Journal of Clinical Medicine

Editorial

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2 pages, 153 KiB

Open AccessEditorial

The Chameleon Behavior of Sarcoidosis

by Claudio Tana and Cosima Schiavone

J. Clin. Med. 2021, 10(13), 2780; https://doi.org/10.3390/jcm10132780 - 24 Jun 2021

Cited by 7 | Viewed by 1624

Abstract

Sarcoidosis is a multisystem disease that raises several diagnostic difficulties in routine clinical practice due to its multisystemic involvement and the presence of nonspecific clinical pictures, except in some isolated cases [...] Full article

(This article belongs to the Special Issue New Frontiers in the Diagnosis of Sarcoidosis)

Review

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15 pages, 2491 KiB

Open AccessReview

Histology of Cardiac Sarcoidosis with Novel Considerations Arranged upon a Pathologic Basis

by Shu Kato, Yasuhiro Sakai, Asako Okabe, Yoshiaki Kawashima, Kazuhiko Kuwahara, Kazuya Shiogama, Masato Abe, Hiroyasu Ito and Shin’ichiro Morimoto

J. Clin. Med. 2022, 11(1), 251; https://doi.org/10.3390/jcm11010251 - 4 Jan 2022

Cited by 16 | Viewed by 3508

Abstract

Sarcoidosis is a rare disease of isolated or diffuse granulomatous inflammation. Although any organs can be affected by sarcoidosis, cardiac sarcoidosis is a fatal disorder, and it is crucial to accurately diagnose it to prevent sudden death due to dysrhythmia. Although endomyocardial biopsy [...] Read more.

Sarcoidosis is a rare disease of isolated or diffuse granulomatous inflammation. Although any organs can be affected by sarcoidosis, cardiac sarcoidosis is a fatal disorder, and it is crucial to accurately diagnose it to prevent sudden death due to dysrhythmia. Although endomyocardial biopsy is invasive and has limited sensitivity for identifying granulomas, it is the only modality that yields a definitive diagnosis of cardiac sarcoidosis. It is imperative to develop novel pathological approaches for the precise diagnosis of cardiac sarcoidosis. Here, we aimed to discuss commonly used diagnostic criteria for cardiac sarcoidosis and to summarize useful and novel histopathologic criteria of cardiac sarcoidosis. While classical histologic observations including noncaseating granulomas and multinucleated giant cells (typically Langhans type) are the most important findings, others such as microgranulomas, CD68⁺ CD163⁻ pro-inflammatory (M1) macrophage accumulation, CD4/CD8 T-cell ratio, Cutibacterium acnes components, lymphangiogenesis, confluent fibrosis, and fatty infiltration may help to improve the sensitivity of endomyocardial biopsy for detecting cardiac sarcoidosis. These novel histologic findings are based on the pathology of cardiac sarcoidosis. We also discussed the principal histologic differential diagnoses of cardiac sarcoidosis, such as tuberculosis myocarditis, fungal myocarditis, giant cell myocarditis, and dilated cardiomyopathy. Full article

(This article belongs to the Special Issue New Frontiers in the Diagnosis of Sarcoidosis)

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15 pages, 5748 KiB

Open AccessReview

Advances in Diagnostic Imaging for Cardiac Sarcoidosis

by Osamu Manabe, Noriko Oyama-Manabe, Tadao Aikawa, Satonori Tsuneta and Nagara Tamaki

J. Clin. Med. 2021, 10(24), 5808; https://doi.org/10.3390/jcm10245808 - 11 Dec 2021

Cited by 9 | Viewed by 4600

Abstract

Sarcoidosis is a systemic granulomatous disease of unknown etiology, and its clinical presentation depends on the affected organ. Cardiac sarcoidosis (CS) is one of the leading causes of death among patients with sarcoidosis. The clinical manifestations of CS are heterogeneous, and range from [...] Read more.

Sarcoidosis is a systemic granulomatous disease of unknown etiology, and its clinical presentation depends on the affected organ. Cardiac sarcoidosis (CS) is one of the leading causes of death among patients with sarcoidosis. The clinical manifestations of CS are heterogeneous, and range from asymptomatic to life-threatening arrhythmias and progressive heart failure due to the extent and location of granulomatous inflammation in the myocardium. Advances in imaging techniques have played a pivotal role in the evaluation of CS because histological diagnoses obtained by myocardial biopsy tend to have lower sensitivity. The diagnosis of CS is challenging, and several approaches, notably those using positron emission tomography and cardiac magnetic resonance imaging (MRI), have been reported. Delayed-enhanced computed tomography (CT) may also be used for diagnosing CS in patients with MRI-incompatible devices and allows acceptable evaluation of myocardial hyperenhancement in such patients. This article reviews the advances in imaging techniques for the evaluation of CS. Full article

(This article belongs to the Special Issue New Frontiers in the Diagnosis of Sarcoidosis)

