Journal of Clinical Medicine

Editorial

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3 pages, 195 KiB

Open AccessEditorial

Deconstructing Multiorgan Sarcoidosis

by Marc A. Judson

J. Clin. Med. 2023, 12(6), 2290; https://doi.org/10.3390/jcm12062290 - 15 Mar 2023

Viewed by 1109

Abstract

Sarcoidosis is a multisystem granulomatous disease of unknown cause [...] Full article

(This article belongs to the Special Issue Advances in the Diagnosis and Treatment of Pulmonary Sarcoidosis)

Research

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15 pages, 844 KiB

Open AccessArticle

Establishing a Diagnosis of Pulmonary Sarcoidosis

by Jan C. Grutters

J. Clin. Med. 2023, 12(21), 6898; https://doi.org/10.3390/jcm12216898 - 2 Nov 2023

Cited by 6 | Viewed by 2108

Abstract

Pulmonary sarcoidosis is the most prevalent manifestation of sarcoidosis and the commonest diagnosis in clinics for ILD. Due to the lack of a simple and reliable test, making the diagnosis is often challenging. There are three criteria that must always be considered: (1) [...] Read more.

Pulmonary sarcoidosis is the most prevalent manifestation of sarcoidosis and the commonest diagnosis in clinics for ILD. Due to the lack of a simple and reliable test, making the diagnosis is often challenging. There are three criteria that must always be considered: (1) compatible clinical presentation; (2) evidence of granuloma formation (usually non-caseating); and (3) exclusion of alternative causes of granulomatous disease. There are various tools available for diagnosis, amongst which serum biomarkers like sACE and sIL-2R, HRCT, BAL, EBUS/EUS and sometimes bronchoscopic or surgical lung biopsy are most contributive. However, the degree of invasiveness of the applied test and associated risk to the patient must be weighed against management consequences. In specific situations (e.g., presentation as Löfgren’s syndrome) or when there is high suspicion based on HRCT in the context of supportive clinical findings, it might be justifiable to decide on a “working diagnosis of sarcoidosis” and to refrain from further invasive procedures for the patient. This should, however, preferably be agreed upon after discussion in an experienced multidisciplinary team and requires close follow-up of the patient. In general, it is advisable to always maintain a healthy dose of skepticism when making the diagnosis of sarcoidosis, especially when the clinical course of disease gives rise to this. Full article

(This article belongs to the Special Issue Advances in the Diagnosis and Treatment of Pulmonary Sarcoidosis)

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Review

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20 pages, 1772 KiB

Open AccessReview

Sarcoidosis-Associated Pulmonary Hypertension

by Dominique Israël-Biet, Jean Pastré and Hilario Nunes

J. Clin. Med. 2024, 13(7), 2054; https://doi.org/10.3390/jcm13072054 - 2 Apr 2024

Viewed by 2454

Abstract

Sarcoidosis-associated pulmonary hypertension (SAPH) is a very severe complication of the disease, largely impacting its morbidity and being one of its strongest predictors of mortality. With the recent modifications of the hemodynamic definition of pulmonary hypertension (mean arterial pulmonary pressure >20 instead of [...] Read more.

Sarcoidosis-associated pulmonary hypertension (SAPH) is a very severe complication of the disease, largely impacting its morbidity and being one of its strongest predictors of mortality. With the recent modifications of the hemodynamic definition of pulmonary hypertension (mean arterial pulmonary pressure >20 instead of <25 mmHg,) its prevalence is presently not precisely known, but it affects from 3 to 20% of sarcoid patients; mostly, although not exclusively, those with an advanced, fibrotic pulmonary disease. Its gold-standard diagnostic tool remains right heart catheterization (RHC). The decision to perform it relies on an expert decision after a non-invasive work-up, in which echocardiography remains the screening tool of choice. The mechanisms underlying SAPH, very often entangled, are crucial to define, as appropriate and personalized therapeutic strategies will aim at targeting the most significant ones. There are no recommendations so far as to the indications and modalities of the medical treatment of SAPH, which is based upon the opinion of a multidisciplinary team of sarcoidosis, pulmonary hypertension and sometimes lung transplant experts. Full article

