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Pulmonary Sarcoidosis: Diagnosis and Differential Diagnosis

Sarcoidosis is a multisystem disorder of unknown origin and poorly understood pathogenesis that predominantly affects lungs and intrathoracic lymph nodes and is characterized by the presence of noncaseating granulomatous inflammation in involved organs. The disease is highly heterogeneous and can mi...

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Autores principales: Bernardinello, Nicol, Petrarulo, Simone, Balestro, Elisabetta, Cocconcelli, Elisabetta, Veltkamp, Marcel, Spagnolo, Paolo
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8472810/
https://www.ncbi.nlm.nih.gov/pubmed/34573900
http://dx.doi.org/10.3390/diagnostics11091558
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author Bernardinello, Nicol
Petrarulo, Simone
Balestro, Elisabetta
Cocconcelli, Elisabetta
Veltkamp, Marcel
Spagnolo, Paolo
author_facet Bernardinello, Nicol
Petrarulo, Simone
Balestro, Elisabetta
Cocconcelli, Elisabetta
Veltkamp, Marcel
Spagnolo, Paolo
author_sort Bernardinello, Nicol
collection PubMed
description Sarcoidosis is a multisystem disorder of unknown origin and poorly understood pathogenesis that predominantly affects lungs and intrathoracic lymph nodes and is characterized by the presence of noncaseating granulomatous inflammation in involved organs. The disease is highly heterogeneous and can mimic a plethora of other disorders, making diagnosis a challenge even for experienced physicians. The evolution and severity of sarcoidosis are highly variable: many patients are asymptomatic and their disease course is generally benign with spontaneous resolution. However, up to one-third of patients develop chronic or progressive disease mainly due to pulmonary or cardiovascular complications that require long-term therapy. The diagnosis of sarcoidosis requires histopathological evidence of noncaseating granulomatous inflammation in one or more organs coupled with compatible clinical and radiological features and the exclusion of other causes of granulomatous inflammation; however, in the presence of typical disease manifestations such as Löfgren’s syndrome, Heerfordt’s syndrome, lupus pernio and asymptomatic bilateral and symmetrical hilar lymphadenopathy, the diagnosis can be established with high level of certainty on clinical grounds alone. This review critically examines the diagnostic approach to sarcoidosis and emphasizes the importance of a careful exclusion of alternative diagnoses.
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spelling pubmed-84728102021-09-28 Pulmonary Sarcoidosis: Diagnosis and Differential Diagnosis Bernardinello, Nicol Petrarulo, Simone Balestro, Elisabetta Cocconcelli, Elisabetta Veltkamp, Marcel Spagnolo, Paolo Diagnostics (Basel) Review Sarcoidosis is a multisystem disorder of unknown origin and poorly understood pathogenesis that predominantly affects lungs and intrathoracic lymph nodes and is characterized by the presence of noncaseating granulomatous inflammation in involved organs. The disease is highly heterogeneous and can mimic a plethora of other disorders, making diagnosis a challenge even for experienced physicians. The evolution and severity of sarcoidosis are highly variable: many patients are asymptomatic and their disease course is generally benign with spontaneous resolution. However, up to one-third of patients develop chronic or progressive disease mainly due to pulmonary or cardiovascular complications that require long-term therapy. The diagnosis of sarcoidosis requires histopathological evidence of noncaseating granulomatous inflammation in one or more organs coupled with compatible clinical and radiological features and the exclusion of other causes of granulomatous inflammation; however, in the presence of typical disease manifestations such as Löfgren’s syndrome, Heerfordt’s syndrome, lupus pernio and asymptomatic bilateral and symmetrical hilar lymphadenopathy, the diagnosis can be established with high level of certainty on clinical grounds alone. This review critically examines the diagnostic approach to sarcoidosis and emphasizes the importance of a careful exclusion of alternative diagnoses. MDPI 2021-08-28 /pmc/articles/PMC8472810/ /pubmed/34573900 http://dx.doi.org/10.3390/diagnostics11091558 Text en © 2021 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Review
Bernardinello, Nicol
Petrarulo, Simone
Balestro, Elisabetta
Cocconcelli, Elisabetta
Veltkamp, Marcel
Spagnolo, Paolo
Pulmonary Sarcoidosis: Diagnosis and Differential Diagnosis
title Pulmonary Sarcoidosis: Diagnosis and Differential Diagnosis
title_full Pulmonary Sarcoidosis: Diagnosis and Differential Diagnosis
title_fullStr Pulmonary Sarcoidosis: Diagnosis and Differential Diagnosis
title_full_unstemmed Pulmonary Sarcoidosis: Diagnosis and Differential Diagnosis
title_short Pulmonary Sarcoidosis: Diagnosis and Differential Diagnosis
title_sort pulmonary sarcoidosis: diagnosis and differential diagnosis
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8472810/
https://www.ncbi.nlm.nih.gov/pubmed/34573900
http://dx.doi.org/10.3390/diagnostics11091558
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