Interstitial Lung Disease

In a patient with new or worsening dyspnea, cough, hypoxemia, or diffuse parenchymal abnormalities, first determine whether the clinical tempo is acute, subacute, or chronic and compare with prior chest imaging, HRCT, spirometry, lung volumes, and DLCO. A restrictive physiology or reduced DLCO supports physiologic impairment but does not establish a specific ILD diagnosis; HRCT is the pivotal imaging study for defining the diffuse parenchymal pattern. ScienceDirect+1ScienceDirectBasic HRCT patterns in diffuse interstitial lung diseaseScienceDirectCorrelation of high resolution CT patterns with pulmonary ...

Treat acute deterioration in a patient with established fibrotic ILD as a separate diagnostic event rather than automatically as fibrosis progression. Acute exacerbations in rheumatoid arthritis-associated ILD have been associated with very high mortality, and acute worsening plus FVC decline are mortality-associated events in IPF. Obtain an updated HRCT and reassess competing causes of deterioration before attributing decline to the underlying ILD alone. Wiley+1WileyInterstitial Lung Disease in Antineutrophil Cytoplasmic ...WileyClinical Course of Interstitial Lung Disease in Patients With ...

Document resting and exertional oxygen requirement, symptom trajectory, smoking history, medication chronology, prior thoracic irradiation or inhalational exposures, occupational exposures, home antigen exposures, and systemic autoimmune features. This history determines whether the leading branch is exposure-associated ILD, CTD-ILD, medication-related disease, or an idiopathic interstitial pneumonia; chronic hypersensitivity pneumonitis is a key alternative diagnosis in a fibrotic presentation. Nature+1NatureThe histologic diagnosis of usual interstitial pneumonia of idiopathic pulmonary fibrosis. Where we are and where we need to go | Modern PathologyWileyDiagnosis and management of hypersensitivity ...

Classify suspected IPF imaging using the four HRCT categories refined in the 2018 ATS/ERS/JRS/ALAT diagnostic framework: UIP, probable UIP, indeterminate for UIP, and alternative diagnosis. The category determines the confidence of IPF diagnosis and whether additional pathology is potentially informative. NatureNatureDiagnostic and prognostic implications of 2018 guideline for the diagnosis of idiopathic pulmonary fibrosis in clinical practice | Scientific Reports

A UIP-pattern distribution is typically basal-predominant, with reticulation and honeycombing increasing from apex to base; traction bronchiectasis and honeycombing also carry prognostic information in ILD. Conversely, air trapping and subpleural sparing are HRCT signs that can redirect the differential away from straightforward IPF toward alternative ILD patterns. ScienceDirect+1ScienceDirectRadiographic Signs and Patterns in Interstitial Lung Diseasepubs rsnaCT Honeycombing and Traction Bronchiectasis Extent ...

When the clinical context contains no alternative cause and HRCT demonstrates UIP, IPF can usually be confirmed through multidisciplinary review without surgical lung biopsy. Histopathology should be considered when clinical and radiologic data leave meaningful diagnostic uncertainty and when a tissue result would alter management. BMJ+1BMJIdiopathic pulmonary fibrosis - Symptoms, diagnosis and treatment | BMJ Best Practice USNatureThe histologic diagnosis of usual interstitial pneumonia of idiopathic pulmonary fibrosis. Where we are and where we need to go | Modern Pathology

Diagnose IPF only after excluding an alternative disease process. IPF is a chronic progressive fibrosing interstitial pneumonia of unknown etiology defined by radiologic and/or histopathologic UIP; in the appropriate clinical setting, multidisciplinary review of HRCT can establish the diagnosis without biopsy. BMJ+1BMJIdiopathic pulmonary fibrosis - Symptoms, diagnosis and treatment | BMJ Best Practice USNatureThe histologic diagnosis of usual interstitial pneumonia of idiopathic pulmonary fibrosis. Where we are and where we need to go | Modern Pathology

For suspected hypersensitivity pneumonitis, make a structured antigen and exposure assessment central to the workup, then integrate the exposure history with HRCT and, when needed, pathology. Chronic hypersensitivity pneumonitis is an important pathologic and clinical mimic of IPF/UIP, so an apparent UIP pattern does not end the exposure evaluation. Nature+1NatureThe histologic diagnosis of usual interstitial pneumonia of idiopathic pulmonary fibrosis. Where we are and where we need to go | Modern PathologyWileyDiagnosis and management of hypersensitivity ...

