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My approach to interstitial lung disease using clinical, radiological and histopathological patterns.

Leslie KO - J. Clin. Pathol. (2009)

Bottom Line: The complex world of interstitial lung disease presents nearly insurmountable challenges to the general surgical pathologist faced with a lung biopsy in this setting.The pathology is often inflammatory and always requires clinical and radiological context for a relevant and clinically useful histopathological diagnosis.A pattern-based histopathological approach to interstitial lung disease provides a "map" for the general pathologist to navigate this area successfully, especially so when used with aid of the clinical and radiological patterns of presentation.

View Article: PubMed Central - PubMed

Affiliation: Division of Anatomic Pathology, Mayo Clinic Arizona, Scottsdale, AZ 85259, USA. leslie.kevin@mayo.edu

ABSTRACT
The complex world of interstitial lung disease presents nearly insurmountable challenges to the general surgical pathologist faced with a lung biopsy in this setting. The pathology is often inflammatory and always requires clinical and radiological context for a relevant and clinically useful histopathological diagnosis. A pattern-based histopathological approach to interstitial lung disease provides a "map" for the general pathologist to navigate this area successfully, especially so when used with aid of the clinical and radiological patterns of presentation.

Show MeSH

Related in: MedlinePlus

Usual interstitial pneumonia (UIP). UIP is characterised by zones of normal lung tissue adjacent to zones of advanced architectural remodelling (temporal heterogeneity). (A) Early in the disease process, an interrupted “rind” of subpleural fibrosis is visible. (B) A more advanced stage shows more extensive perilobular fibrosis with relative centrilobular sparing, producing ring-like scarring at scanning magnification. (A,B) H&E stain, 1× original magnification.
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CPT-62-05-0387-f09: Usual interstitial pneumonia (UIP). UIP is characterised by zones of normal lung tissue adjacent to zones of advanced architectural remodelling (temporal heterogeneity). (A) Early in the disease process, an interrupted “rind” of subpleural fibrosis is visible. (B) A more advanced stage shows more extensive perilobular fibrosis with relative centrilobular sparing, producing ring-like scarring at scanning magnification. (A,B) H&E stain, 1× original magnification.

Mentions: Usual interstitial pneumonia (UIP) is the prototypic chronic interstitial pneumonia with “temporally heterogeneous” interstitial fibrosis and honeycombing (both microscopic and macroscopic), originally described by Liebow and Carrington.16 Patients with cryptogenic fibrosing alveolitis have UIP on surgical lung biopsy.17 UIP is characterised by zones of normal lung tissue adjacent to zones of advanced architectural remodelling (fig 9).18 The latter is recognised by confluent and dense scarring of the alveolar parenchyma. Microscopic honeycombing occurs early in the process and consists of irregular cysts containing mucus, aggregated in dense fibrosis (fig 10). For me, microscopic honeycombing requires fibrosis on at least three sides of the aggregated cysts, a criterion that helps to avoid including foci of peribronchiolar metaplasia under this designation. Small discrete foci of active fibroplasia are always present in UIP, but they are not specific for UIP. These “fibroblastic foci” occur at the interface between dense scar and adjacent normal lung (fig 11). These three key elements of UIP are often related to one another in the biopsy specimen, as a transition from old disease (fibrosis) to normal lung occurs, with active “fibroblast foci” forming a leading edge between them (this is the concept underlying the term “temporal heterogeneity”—heterogeneous in time—with yesterday’s lung destroyed by fibrosis, tomorrow’s lung waiting to be consumed, and fibroblast foci sitting at the interface (today)). A peripheral acinar pattern can often be recognised in UIP, accompanied by relative centriacinar sparing. These findings help to distinguish UIP from other lesions (see below) with interstitial fibrosis and honeycombing (box 4). Rarely, other diseases can simulate the “UIP pattern”, such as chronic hypersensitivity pneumonitis, the rheumatic diseases and asbestosis.