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12 pages, 2521 KiB

Open AccessReview

Novelties in Imaging of Thoracic Sarcoidosis

by Lucio Calandriello, Rosa D’Abronzo, Giuliana Pasciuto, Giuseppe Cicchetti, Annemilia del Ciello, Alessandra Farchione, Cecilia Strappa, Riccardo Manfredi and Anna Rita Larici

J. Clin. Med. 2021, 10(11), 2222; https://doi.org/10.3390/jcm10112222 - 21 May 2021

Cited by 9 | Viewed by 3108

Abstract

Sarcoidosis is a systemic granulomatous disease affecting various organs, and the lungs are the most commonly involved. According to guidelines, diagnosis relies on a consistent clinical picture, histological demonstration of non-caseating granulomas, and exclusion of other diseases with similar histological or clinical picture. [...] Read more.

Sarcoidosis is a systemic granulomatous disease affecting various organs, and the lungs are the most commonly involved. According to guidelines, diagnosis relies on a consistent clinical picture, histological demonstration of non-caseating granulomas, and exclusion of other diseases with similar histological or clinical picture. Nevertheless, chest imaging plays an important role in both diagnostic assessment, allowing to avoid biopsy in some situations, and prognostic evaluation. Despite the demonstrated lower sensitivity of chest X-ray (CXR) in the evaluation of chest findings compared to high-resolution computed tomography (HRCT), CXR still retains a pivotal role in both diagnostic and prognostic assessment in sarcoidosis. Moreover, despite the huge progress made in the field of radiation dose reduction, chest magnetic resonance (MR), and quantitative imaging, very little research has focused on their application in sarcoidosis. In this review, we aim to describe the latest novelties in diagnostic and prognostic assessment of thoracic sarcoidosis and to identify the fields of research that require investigation. Full article

(This article belongs to the Special Issue New Frontiers in the Diagnosis of Sarcoidosis)

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11 pages, 1791 KiB

Open AccessReview

Clinical Features and Diagnosis of Cardiac Sarcoidosis

by Claudio Tana, Cesare Mantini, Iginio Donatiello, Luciano Mucci, Marco Tana, Fabrizio Ricci, Francesco Cipollone and Maria Adele Giamberardino

J. Clin. Med. 2021, 10(9), 1941; https://doi.org/10.3390/jcm10091941 - 1 May 2021

Cited by 12 | Viewed by 3846

Abstract

Cardiac sarcoidosis (CS) is an unusual, but potentially harmful, manifestation of systemic sarcoidosis (SA), a chronic disease characterized by organ involvement from noncaseating and nonnecrotizing granulomas. Lungs and intrathoracic lymph nodes are usually the sites that are most frequently affected, but no organ [...] Read more.

Cardiac sarcoidosis (CS) is an unusual, but potentially harmful, manifestation of systemic sarcoidosis (SA), a chronic disease characterized by organ involvement from noncaseating and nonnecrotizing granulomas. Lungs and intrathoracic lymph nodes are usually the sites that are most frequently affected, but no organ is spared and CS can affect a variable portion of SA patients, up to 25% from post-mortem studies. The cardiovascular involvement is usually associated with a bad prognosis and is responsible for the major cause of death and complications, particularly in African American patients. Furthermore, the diagnosis is often complicated by the occurrence of non-specific clinical manifestations, which can mimic the effect of more common heart disorders, and imaging and biopsies are the most valid approach to avoid misdiagnosis. This narrative review summarizes the main clinical features of CS and imaging findings, particularly of CMR and 18-Fluorodeoxyglucose Positron Emission Tomography (18F-FDG PET) that can give the best cost/benefit ratio in terms of the diagnostic approach. Imaging can be very useful in replacing the endomyocardial biopsy in selected cases, to avoid unnecessary, and potentially dangerous, invasive maneuvers. Full article

(This article belongs to the Special Issue New Frontiers in the Diagnosis of Sarcoidosis)

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14 pages, 6433 KiB

Open AccessReview

Immunohistochemical Detection of Potential Microbial Antigens in Granulomas in the Diagnosis of Sarcoidosis

by Tetsuo Yamaguchi, Ulrich Costabel, Andrew McDowell, Josune Guzman, Keisuke Uchida, Kenichi Ohashi and Yoshinobu Eishi

J. Clin. Med. 2021, 10(5), 983; https://doi.org/10.3390/jcm10050983 - 2 Mar 2021

Cited by 29 | Viewed by 3647

Abstract

Sarcoidosis may have more than a single causative agent, including infectious and non-infectious agents. Among the potential infectious causes of sarcoidosis, Mycobacterium tuberculosis and Propionibacterium acnes are the most likely microorganisms. Potential latent infection by both microorganisms complicates the findings of molecular and [...] Read more.

