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
- dospělí MeSH
- idiopatická plicní fibróza patologie komplikace MeSH
- intersticiální plicní nemoci * patologie komplikace MeSH
- lidé středního věku MeSH
- lidé MeSH
- nemoci pojiva * patologie komplikace MeSH
- plíce patologie diagnostické zobrazování MeSH
- počítačová rentgenová tomografie MeSH
- senioři MeSH
- Check Tag
- dospělí MeSH
- lidé středního věku MeSH
- lidé MeSH
- mužské pohlaví MeSH
- senioři MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- kazuistiky MeSH
Pulmonary alveolar proteinosis (PAP) is a rare disease characterised by excessive accumulation of surfactant components in alveolar macrophages, alveoli, and peripheral airways. The accumulation of surfactant is associated with only a minimal inflammatory response but can lead to the development of pulmonary fibrosis. Three clinical forms of PAP are distinguished - primary, secondary and congenital. In recent years, significant findings have helped to clarify the ethiology and pathogenesis of the disease. Apart from impaired surfactant protein function, a key role in the development of PAP is played by signal pathway of granulocyte and macrophage colonies stimulating growth factor (GM-CSF) which is necessary for the functioning of alveolar macrophages and for surfactant homeostasis. Surfactant is partially degraded by alveolar macrophages that are stimulated by GM-CSF. The role of GM-CSF has been shown especially in primary PAP, which is currently considered an autoimmune disease involving the development of GM-CSF neutralising autoantibodies. Clinically, the disease may be silent or manifest with dyspnoeic symptoms triggered by exertion and cough. However, there is a 10 to 15% rate of patients who develop respiratory failure. Total pulmonary lavage is regarded as the standard method of treatment. In addition, recombinant human GM-CSF has been studied as a prospective therapy for the treatment of PAP.
- MeSH
- alveolární makrofágy * imunologie patologie MeSH
- faktor stimulující granulocyto-makrofágové kolonie * metabolismus MeSH
- lidé MeSH
- plicní alveolární proteinóza * patologie MeSH
- vzácné nemoci * patologie MeSH
- zvířata MeSH
- Check Tag
- lidé MeSH
- zvířata MeSH
- Publikační typ
- časopisecké články MeSH
- přehledy 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
- bronchoalveolární laváž metody MeSH
- bronchoskopie metody MeSH
- diagnostické zobrazování metody MeSH
- dospělí MeSH
- lidé středního věku MeSH
- lidé MeSH
- plicní alveolární proteinóza * diagnóza terapie MeSH
- Check Tag
- dospělí MeSH
- lidé středního věku MeSH
- lidé MeSH
- mužské pohlaví MeSH
- ženské pohlaví MeSH
- Publikační typ
- kazuistiky MeSH
Napriek zvyšujúcej sa životnej úrovni ľudstva, dostupnosti modernej antiinfekčnej liečby, informovanosti a vzdelanosti obyvateľstva, patrí tuberkulóza stále k najzávažnejším infekčným ochoreniam na svete. Lieková rezistencia je významnou hrozbou a jednou z hlavných výziev menežmentu tuberkulózy. Multirezistentná tuberkulóza je charakterizovaná rezistenciou na minimálne dve základné antituberkulotiká a to izoniazid a rifampicín. Trend výskytu rezistentných foriem tuberkulózy mal na Slovensku klesajúci charakter do roku 2020. K nárastu došlo v roku 2021, ku ktorému prispel aj presun migrantov z Ukrajiny, z krajiny s vysokou incidenciou farmakorezistentnej tuberkulózy. Rizikovými faktormi súvisiacimi s tuberkulózou sú mužské pohlavie, vyšší vek, abúzy (najmä fajčenie tabaku a pitie alkoholu), pridružené ochorenia, sociálno-ekonomické podmienky atď. Cieľom článku je priblížiť etiopatogenézu, klinický obraz, diagnostiku a liečbu tuberkulózy, vrátane nových liečebných režimov pre multirezistentné formy tuberkulózy. Súčasťou článku sú výsledky štatistickej analýzy dát 36 pacientov s multirezistentnou tuberkulózou. Definujeme vybrané parametre skúmaných pacientov, nenáhodné asociácie týchto parametrov medzi sebou, ako aj ich asociáciu s rezistenciou na vybrané antituberkulotiká. Z výsledkov vyplýva asociácia medzi pozitívnym spútom, dĺžkou fajčenia a BMI, ďalej zvyšujúca sa pravdepodobnosť úmrtia s pozitivitou spúta a rezistenciou na fluorochinolóny. Zistili sme tiež vyššiu šancu rezistencie na streptomycín pri rezistencii na etambutol.
