Distinct Airway Involvement in Subtypes of End-Stage Fibrotic Pulmonary Sarcoidosis

Verleden, Stijn E.; Vanstapel, Arno; Sadeleer, Laurens J. De; Dubbeldam, Adriana; Goos, Tinne; Gyselinck, Iwein; Geudens, Vincent; Kaes, Janne; Raemdonck, Dirk Van; Ceulemans, Laurens J.; Yserbyt, Jonas; Vos, Robin; Vanaudenaerde, Bart M.; Weynand, Birgit; Verschakelen, Johny; Wuyts, Wim

doi:10.1016/j.chest.2021.01.003

Cited by 12 publications

(13 citation statements)

References 22 publications

(25 reference statements)

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“…113,114 Verleden and colleagues correlated histopathologic features to CT and micro-CT of explanted lungs in patients with end-stage sarcoid and identified three main patterns. 117 Firstly, central fibrotic masses were associated with compression and obstruction of the centrilobular airways, loss of small airways, and architectural distortion. A second pattern of diffuse bronchiectasis was associated with septal thickening and upper lobe subpleural fibrosis.…”

Section: Sarcoidosismentioning

confidence: 99%

High-Resolution Computed Tomography of Fibrotic Interstitial Lung Disease

Rodriguez

Ashby

Varela

et al. 2022

Semin Respir Crit Care Med

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While radiography is the first-line imaging technique for evaluation of pulmonary disease, high-resolution computed tomography (HRCT) provides detailed assessment of the lung parenchyma and interstitium, allowing normal anatomy to be differentiated from superimposed abnormal findings. The fibrotic interstitial lung diseases have HRCT features that include reticulation, traction bronchiectasis and bronchiolectasis, honeycombing, architectural distortion, and volume loss. The characterization and distribution of these features result in distinctive CT patterns. The CT pattern and its progression over time can be combined with clinical, serologic, and pathologic data during multidisciplinary discussion to establish a clinical diagnosis. Serial examinations identify progression, treatment response, complications, and can assist in determining prognosis. This article will describe the technique used to perform HRCT, the normal and abnormal appearance of the lung on HRCT, and the CT patterns identified in common fibrotic lung diseases.

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Section: Sarcoidosismentioning

confidence: 99%

High-Resolution Computed Tomography of Fibrotic Interstitial Lung Disease

Rodriguez

Ashby

Varela

et al. 2022

Semin Respir Crit Care Med

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“…Хирургические диагностические операции рекомендуются всем пациентам (дети и взрослые) при невозможности малоинвазивной диагностики для верификации диагноза, принимать решение рекомендуется лечащему врачу во взаимодействии с врачом-эндоскопистом и врачом-хирургом [20,[65][66][67][68][69][70][71].…”

Section: уровень убедительности рекомендаций с (уровень достоверности...unclassified

“…Инвазивная диагностика саркоидоза внелегочной локализации (биопсия периферического ЛУ, кожи, опухолей, опухолеподобных образований мягких тканей (подкожные образования), печени и других органов в соответствии с поражением) рекомендуется пациентам с саркоидозом для получения материала пораженного органа. Выбор метода получения материала рекомендуется проводить лечащим врачом совместно с врачом-хирургом [20,[65][66][67][68][69][70][71][72][73][74].…”

Section: уровень убедительности рекомендаций B (уровень достоверности...unclassified

Sarcoidosis: federal clinical guidelines for diagnosis and treatment

Чучалин

Avdeev

Айсанов

et al. 2022

Pulʹmonologiâ (Mosk.)