Sarcoidosis may have more than a single causative agent, including infectious and non-infectious agents. Among the potential infectious causes of sarcoidosis, Mycobacterium tuberculosis and Propionibacterium acnes are the most likely microorganisms. Potential latent infection by both microorganisms complicates the findings of molecular and immunologic studies. Immune responses to potential infectious agents of sarcoidosis should be considered together with the microorganisms detected in sarcoid granulomas, because immunologic reactivities to infectious agents reflect current and past infection, including latent infection unrelated to the cause of the granuloma formation. Histopathologic data more readily support P. acnes as a cause of sarcoidosis compared with M. tuberculosis, suggesting that normally symbiotic P. acnes leads to granuloma formation in some predisposed individuals with Th1 hypersensitivity against intracellular proliferation of latent P. acnes, which may be triggered by certain host or drug-induced conditions. Detection of bacterial nucleic acids in granulomas does not necessarily indicate co-localization of the bacterial proteins in the granulomas. In the histopathologic diagnosis of sarcoidosis, M. tuberculosis-associated and P. acnes-associated sarcoidosis will possibly be differentiated in some patients by immunohistochemistry with appropriate antibodies that specifically react with mycobacterial and propionibacterial antigens, respectively, for each etiology-based diagnosis and potential antimicrobial intervention against sarcoidosis. Full article

(This article belongs to the Special Issue New Frontiers in the Diagnosis of Sarcoidosis)

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18 pages, 4580 KiB

Open AccessReview

CT Findings in Pulmonary and Abdominal Sarcoidosis. Implications for Diagnosis and Classification

by Claudio Tana, Iginio Donatiello, Maria Gabriella Coppola, Fabrizio Ricci, Marica Tina Maccarone, Tiziana Ciarambino, Francesco Cipollone and Maria Adele Giamberardino

J. Clin. Med. 2020, 9(9), 3028; https://doi.org/10.3390/jcm9093028 - 20 Sep 2020

Cited by 20 | Viewed by 11438

Abstract

Sarcoidosis is a granulomatous disorder of unknown etiology characterized by noncaseating granulomas virtually in every organ and tissue. This finding represents the most important diagnostic clue to reach a correct definition of sarcoidosis, although the biopsy is invasive and has several risk procedures. [...] Read more.

Sarcoidosis is a granulomatous disorder of unknown etiology characterized by noncaseating granulomas virtually in every organ and tissue. This finding represents the most important diagnostic clue to reach a correct definition of sarcoidosis, although the biopsy is invasive and has several risk procedures. Several efforts are made to suspect the diagnosis of sarcoidosis by combining noninvasive elements, in particular from imaging, though these findings are often nonspecific and reflect the wide multifactorial pathogenesis. Every effort should be made to obtain a detailed radiological picture that, if associated with a suggestive clinical picture, could avoid the need of biopsy in some specific cases. In this narrative review, we aim to describe main computed tomography (CT) features of pulmonary and abdominal sarcoidosis, by reporting strengths and limits of this technique, in particular for the identification of extrapulmonary, isolated disease. Full article

(This article belongs to the Special Issue New Frontiers in the Diagnosis of Sarcoidosis)

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17 pages, 2424 KiB

Open AccessReview

Clinical Presentations, Pathogenesis, and Therapy of Sarcoidosis: State of the Art

by Francesca Polverino, Elisabetta Balestro and Paolo Spagnolo

J. Clin. Med. 2020, 9(8), 2363; https://doi.org/10.3390/jcm9082363 - 24 Jul 2020

Cited by 32 | Viewed by 7369

Abstract

Sarcoidosis is a systemic disease of unknown etiology characterized by the presence of noncaseating granulomas that can occur in any organ, most commonly the lungs. Early and accurate diagnosis of sarcoidosis remains challenging because initial presentations may vary, many patients are asymptomatic, and [...] Read more.

Sarcoidosis is a systemic disease of unknown etiology characterized by the presence of noncaseating granulomas that can occur in any organ, most commonly the lungs. Early and accurate diagnosis of sarcoidosis remains challenging because initial presentations may vary, many patients are asymptomatic, and there is no single reliable diagnostic test. Prognosis is variable and depends on epidemiologic factors, mode of onset, initial clinical course, and specific organ involvement. From a pathobiological standpoint, sarcoidosis represents an immune paradox, where an excessive spread of both the innate and the adaptive immune arms of the immune system is accompanied by a state of partial immune anergy. For all these reasons, the optimal treatment for sarcoidosis remains unclear, with corticosteroid therapy being the current gold standard for those patients with significantly symptomatic or progressive pulmonary disease or serious extrapulmonary disease. This review is a state of the art of clinical presentations and immunological features of sarcoidosis, and the current therapeutic approaches used to treat the disease. Full article

(This article belongs to the Special Issue New Frontiers in the Diagnosis of Sarcoidosis)

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