosis On Pathologists
Jordan M. Eldersveld, Eunhee S. Yi, Katie L. Kunze, Maxwell L. Smith, Henry D. Tazelaar, Brandon T. Larsen Usual Interstitial Pneumonia in Contemporary Surgical Pathology Practice: Impact of International Consensus Guidelines for Idiopathic Pulmonary Fibrosis on Pathologists. Arch Pathol Lab Med 1 June 2021 145 : 717727. doi:
Idiopathic pulmonary fibrosis is a clinical syndrome characterized by the presence of usual interstitial pneumonia radiologically and pathologically. Per consensus criteria adopted in 2011, diagnosis of idiopathic pulmonary fibrosis no longer requires a biopsy in an appropriate context if UIP is seen on imaging. As a result, lung biopsies are now typically reserved for patients having indeterminate clinical or imaging findings or suspicion for alternative diagnoses, but the impact of updated guidelines on pathology practice remains unclear.
To determine the frequency of histologic UIP before and after 2011.
Surgical lung biopsies from adults were studied within two 4-year periods: July 1, 2006 through June 30, 2010 and January 1, 2012 through December 31, 2015. Pathology slides were reviewed in a fashion blinded to clinical information and were classified using current guidelines.
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Criteria For Uip Pattern
Usual interstitial pneumonia is the most common idiopathic interstitial pneumonia31 and carries the worst prognosis.30 UIP is the radiologic and pathologic pattern observed in patients with IPF,45 but UIP-like patterns are also caused by secondary conditions such as collagen vascular disease, chronic hypersensitivity pneumonitis, asbestosis, or drug toxicity,3233 highlighting the importance of identifying any possible causes of lung disease prior to diagnosing idiopathic UIP.45
Differentiating Uip And Nsip
Several key features can help to distinguish NSIP from UIP. While helpful, the differences between UIP and NSIP are often not as obvious as this figure suggests, and the difficulty lies in distinguishing the cases that lie toward the middle. NSIP is temporally and spatially homogeneous, while UIP is typically heterogeneous, patchy, and irregular in size.36 The extent of honeycombing and traction bronchiectasis is greater in UIP than the extent of ground glass opacity or micronodules, which are more commonly associated with an NSIP pattern.536
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