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Sarcoidosis is a multi-organ granulomatosis of unknown origin. Modern diagnostic methods allow detecting this disease at an early stage. The absence of specific markers requires a comprehensive approach to diagnosis based on comparison of radiation, clinical, morphological and functional data. The course of sarcoidosis without damage to the respiratory system presents significant difficulties. It is extremely important to understand the time and means of starting Sarcoidosis’s treatment to avoid the early initiation of hormones and cytostatics and, on the other hand, to timely respond to progression and threatening conditions. Methods. Clinical recommendations are based on the analysis of Russian and English publications of the latest sarcoidosis research. The target audience of these clinical guidelines are therapists, general practitioners, pulmonologists, TB doctors, rheumatologists, dermatologists, radiation diagnosticians, immunologists, and clinical pharmacologists. Each thesis-recommendation for diagnosis and treatment is evaluated on an 1 to 5 scale of levels of evidence and an A, B, C scale of the grades of recommendations. The clinical guidelines also contain comments and explanations for the theses-recommendations, diagnostic algorithms, treatment strategies, reference materials on the use of recommended drugs. Conclusion. Current information on epidemiology, clinical manifestations, diagnosis and management strategies for patients with sarcoidosis are covered in the presented clinical guidelines. Approved by the decision of the Scientific and Practical Council of the Ministry of Health of the Russian Federation (2022).

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“…Diversely, in UIP pattern, there is more initial diffuse parenchymal fibrosis and the airways become soon dilated, while the terminal bronchioles are spared resulting in a totally remodelled matrix with less involvement of the small airways. Preventing small airways loss could be an important future therapeutic target in end-stage sarcoidosis [31 ▪ ].…”

Section: From Granuloma To Progressive Fibrosismentioning

confidence: 99%

“…Fibrocystic lesions appear typically larger (macrocystic) than traditional honeycomb lesions and typically affect the upper and perihilar/peribronchial regions [41]. Some CT features may discriminate between active lung inflammation and fibrosis: inflammatory findings include a bilateral distribution of micronodules, perilymphatic and bronchocentric distribution, and perihilar ground-glass opacities [31 ▪ ,33].…”

Section: Diagnosismentioning

confidence: 99%

Novel insights in fibrotic pulmonary sarcoidosis

Comes

Chrisafi

Richeldi

2022

Current Opinion in Pulmonary Medicine

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Purpose of reviewIn chronic pulmonary sarcoidosis, the transition from the inflammatory to the fibrotic stage of the lungs occurs in about 10--20% of cases, eventually causing end-stage fibrotic disease. To date, pathogenetic mechanisms and clinical management remain challenging; thus, we highlight the recent evidence in pulmonary fibrotic processes, clinical signs for an early detection and the potential role of the current investigated antifibrotic agents and promising targeted therapies. Recent findingsRecent findings of relevant key cellular pathways can be considered as a glimmer of light in the complexity of sarcoidosis. In some patients, granulomas persist and serve as a nidus for fibrosis growth, sustained by several fibrosis-stimulating cytokines. Preclinical studies have detected profibrotic, antifibrotic and pleiotropic T cells as promoters of fibrosis. Epigenetics, genetics and transcriptomics research can lead to new target therapies. Antifibrotic drug nintedanib has shown a positive effect on non-idiopathic pulmonary fibrosis fibrotic lung diseases including fibrotic sarcoidosis; other antifibrotic drugs are under investigation. SummaryPulmonary fibrosis strongly impacts the outcome of sarcoidosis, and a better understanding of the underlying pathogenic mechanisms can facilitate the development of novel treatments, improving clinical care and life expectancy of these patients. The greatest challenge is to investigate effective antifibrotic therapies once fibrosis develops. The role of these findings in fibrotic sarcoidosis can be translated into other interstitial lung diseases characterized by the coexistence of inflammatory and fibrotic processes.

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Distinct Airway Involvement in Subtypes of End-Stage Fibrotic Pulmonary Sarcoidosis

Cited by 12 publications

References 22 publications

High-Resolution Computed Tomography of Fibrotic Interstitial Lung Disease

High-Resolution Computed Tomography of Fibrotic Interstitial Lung Disease

Sarcoidosis: federal clinical guidelines for diagnosis and treatment

Novel insights in fibrotic pulmonary sarcoidosis

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