The Value of a Multidisciplinary Approach to the Diagnosis of Usual Interstitial Pneumonitis and Idiopathic Pulmonary Fibrosis: Radiology, Pathology, and Clinical Correlation

2016 ◽  
Vol 206 (3) ◽  
pp. 463-471 ◽  
Author(s):  
Jonathan H. Chung ◽  
David A. Lynch
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Interstitial Pneumonitis ◽  
Multidisciplinary Approach ◽  
Clinical Correlation ◽  
Usual Interstitial Pneumonitis

scholarly journals Practical application and validation of the 2018 ATS/ERS/JRS/ALAT and Fleischner Society guidelines for the diagnosis of idiopathic pulmonary fibrosis

2021 ◽  
Vol 22 (1) ◽  
Author(s):  
Angela R. Shih ◽  
Chayanin Nitiwarangkul ◽  
Brent P. Little ◽  
Benjamin W. Roop ◽  
Sreyankar Nandy ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Interobserver Variability ◽  
High Specificity ◽  
Classification Performance ◽  
Accurate Diagnosis ◽  
Practical Application ◽  
Test Characteristics ◽  
High Positive Predictive Value ◽  
Usual Interstitial Pneumonitis

Abstract Background Accurate diagnosis of idiopathic pulmonary fibrosis (IPF) is essential to inform prognosis and treatment. In 2018, the ATS/ERS/JRS/ALAT and Fleischner Society released new diagnostic guidelines for usual interstitial pneumonitis (UIP)/IPF, adding Probable UIP as a CT category based on prior studies demonstrating this category had relatively high positive predictive value (PPV) for histopathologic UIP/Probable UIP. This study applies the 2018 ATS/ERS/JRS/ALAT and Fleischner Society guidelines to determine test characteristics of CT categories in academic clinical practice. Methods CT and histopathology were evaluated by three thoracic radiologists and two thoracic pathologists. Comparison of consensus categorization by the 2018 ATS and Fleischner Society guidelines by CT and histopathology was performed. Results Of patients with CT UIP, 87% (PPV, 95% CI: 60–98%) had histopathologic UIP with 97% (CI: 90–100%) specificity. Of patients with CT Probable UIP, 38% (PPV, CI: 14–68%) had histopathologic UIP and 46% (PPV, CI: 19–75%) had either histopathologic UIP or Probable UIP, with 88% (CI: 77–95%) specificity. Patients with CT Indeterminate and Alternative Diagnosis had histopathologic UIP in 27% (PPV, CI: 6–61%) and 21% (PPV, CI: 11–33%) of cases with specificities of 90% (CI: 80–96%) and 25% (CI: 16–37%). Interobserver variability (kappa) between radiologists ranged 0.32–0.81. Conclusions CT UIP and Probable UIP have high specificity for histopathologic UIP, and CT UIP has high PPV for histopathologic UIP. PPV of CT Probable UIP was 46% for combined histopathologic UIP/Probable UIP. Our results indicate that additional studies are needed to further assess and refine the guideline criteria to improve classification performance.

Autoimmunity associated with cardiac, respiratory, and renal disease

2013 ◽  
pp. 201-212
Author(s):  
Gavin P Spickett
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Renal Disease ◽  
Rheumatic Fever ◽  
Respiratory Disease ◽  
Cardiac Disease ◽  
Interstitial Pneumonitis ◽  
Fibrosing Alveolitis ◽  
Cryptogenic Fibrosing Alveolitis ◽  
Alveolar Proteinosis

Cardiac disease 1: myocarditis and cardiomyopathy Cardiac disease 2: eosinophilic syndromes Cardiac disease 3: recurrent pericarditis and Dressler’s syndrome Cardiac disease 4: rheumatic fever Respiratory disease 1: idiopathic pulmonary fibrosis (cryptogenic fibrosing alveolitis) Respiratory disease 2: pulmonary alveolar proteinosis (PAP) Respiratory disease 3: lymphoid interstitial pneumonitis and sarcoidosis...

scholarly journals The multidisciplinary approach in the diagnosis of idiopathic pulmonary fibrosis: a patient case-based review

2015 ◽  
Vol 24 (135) ◽  
pp. 69-77 ◽  
Author(s):  
Sara Tomassetti ◽  
Sara Piciucchi ◽  
Paola Tantalocco ◽  
Alessandra Dubini ◽  
Venerino Poletti
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Interstitial Pneumonia ◽  
Multidisciplinary Approach ◽  
Accurate Diagnosis ◽  
Diagnostic Assessment ◽  
Patient Case ◽  
Specialist Nurse ◽  
Unnecessary Testing ◽  
Worse Prognosis

