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Pathological features of connective tissue disease-associated interstitial lung disease in transbronchial
Andrew Churg1, Venerino Poletti2, Claudia Ravaglia2
1Department of Pathology, University of British Columbia, and Vancouver General Hospital, Vancouver, BC, Canada.
Insights
Transbronchial cryobiopsies can help diagnose interstitial lung diseases (ILD). Specific patterns like non-specific interstitial pneumonia (NSIP) favor connective tissue disease-associated ILD (CTD-ILD), but usual interstitial pneumonia (UIP) patterns are hard to distinguish.
Area of Science:
- Pulmonology
- Pathology
- Radiology
Background:
- Transbronchial cryobiopsies are increasingly utilized for diagnosing interstitial lung diseases (ILD).
- Limited data exists on distinguishing specific ILD patterns within cryobiopsies.
- Pathological guidelines are needed to differentiate common ILDs in cryobiopsy specimens.
Purpose of the Study:
- To establish pathological guidelines for differentiating usual interstitial pneumonia (UIP) in idiopathic pulmonary fibrosis (IPF), fibrotic hypersensitivity pneumonitis (FHP), and connective tissue disease-associated ILD (CTD-ILD) using transbronchial cryobiopsies.
- To analyze the frequency of specific histopathological patterns in cryobiopsies from patients with established CTD-ILD, IPF, and FHP.
Main Methods:
- Analysis of 120 transbronchial cryobiopsies from patients with multidisciplinary discussion (MDD)-confirmed CTD-ILD.
- Comparison with a previous cohort of 121 cryobiopsies from patients with MDD-confirmed IPF or FHP.
- Evaluation of histopathological patterns including non-specific interstitial pneumonia (NSIP), NSIP with organizing pneumonia (OP), and UIP patterns.
Main Results:
- Non-specific interstitial pneumonia (NSIP) alone (30%) and NSIP with organizing pneumonia (OP) (24%) were significantly more frequent in CTD-ILD cases, favoring this diagnosis.
- A usual interstitial pneumonia (UIP) pattern was identified in a majority of FHP (54%) and IPF (71%) cases, supporting these diagnoses.
- While interstitial giant cells suggested FHP or CTD-ILD over IPF, they were infrequent. Lymphoid aggregates and fibroblast foci counts did not differentiate UIP patterns among the studied ILDs.
Conclusions:
- Pathological findings of NSIP, particularly NSIP with OP, in cryobiopsies strongly suggest CTD-ILD in the appropriate clinical context.
- Distinguishing CTD-ILD with a UIP pattern, FHP with UIP, and IPF based solely on cryobiopsy findings remains challenging.
- Cryobiopsy interpretation requires integration with clinical and radiological data for accurate ILD diagnosis.
Aim:
Transbronchial cryobiopsies are increasingly used for the diagnosis of interstitial lung disease (ILD), but there is a lack of published information on the features of specific ILD in cryobiopsies. Here we attempt to provide pathological guidelines for separating usual interstitial pneumonia (UIP) of idiopathic pulmonary fibrosis (IPF), fibrotic hypersensitivity pneumonitis (FHP) and connective tissue disease-associated ILD (CTD-ILD) in cryobiopsies.
Methods:
We examined 120 cryobiopsies from patients with multidisciplinary discussion (MDD)-established CTD-ILD and compared them to a prior series of 121 biopsies from patients with MDD-established IPF or FHP.
Results:
A non-specific interstitial pneumonia (NSIP) pattern alone was seen in 36 of 120 (30%) CTD-ILD, three of 83 (3.6%) FHP and two of 38 (5.2%) IPF cases, statistically favouring a diagnosis of CTD-ILD. The combination of NSIP + OP was present in 29 of 120 (24%) CTD-ILD, two of 83 (2.4%) FHP and none of 38 (0%) IPF cases, favouring a diagnosis of CTD-ILD. A UIP pattern, defined as fibroblast foci plus any of patchy old fibrosis/fibrosis with architectural distortion/honeycombing, was identified in 28 of 120 (23%) CTD-ILD, 45 of 83 (54%) FHP and 27 of 38 (71%) IPF cases and supported a diagnosis of FHP or IPF. The number of lymphoid aggregates/mm2 and fibroblast foci/mm2 was not different in IPF, CTD-ILD or FHP cases with a UIP pattern. Interstitial giant cells supported a diagnosis of FHP or CTD-ILD over IPF, but were infrequent.
Conclusions:
In the correct clinical/radiological context the pathological findings of NSIP, and particularly NSIP plus OP, favour a diagnosis of CTD-ILD in a cryobiopsy, but CTD-ILD with a UIP pattern, FHP with a UIP pattern and IPF generally cannot be distinguished.
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