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- MeSH
- analýza dat MeSH
- antituberkulotika farmakologie terapeutické užití MeSH
- bakteriální léková rezistence MeSH
- lidé MeSH
- multirezistentní tuberkulóza * chirurgie diagnóza mikrobiologie mortalita MeSH
- průzkumy zdravotní péče metody MeSH
- retrospektivní studie MeSH
- rizikové faktory MeSH
- Check Tag
- lidé MeSH
OBJECTIVES: The aim of the study was to evaluate pulmonary sequestration (PS). We report on location, blood supply, histology, clinical manifestation, and surgical treatment of PS, as well as on postoperative course in patients with PS. BACKGROUND: PS is a rare congenital defect of the lower respiratory tract, it represents locus minoris resistentiae of the body. Occasionally, PS is diagnosed for the first time in adulthood. METHODS: We evaluated 7 cases of PS treated at the Centre of Thoracic Surgery in Vyšné Hágy, Slovakia, between years 2013 and 2020. RESULTS: Four of our seven patients were asymptomatic; the PS was found incidentally upon chest imaging. Three patients had recurrent bronchopneumonia related specifically to the intralobar type of sequestration. The most significant complication, observed in a singular patient, was a life-threatening episode of haemoptysis, requiring urgent surgical intervention. In the other 6 cases, the sequestra were surgically resected during the period when they were asymptomatic. and their sputum was confirmed negative upon microbiological examination. Anatomical resection of the affected pulmonary lobe by thoracotomy was the most common type of operation performed (4 cases, n = 7). There was no surgical mortality. CONCLUSION: To prevent complications, it is crucial to perform surgical treatment for pulmonary sequestration in patients who have sufficient functional capacity (Tab. 2, Fig. 4, Ref. 30).
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.
Článok referuje troch pacientov so solitárnym fibróznym tumorom hrudníka. Prvý pacient mal nádor v oblasti kupoly pravej pohrudnicovej dutiny, ktorý bol radikálne resekovaný spolu s hrudníkovou stenou v okolí jeho origa. V druhom prípade išlo o nádor fixovaný cievnou stopkou k dolnému laloku pravých pľúc. Tento nádor bol resekovaný atypicky, cestou torakotómie, spolu s bezpečnostným lemom zdravého pľúcneho tkaniva v oblasti bázy jeho stopky. Posledný pacient mal nádor dolného laloka pravých pľúc, ktorý obklopoval dolnú pľúcnu žilu a miestami nemal zreteľnú hranicu so zdravým pľúcnym tkanivom. Nález si vyžiadal dolnú lobektómiu pravých pľúc cestou posterolaterálnej torakotómie. Kazuistiky poukazujú na zriedkavý typ nádorov v oblasti hrudníka, ktoré v čase zistenia dosahujú často veľké rozmery, nútiace k rozsiahlym operačným výkonom. Vzhľadom k biologickej povahe týchto nádorov, je vhodné daných pacientov dlhodobo dispenzarizovať.
The article reports on three patients with a solitary fibrous tumor of the chest. The first patient had a tumor in the area of the dome of the right pleural cavity which was radically resected together with the chest wall around its origin. In the second case, the tumor was attached by a vascular pedicle to the lower lobe of the right lung. This tumor was resected atypically, via thoracotomy, along with a margin of healthy lung tissue at the base of its pedicle. The last patient had a tumor of the lower lobe of the right lung, surrounding the lower pulmonary vein, which did not have a clear margin of healthy lung tissue. This finding required right lower lobectomy via posterolateral thoracotomy. The presented cases describe rare types of tumors in the chest area which at the time of detection often reach large dimensions, necessitating extensive surgical procedures. Due to the biological nature of these tumors, long-term patient follow-up is advisable.