For possible CTD-ILD, use systemic history, examination, directed autoimmune evaluation, HRCT pattern, and pulmonary physiology together rather than relying on a single serologic result. In Sjögren syndrome-associated ILD, UIP pattern was more frequent among patients meeting progressive pulmonary fibrosis criteria than among nonprogressors in one cohort, illustrating that a UIP pattern can occur in autoimmune ILD and may identify higher-risk disease behavior. BMJBMJPOS1453 PROGRESSIVE PULMONARY FIBROSIS ACCORDING TO THE NEW ATS DEFINITION IS NOT UNCOMMON IN THE CONTEXT OF SJÖGREN’S SYNDROME-ASSOCIATED INTERSTITIAL LUNG DISEASE | Annals of the Rheumatic Diseases

At baseline, document symptoms, FVC, DLCO, and HRCT extent/pattern so that subsequent change can be judged against an interpretable reference. Across fibrotic ILDs other than IPF, progression occurs in an estimated 18% to 32% of patients; IPF is intrinsically progressive over time. NatureNatureSerum KL-6 as a biomarker to predict progression at one year in interstitial lung disease | Scientific Reports

Use serial symptoms, pulmonary function, and imaging together. The ATS progressive pulmonary fibrosis framework applied in Sjögren-associated ILD defines progression within 1 year by at least two of three domains: worsening symptoms, physiologic decline, and radiologic progression. In that cohort, physiologic progression was FVC decline of at least 5% and/or DLCO decline of at least 10%. BMJBMJPOS1453 PROGRESSIVE PULMONARY FIBROSIS ACCORDING TO THE NEW ATS DEFINITION IS NOT UNCOMMON IN THE CONTEXT OF SJÖGREN’S SYNDROME-ASSOCIATED INTERSTITIAL LUNG DISEASE | Annals of the Rheumatic Diseases

Interpret FVC decline in clinical context. A decline of at least 10% in FVC and at least 15% in DLCO has also been used as a PFT progression definition in ILD research, illustrating that thresholds vary across studies and should not replace integrated review of symptoms and HRCT. BMJ+2BMJAB0829 PROGRESSION OF ILD (INTERSTITIAL LUNG ...BMJPOS1453 PROGRESSIVE PULMONARY FIBROSIS ACCORDING TO THE NEW ATS DEFINITION IS NOT UNCOMMON IN THE CONTEXT OF SJÖGREN’S SYNDROME-ASSOCIATED INTERSTITIAL LUNG DISEASE | Annals of the Rheumatic DiseasesNatureSerum KL-6 as a biomarker to predict progression at one year in interstitial lung disease | Scientific Reports

Escalate reassessment when symptoms worsen, FVC or DLCO falls, oxygen needs rise, or HRCT shows expanding fibrosis. In addition to confirming progression, search for superimposed triggers or alternate causes of deterioration before changing long-term ILD therapy. BMJ+3BMJPOS1453 PROGRESSIVE PULMONARY FIBROSIS ACCORDING TO THE NEW ATS DEFINITION IS NOT UNCOMMON IN THE CONTEXT OF SJÖGREN’S SYNDROME-ASSOCIATED INTERSTITIAL LUNG DISEASE | Annals of the Rheumatic DiseasesWileyInterstitial Lung Disease in Antineutrophil Cytoplasmic ...WileyClinical Course of Interstitial Lung Disease in Patients With ...pubs rsnaCT Honeycombing and Traction Bronchiectasis Extent ...

For confirmed IPF, initial disease-modifying therapy is an antifibrotic agent: pirfenidone or nintedanib. Choice should be individualized through discussion of adverse-effect tolerance, comorbidities, drug interactions, treatment burden, and patient goals; the diagnostic label should be secure because IPF-directed therapy follows exclusion of alternative fibrotic ILD causes. BMJBMJIdiopathic pulmonary fibrosis - Symptoms, diagnosis and treatment | BMJ Best Practice US

Nerandomilast, an oral phosphodiesterase-4 inhibitor, is an FDA-approved IPF treatment and reduced FVC decline versus placebo over 1 year in a randomized phase 3 trial. Incorporate it through an IPF-specific medication review, with selection and monitoring based on current prescribing information and patient-specific factors. BMJBMJIdiopathic pulmonary fibrosis - Symptoms, diagnosis and treatment | BMJ Best Practice US

Prescribe smoking cessation support, pulmonary rehabilitation, and supplemental oxygen when indicated as parallel management rather than as substitutes for antifibrotic treatment. Discuss lung-transplant referral and palliative-care involvement early, including for patients receiving active disease-modifying therapy. BMJBMJIdiopathic pulmonary fibrosis - Symptoms, diagnosis and treatment | BMJ Best Practice US