(This article belongs to the Special Issue Advances in the Diagnosis and Treatment of Pulmonary Sarcoidosis)

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32 pages, 598 KiB

Open AccessReview

Clinical Pharmacology in Sarcoidosis: How to Use and Monitor Sarcoidosis Medications

by Sooyeon Kwon and Marc A. Judson

J. Clin. Med. 2024, 13(5), 1250; https://doi.org/10.3390/jcm13051250 - 22 Feb 2024

Cited by 1 | Viewed by 2601

Abstract

When sarcoidosis needs treatment, pharmacotherapy is usually required. Although glucocorticoids work reliably and relatively quickly for sarcoidosis, these drugs are associated with numerous significant side effects. Such side effects are common in sarcoidosis patients, as the disease frequently has a chronic course and [...] Read more.

When sarcoidosis needs treatment, pharmacotherapy is usually required. Although glucocorticoids work reliably and relatively quickly for sarcoidosis, these drugs are associated with numerous significant side effects. Such side effects are common in sarcoidosis patients, as the disease frequently has a chronic course and glucocorticoid treatment courses are often prolonged. For these reasons, corticosteroid-sparing and corticosteroid-replacing therapies are often required for sarcoidosis. Unfortunately, many healthcare providers who care for sarcoidosis patients are not familiar with the use of these agents. In this manuscript, we provide a review of the pharmacotherapy of sarcoidosis. We discuss the mechanism of action, dosing, side-effect profile, approach to monitoring and patient counselling concerning glucocorticoids, and the common alternative drugs recommended for use in the recent European Respiratory Society (Lausanne, Switzerland) Sarcoidosis Treatment Guidelines. We also discuss the use of these agents in special situations including hepatic insufficiency, renal insufficiency, pregnancy, breastfeeding, vaccination, and drug–drug interactions. It is hoped that this manuscript will provide valuable practical guidance to clinicians who care for sarcoidosis patients. Full article

(This article belongs to the Special Issue Advances in the Diagnosis and Treatment of Pulmonary Sarcoidosis)

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19 pages, 8138 KiB

Open AccessEditor’s ChoiceReview

Imaging of Pulmonary Sarcoidosis—A Review

by Georgina L. Bailey, Athol U. Wells and Sujal R. Desai

J. Clin. Med. 2024, 13(3), 822; https://doi.org/10.3390/jcm13030822 - 31 Jan 2024

Cited by 6 | Viewed by 4324

Abstract

Sarcoidosis is the classic multisystem granulomatous disease. First reported as a disorder of the skin, it is now clear that, in the overwhelming majority of patients with sarcoidosis, the lungs will bear the brunt of the disease. This review explores some of the [...] Read more.

Sarcoidosis is the classic multisystem granulomatous disease. First reported as a disorder of the skin, it is now clear that, in the overwhelming majority of patients with sarcoidosis, the lungs will bear the brunt of the disease. This review explores some of the key concepts in the imaging of pulmonary sarcoidosis: the wide array of typical (and some of the less common) findings on high-resolution computed tomography (HRCT) are reviewed and, with this, the concept of morphologic/HRCT phenotypes is discussed. The pathophysiologic insights provided by HRCT through studies where morphologic abnormalities and pulmonary function tests are compared are evaluated. Finally, this review outlines the important contribution of HRCT to disease monitoring and prognostication. Full article

(This article belongs to the Special Issue Advances in the Diagnosis and Treatment of Pulmonary Sarcoidosis)

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15 pages, 817 KiB

Open AccessFeature PaperReview

Treatment of Granulomatous Inflammation in Pulmonary Sarcoidosis

by Alicia K. Gerke

J. Clin. Med. 2024, 13(3), 738; https://doi.org/10.3390/jcm13030738 - 27 Jan 2024

Viewed by 1683

Abstract

The management of pulmonary sarcoidosis is a complex interplay of disease characteristics, the impact of medications, and patient preferences. Foremost, it is important to weigh the risk of anti-granulomatous treatment with the benefits of lung preservation and improvement in quality of life. Because [...] Read more.

