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Histopathological aspects of usual interstitial pneumonia in patients with systemic connective tissue diseases

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dc.contributor.authorMakovická, Mária
dc.contributor.authorDurcová, Barbora
dc.contributor.authorVrbenská, Adela
dc.contributor.authorMakovicky, Peter
dc.contributor.authorMichalčová, Patricie
dc.contributor.authorKráľová, Klaudia
dc.contributor.authorMuri, Jozef
dc.date.accessioned2025-01-08T11:59:42Z
dc.date.available2025-01-08T11:59:42Z
dc.date.issued2025
dc.description.abstractFive cases of patients with systemic connective tissue diseases (CTD) who developed connective tissue disease-associated interstitial lung disease (CTD-ILD) with progressive pulmonary fibrosis (PPF) are reported here. Unspecified ILD was diagnosed using high-resolution computed tomography (HRCT). Histologically, all cases were usual interstitial pneumonia (UIP) with findings of advanced (3/5) to diffuse (2/5) fibrosis, with a partially (4/5) to completely (1/5) formed image of a honeycomb lung. The fibrosis itself spread subpleurally and periseptally to more central parts (2/5) of the lung, around the alveolar ducts (2/5), or even without predisposition (1/5). Simultaneously, there was architectural reconstruction based on the mutual fusion of fibrosis without compression of the surrounding lung parenchyma (1/5), or with its compression (4/5). The whole process was accompanied by multifocal (1/5), dispersed (2/5), or organized inflammation in aggregates and lymphoid follicles (2/5). As a result of continuous fibroproduction and maturation of the connective tissue, the alveolar septa thickened, delimiting groups of alveoli that merged into air bullae. Few indistinctly visible (2/5), few clearly visible (1/5), multiple indistinctly visible (1/5), and multiple clearly visible (1/5) fibroblastic foci were present. Among the concomitant changes, areas of emphysema, bronchioloectasia, and bronchiectasis, as well as bronchial and vessel wall hypertrophy, and mucostasis in the alveoli and edema were observed. The differences in the histological appearance of usual interstitial pneumonia associated with systemic connective tissue diseases (CTD-UIP) versus the pattern associated with idiopathic pulmonary fibrosis (IPF-UIP) are discussed here. The main differences lie in spreading lung fibrosis, architectural lung remodeling, fibroblastic foci, and inflammatory infiltrates.es
dc.formatapplication/pdfes
dc.format.extent8es
dc.identifier.doihttps://doi.org/10.14670/HH-18-777
dc.identifier.eisbnHistology and Histopathology Vol. 40, nº01 (2025)es
dc.identifier.issn0213-3911
dc.identifier.issn1699-5848
dc.identifier.urihttp://hdl.handle.net/10201/148042
dc.languageenges
dc.publisherUniversidad de Murcia, Departamento de Biologia Celular e Histiologiaes
dc.relationSin financiación externa a la Universidades
dc.rightsinfo:eu-repo/semantics/openAccesses
dc.rightsAttribution-NonCommercial-NoDerivatives 4.0 Internacional*
dc.rights.urihttp://creativecommons.org/licenses/by-nc-nd/4.0/*
dc.subjectFibroblastic foci,es
dc.subjectHoneycombinges
dc.subjectIdiopathic pulmonary fibrosises
dc.subjectInterstitial lung diseasees
dc.subjectProgressive pulmonary fibrosises
dc.subject.otherCDU::6 - Ciencias aplicadas::61 - Medicina::616 - Patología. Medicina clínica. Oncologíaes
dc.titleHistopathological aspects of usual interstitial pneumonia in patients with systemic connective tissue diseaseses
dc.typeinfo:eu-repo/semantics/articlees
dspace.entity.typePublicationes
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Histology and histopathology Vol.40, nº1 (2025)