My approach to interstitial lung disease using clinical, radiological and histopathological patterns.

Leslie KO - J. Clin. Pathol. (2009)

Usual interstitial pneumonia (UIP). UIP is characterised by zones of normal lung tissue adjacent to zones of advanced architectural remodelling (temporal heterogeneity). (A) Early in the disease process, an interrupted “rind” of subpleural fibrosis is visible. (B) A more advanced stage shows more extensive perilobular fibrosis with relative centrilobular sparing, producing ring-like scarring at scanning magnification. (A,B) H&E stain, 1× original magnification.
© Copyright Policy - openaccess
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2668105&req=5

CPT-62-05-0387-f09: Usual interstitial pneumonia (UIP). UIP is characterised by zones of normal lung tissue adjacent to zones of advanced architectural remodelling (temporal heterogeneity). (A) Early in the disease process, an interrupted “rind” of subpleural fibrosis is visible. (B) A more advanced stage shows more extensive perilobular fibrosis with relative centrilobular sparing, producing ring-like scarring at scanning magnification. (A,B) H&E stain, 1× original magnification.
Mentions: Usual interstitial pneumonia (UIP) is the prototypic chronic interstitial pneumonia with “temporally heterogeneous” interstitial fibrosis and honeycombing (both microscopic and macroscopic), originally described by Liebow and Carrington.16 Patients with cryptogenic fibrosing alveolitis have UIP on surgical lung biopsy.17 UIP is characterised by zones of normal lung tissue adjacent to zones of advanced architectural remodelling (fig 9).18 The latter is recognised by confluent and dense scarring of the alveolar parenchyma. Microscopic honeycombing occurs early in the process and consists of irregular cysts containing mucus, aggregated in dense fibrosis (fig 10). For me, microscopic honeycombing requires fibrosis on at least three sides of the aggregated cysts, a criterion that helps to avoid including foci of peribronchiolar metaplasia under this designation. Small discrete foci of active fibroplasia are always present in UIP, but they are not specific for UIP. These “fibroblastic foci” occur at the interface between dense scar and adjacent normal lung (fig 11). These three key elements of UIP are often related to one another in the biopsy specimen, as a transition from old disease (fibrosis) to normal lung occurs, with active “fibroblast foci” forming a leading edge between them (this is the concept underlying the term “temporal heterogeneity”—heterogeneous in time—with yesterday’s lung destroyed by fibrosis, tomorrow’s lung waiting to be consumed, and fibroblast foci sitting at the interface (today)). A peripheral acinar pattern can often be recognised in UIP, accompanied by relative centriacinar sparing. These findings help to distinguish UIP from other lesions (see below) with interstitial fibrosis and honeycombing (box 4). Rarely, other diseases can simulate the “UIP pattern”, such as chronic hypersensitivity pneumonitis, the rheumatic diseases and asbestosis.

Bottom Line: The complex world of interstitial lung disease presents nearly insurmountable challenges to the general surgical pathologist faced with a lung biopsy in this setting.The pathology is often inflammatory and always requires clinical and radiological context for a relevant and clinically useful histopathological diagnosis.A pattern-based histopathological approach to interstitial lung disease provides a "map" for the general pathologist to navigate this area successfully, especially so when used with aid of the clinical and radiological patterns of presentation.

View Article: PubMed Central - PubMed

Affiliation: Division of Anatomic Pathology, Mayo Clinic Arizona, Scottsdale, AZ 85259, USA. leslie.kevin@mayo.edu

ABSTRACT
The complex world of interstitial lung disease presents nearly insurmountable challenges to the general surgical pathologist faced with a lung biopsy in this setting. The pathology is often inflammatory and always requires clinical and radiological context for a relevant and clinically useful histopathological diagnosis. A pattern-based histopathological approach to interstitial lung disease provides a "map" for the general pathologist to navigate this area successfully, especially so when used with aid of the clinical and radiological patterns of presentation.

Show MeSH
Related in: MedlinePlus