- MeSH
- dospělí MeSH
- dyspnoe etiologie MeSH
- hrudník diagnostické zobrazování patologie MeSH
- lidé středního věku MeSH
- lidé MeSH
- nádory pleury chirurgie diagnostické zobrazování patologie MeSH
- nádory plic chirurgie diagnostické zobrazování patologie MeSH
- pleurální dutina chirurgie diagnostické zobrazování patologie MeSH
- počítačová rentgenová tomografie MeSH
- senioři MeSH
- solitární fibrózní tumory * chirurgie diagnostické zobrazování patologie MeSH
- výsledek terapie MeSH
- Check Tag
- dospělí MeSH
- lidé středního věku MeSH
- lidé MeSH
- senioři MeSH
- Publikační typ
- kazuistiky MeSH
- Geografické názvy
- Slovenská republika MeSH
Súhrn Článok referuje prípad pacienta s bronchopulmonálnou sekvestráciou komplikovanou deštruktívnym aktinomykotickým zápalom vedúcim k život ohrozujúcemu hemoptoe. Išlo o dospelého pacienta s anamnézou opakovaných pravostranných zápalov pľúc, ktorých komplexná príčina nebola v minulosti bližšie vyšetrovaná. K bližšiemu vyšetrovaniu pozadia zápalov pľúc viedla až hemoptýza, ktorá sa objavila ako komplikácia. CT vyšetrenie hrudníka odhalilo léziu stredného laloka pravých pľúc s anomálnou vaskularizáciou – obraz kompatibilný s intralobárnou pľúcnou sekvestráciou. Spádové pracovisko, kde bol pacient zachytený, začalo konzervatívnu antibiotickú liečbu zápalu pľúc. Pre pretrvávanie vykašliavania krvi bola indikovaná embolizácia aferentných ciev sekvestra, ktorá viedla k redukcii jeho prekrvenia, čo bolo preukázané kontrolným CT vyšetrením hrudníka. Klinicky došlo k ústupu hemoptýzy. Neskôr, s odstupom troch týždňov, nastala recidíva hemoptýzy. Pacient bol akútne hospitalizovaný na špecializovanom pracovisku hrudníkovej chirurgie, kde krátko po prijatí došlo k progresii hemoptýzy do život ohrozujúceho hemoptoe. Pacient, za účelom sanácie zdroja krvácania, urgentne podstúpil strednú lobektómiu pravých pľúc cestou torakotómie. Prípad poukazuje na nerozpoznanú bronchopulmonálnu sekvestráciu ako na možnú príčinu recidivujúcich ipsilaterálnych zápalov pľúc v dospelosti, zdôrazňuje možné riziká spojené s patologicky zmeneným tkanivovým mikroprostredím pľúcneho sekvestra a akcentuje potrebu chirurgického riešenia vo všetkých indikovaných prípadoch.
The article reports the case of a patient with bronchopulmonary sequestration complicated by destructive actinomycotic inflammation leading to life-threatening hemoptysis. It was an adult patient with the history of repeated right-sided pneumonia the cause of which had not been investigated in detail in the past. Only hemoptysis, which appeared as a complication, led to a closer investigation of the background of repeated right-sided pneumonia. CT scan of the chest revealed a lesion of the middle lobe of the right lung with anomalous vascularization – compatible with intralobar sequestration. Initially, conservative antibiotic treatment of pneumonia was provided at a local clinic. Embolization of the afferent vessels of the sequestrum was indicated due to persistent hemoptysis; this led to a reduction of its blood supply, proven by a follow-up CT examination of the chest. Clinically, the hemoptysis subsided. Three weeks later, the hemoptysis reocurred. The patient was acutely hospitalized at a specialized thoracic surgery department where shortly after admission, hemoptysis progressed to life-threatening hemoptea. Urgent middle lobectomy of the right lung was approached via thoracotomy to treat the source of bleeding. The case describes unrecognized bronchopulmonary sequestration as a possible cause of recurrent ipsilateral pneumonia in adulthood; additionally, it emphasizes the possible risks associated with a pathologically altered tissue microenvironment of pulmonary sequestration, and the need for surgical removal in all indicated cases.