The management of pulmonary sarcoidosis is a complex interplay of disease characteristics, the impact of medications, and patient preferences. Foremost, it is important to weigh the risk of anti-granulomatous treatment with the benefits of lung preservation and improvement in quality of life. Because of its high spontaneous resolution rate, pulmonary sarcoidosis should only be treated in cases of significant symptoms due to granulomatous inflammation, lung function decline, or substantial inflammation on imaging that can lead to irreversible fibrosis. The longstanding basis of treatment has historically been corticosteroid therapy for the control of granulomatous inflammation. However, several corticosteroid-sparing options have increasing evidence for use in refractory disease, inability to taper steroids to an acceptable dose, or in those with toxicity to corticosteroids. Treatment of sarcoidosis should be individualized for each patient due to the heterogeneity of the clinical course, comorbid conditions, response to therapy, and tolerance of medication side effects. Full article

(This article belongs to the Special Issue Advances in the Diagnosis and Treatment of Pulmonary Sarcoidosis)

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20 pages, 352 KiB

Open AccessReview

Infectious Complications of Pulmonary Sarcoidosis

by Dominique Valeyre, Jean-François Bernaudin, Michel Brauner, Hilario Nunes and Florence Jeny

J. Clin. Med. 2024, 13(2), 342; https://doi.org/10.3390/jcm13020342 - 7 Jan 2024

Viewed by 1962

Abstract

In this review, the infectious complications observed in sarcoidosis are considered from a practical point of view to help the clinician not to overlook them in a difficult context, as pulmonary sarcoidosis makes the recognition of superinfections more difficult. An increased incidence of [...] Read more.

In this review, the infectious complications observed in sarcoidosis are considered from a practical point of view to help the clinician not to overlook them in a difficult context, as pulmonary sarcoidosis makes the recognition of superinfections more difficult. An increased incidence of community-acquired pneumonia and of opportunistic pneumonia has been reported, especially in immunosuppressed patients. Pulmonary destructive lesions of advanced sarcoidosis increase the incidence of chronic pulmonary aspergillosis and infection by other agents. Screening and treatment of latent tuberculosis infection are crucial to prevent severe tuberculosis. Severity in COVID-19 appears to be increased by comorbidities rather than by sarcoidosis per se. The diagnosis of infectious complications can be challenging and should be considered as a potential differential diagnosis when the exacerbation of sarcoidosis is suspected. These complications not only increase the need for hospitalizations, but also increase the risk of death. This aspect must be carefully considered when assessing the overall health burden associated with sarcoidosis. The impact of immune dysregulation on infectious risk is unclear except in exceptional cases. In the absence of evidence-based studies on immunosuppressants in the specific context of pulmonary sarcoidosis, it is recommended to apply guidelines used in areas outside sarcoidosis. Preventive measures are essential, beginning with an appropriate use of immunosuppressants and the avoidance of unjustified treatments and doses. This approach should take into account the risk of tuberculosis, especially in highly endemic countries. Additionally, parallel emphasis should be placed on vaccinations, especially against COVID-19. Full article

(This article belongs to the Special Issue Advances in the Diagnosis and Treatment of Pulmonary Sarcoidosis)

16 pages, 3740 KiB

Open AccessReview

Clinical Manifestations and Management of Fibrotic Pulmonary Sarcoidosis

by Jin Sun Kim and Rohit Gupta

J. Clin. Med. 2024, 13(1), 241; https://doi.org/10.3390/jcm13010241 - 31 Dec 2023

Cited by 1 | Viewed by 1891

Abstract

Fibrotic pulmonary sarcoidosis represents a distinct and relatively uncommon manifestation within the spectrum of sarcoidosis and has substantial morbidity and mortality. Due to the scarcity of research focused on this specific disease subtype, our current understanding of pathogenesis and optimal management remains constrained. [...] Read more.

