Five 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.
- MeSH
- Adult MeSH
- Idiopathic Pulmonary Fibrosis pathology complications MeSH
- Lung Diseases, Interstitial * pathology complications MeSH
- Middle Aged MeSH
- Humans MeSH
- Connective Tissue Diseases * pathology complications MeSH
- Lung pathology diagnostic imaging MeSH
- Tomography, X-Ray Computed MeSH
- Aged MeSH
- Check Tag
- Adult MeSH
- Middle Aged MeSH
- Humans MeSH
- Male MeSH
- Aged MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
- Case Reports MeSH
V našom článku, na podklade dvoch prípadov z praxe, popisujeme priebeh, diagnostiku a liečbu pľúcnej alveolárnej proteinózy (PAP). V prvom prípade ide o 52-ročnú ženu, v druhom o 34-ročného muža. Obaja pacienti boli vyšetrovaní spádovým pneumológom pre intersticiálne pľúcne ochorenie, v prvom prípade aj s prechodom do pľúcnej fibrózy. V rámci diferenciálnej diagnostiky boli hospitalizovaní v NÚTPCHaHCH vo Vyšných Hágoch. RTG hrudníka ukázalo nález difúznych obojstranných infiltrátov pľúc, ktoré u prvého pacienta vykazovali miestami splývavý charakter. CT hrudníka zobrazilo parenchýmové postihnutie pľúc s obojstrannými areálmi charakteru mliečneho skla („ground-glass opacity“/GGO) so zhrubnutými interlobulárnymi septami (crazy paving). U oboch pacientov bolo urobené bronchoskopické vyšetrenie s bronchoalveolárnou lavážou, ktorá mala charakteristický mliečne skalený vzhľad. Pacientom bola dodatočne indikovaná videotorakoskopická biopsia pľúc a histopatologicky potvrdená pľúcna alveolárna proteinóza. Terapeuticky pacienti podstúpili veľkoobjemovú laváž pľúc, po ktorej došlo k zlepšeniu ich klinického stavu a tiež zlepšeniu rádiologického nálezu. Poukazujeme na základné piliere diagnostiky pľúcnej alveolárnej proteinózy, ktorými sú vzor pľúcneho postihnutia v RTG a CT (resp. HRCT) obraze, charakteristický vzhľad bronchoalveolárnej lavážovej tekutiny a dodatočne dokumentujeme aj histopatologický obraz pľúcneho postihnutia pri tomto ochorení. Akcentujeme potrebu centralizácie manažmentu pacientov s pľúcnymi ochoreniami, ktorá je obzvlášť naliehavou v prípadoch raritných ochorení, kedy umožňuje rýchlu dostupnosť všetkých relevantných diagnostických a terapeutických možností, vrátane veľkoobjemovej laváže pľúc.
In this article, we describe the course and diagnosis of pulmonary alveolar proteinosis (PAP) based on two cases from our practice. The first case is a 52-yearold woman, the second a 34-year-old man. Both referred patients were examined by a pulmonologist for interstitial lung disease, in the first case also with transition to pulmonary fibrosis. As part of the differential diagnosis, these patients were hospitalized at the NÚTPCHaHCH in Vyšné Hágy. Chest X-ray showed diffuse bilateral lung infiltrates, in the first patient locally confluent. Chest CT showed parenchymal involvement of the lungs with bilateral ground-glass opacities with thickened interlobular septa (crazy paving). Bronchoscopic examination was performed in both patients with bronchoalveolar lavage, which had a characteristic milky-glazed appearance. Videothoracoscopic lung biopsy was additionally indicated and histopathologically there were pulmonary alveolar proteinosis confirmed. Therapeutically, the patients underwent large volume lung lavage, with clinical condition improvement, including radiological findings improvement. We point out the basic pillars of the diagnosis of pulmonary alveolar proteinosis, which are the pattern of pulmonary involvement in the radiographic and CT (or HRCT) images, the characteristic appearance of the bronchoalveolar lavage fluid, and additionally also the histopathologic pattern of pulmonary involvement in this disease. We emphasize the need for centralized management of patients with lung diseases, which is particularly urgent in cases of rare diseases, where it provides rapid availability of all relevant diagnostic and therapeutic options, including large-volume lung lavage.
- MeSH
- Bronchoalveolar Lavage methods MeSH
- Bronchoscopy methods MeSH
- Diagnostic Imaging methods MeSH
- Adult MeSH
- Middle Aged MeSH
- Humans MeSH
- Pulmonary Alveolar Proteinosis * diagnosis therapy MeSH
- Check Tag
- Adult MeSH
- Middle Aged MeSH
- Humans MeSH
- Male MeSH
- Female MeSH
- Publication type
- Case Reports MeSH
The objective of this article is to describe and classify usual interstitial pneumonia (UIP) changes according to their relevance in the pathology of the idiopathic pulmonary fibrosis (IPF) process. In a cohort of 50 patients (25♀, 25♂) with UIP findings, the percentage ratio between fibrotic and preserved parts of the lungs was quantified. Three quantitative stages of fibrotic involvement of the lung parenchyma and concomitant changes were defined. These are initial (≤20%), advanced (21-40%), and diffuse (≥41%) fibrosis of the lungs. Histologically, temporal heterogeneity is predominant with thickened alveolar septa, interstitial fibrosis, and the presence of fibroblastic foci up to mature diffuse fibrosis with honeycomb changes. The finding is accompanied by variably mature lymphocytic inflammation, presence of macrophages, emphysema, bronchioloectasia of the alveoli, bronchiectasis, bronchial muscle wall hypertrophy, hypertrophy of the vessel walls, alveolar mucosa, focal haemorrhage, and hyalinization of the lungs. Pneumocyte hyperplasia, occasionally atypical in appearance with hobnail changes, as well as squamous metaplasia are observed. In the methodically quantified stages of fibrous involvement, 14 subjects were classified (6♀, 8♂) into the stage of initial fibrosis, 21 subjects (11♀; 10♂) into the stage of advanced fibrosis, and 15 subjects (8♀; 7♂) into the stage of diffuse fibrosis.