Idiopathic pulmonary fibrosis (IPF) is a specific form of chronic, progressively fibrosing interstitial pneumonia that is associated with a significantly worse prognosis than other forms of chronic interstitial pneumonia. An early and accurate diagnosis of IPF is important to enable the initiation of disease-specific therapies, which have the potential to reduce disease progression, and the avoidance of inappropriate and potentially harmful drugs. Establishing an accurate diagnosis of IPF can be challenging. Recent studies and international guidelines advocate the importance of a multidisciplinary team (MDT) in the initial diagnostic assessment of patients with suspected IPF. Typical MDT members include a pulmonologist, a radiologist and a pathologist, with further input from a thoracic surgeon, a rheumatologist, a specialist nurse and an occupational physician where appropriate. Multidisciplinary diagnosis is considered the gold standard because it can improve the accuracy of diagnosis of IPF, avoid unnecessary testing (e.g. lung biopsy), and optimise patient management. Here we highlight the strengths and limitations of the multidisciplinary approach to IPF diagnosis through MDT discussion of two patient cases.

Comparative analysis of connective tissue disease-associated interstitial lung disease and idiopathic pulmonary fibrosis from a tertiary care centre in Pakistan

2021 ◽  
Vol 71 (10) ◽  
pp. 2330-2334
Author(s):  
Ali Bin Sarwar Zubairi ◽  
Huzaifa Ahmad ◽  
Maryam Hassan ◽  
Faraz Siddiqui ◽  
Nousheen Iqbal ◽  
...  
Keyword(s):  
Comparative Analysis ◽  
Interstitial Lung Disease ◽  
Idiopathic Pulmonary Fibrosis ◽  
Connective Tissue ◽  
Pulmonary Fibrosis ◽  
Lung Disease ◽  
Connective Tissue Disease ◽  
Interstitial Pneumonitis ◽  
Tertiary Care ◽  
Data Registry

Objectives: The burden of interstitial lung disease (ILD) is rising globally. This study aimed to describe and compare characteristics of Connective Tissue Disease-associated ILD with Idiopathic Pulmonary Fibrosis, the two most commonly observed ILDs among outpatients at a tertiary care hospital in Karachi, Pakistan. Methods: A retrospective research study was conducted. Patients with ILD were identified through the outpatient data registry at the Aga Khan University Hospital (AKUH), Karachi from October 2016 to October 2017. We obtained data pertaining to demographics, clinical and radiologic features. A comparative analysis was done to compare the patient characteristics and key features between CTD-ILD and IPF patients. The analysis was done using STATA version 12.0. Results: We identified 184 patients with ILD, which included 52 (29.3%) with CTD-ILD and 62 (35%) with IPF. The most prevalent conditions among CTD-ILD patients included rheumatoid arthritis (42.3%) and scleroderma (25%). Usual interstitial pneumonitis was the common radiologic pattern in RA-ILD (63.6%) and scleroderma (61.5%) while non-specific interstitial pneumonitis was more common in MCTD (85.7%) and SLE (80%). Compared to patients with IPF, those with CTD-ILD were predominantly younger (p<0.001) and female (88.5 % v 45.2%, p<0.001). History of GERD was also significantly lower in CTD-ILD (p=0.05). Conclusion: CTD-ILD patients in our registry were younger and predominantly female compared to IPF. Further studies and ongoing data registry are needed to understand the full spectrum of this disease and long term clinical outcomes. Keywords: Connective tissue disease, Interstitial lung disease, Idiopathic pulmonary fibrosis. Continuous...

scholarly journals Interaction between the promoter MUC5B polymorphism and mucin expression: is there a difference according to ILD subtype?

Thorax ◽  
2020 ◽  
Vol 75 (10) ◽  
pp. 901-903 ◽  
Author(s):  
Carmel J Stock ◽  
Caterina Conti ◽  
Ángeles Montero-Fernandez ◽  
Gaetano Caramori ◽  
Philip L Molyneaux ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Lung Disease ◽  
Interstitial Pneumonitis ◽  
Carrier Status ◽  
Immunohistochemical Expression ◽  
Disease Patterns ◽  
Mucin Expression

The MUC5B promoter variant rs35705950 is associated with idiopathic pulmonary fibrosis (IPF). MUC5B glycoprotein is overexpressed in IPF lungs. We examined immunohistochemical expression of MUC5B in different interstitial lung disease patterns according to rs35705950 T-allele carriage. We observed increased expression of MUC5B in T-allele carriers in both distal airways and honeycomb cysts in patients with IPF (n=23), but no difference in MUC5B expression according to T-carrier status in the distal airways of patients with idiopathic non-specific interstitial pneumonitis (n=17), in scleroderma-associated non-specific interstitial pneumonitis (n=15) or in control lungs (n=20), suggesting that tissue overexpression in MUC5B rs35705950 T-carriers is specific to IPF.

Sign in / Sign up

Export Citation Format

Share Document