Fibrotic pulmonary sarcoidosis represents a distinct and relatively uncommon manifestation within the spectrum of sarcoidosis and has substantial morbidity and mortality. Due to the scarcity of research focused on this specific disease subtype, our current understanding of pathogenesis and optimal management remains constrained. This knowledge gap underscores the need for further investigation into areas such as targeted therapies, lung transplantation, and quality of life of patients with fibrotic pulmonary sarcoidosis. The primary aim of this review is to discuss recent developments within the realm of fibrotic pulmonary sarcoidosis to foster a more comprehensive understanding of the underlying mechanisms, prognosis, and potential treatment modalities. Full article

(This article belongs to the Special Issue Advances in the Diagnosis and Treatment of Pulmonary Sarcoidosis)

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20 pages, 757 KiB

Open AccessReview

Emerging Therapeutic Options for Refractory Pulmonary Sarcoidosis: The Evidence and Proposed Mechanisms of Action

by Nathaniel C. Nelson, Rebecca Kogan, Rany Condos and Kerry M. Hena

J. Clin. Med. 2024, 13(1), 15; https://doi.org/10.3390/jcm13010015 - 19 Dec 2023

Viewed by 2272

Abstract

Sarcoidosis is a systemic disease with heterogenous clinical phenotypes characterized by non-necrotizing granuloma formation in affected organs. Most disease either remits spontaneously or responds to corticosteroids and second-line disease-modifying therapies. These medications are associated with numerous toxicities that can significantly impact patient quality-of-life [...] Read more.

Sarcoidosis is a systemic disease with heterogenous clinical phenotypes characterized by non-necrotizing granuloma formation in affected organs. Most disease either remits spontaneously or responds to corticosteroids and second-line disease-modifying therapies. These medications are associated with numerous toxicities that can significantly impact patient quality-of-life and often limit their long-term use. Additionally, a minority of patients experience chronic, progressive disease that proves refractory to standard treatments. To date, there are limited data to guide the selection of alternative third-line medications for these patients. This review will outline the pathobiological rationale behind current and emerging therapeutic agents for refractory or drug-intolerant sarcoidosis and summarize the existing clinical evidence in support of their use. Full article

(This article belongs to the Special Issue Advances in the Diagnosis and Treatment of Pulmonary Sarcoidosis)

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11 pages, 930 KiB

Open AccessReview

Pulmonary Function in Pulmonary Sarcoidosis

by Qian Yao, Qiuliang Ji and Ying Zhou

J. Clin. Med. 2023, 12(21), 6701; https://doi.org/10.3390/jcm12216701 - 24 Oct 2023

Cited by 2 | Viewed by 1554

Abstract

The pulmonary function test (PFT) has been widely used in sarcoidosis. It may vary due to the severity, extent, and the presence of complications of the disease. Although the PFT of most sarcoidosis patients is normal, there are still 10–30% of cases who [...] Read more.

The pulmonary function test (PFT) has been widely used in sarcoidosis. It may vary due to the severity, extent, and the presence of complications of the disease. Although the PFT of most sarcoidosis patients is normal, there are still 10–30% of cases who may experience a decrease in the PFT, with a progressive involvement of lungs. Restrictive ventilatory impairment due to parenchymal involvement has been commonly reported, and an obstructive pattern can also be present related to airway involvement. The PFT may influence treatment decisions. A diffusing capacity for carbon monoxide (DLCO) < 60% as well as a forced vital capacity (FVC) < 70% portends clinically significant pulmonary sarcoidosis pathology and warrants treatment. During follow-up, a 5% decline in FVC from baseline or a 10% decline in DLCO has been considered significant and reflects the disease progression. FVC has been recommended as the favored objective endpoint for monitoring the response to therapy, and an improvement in predicted FVC percentage of more than 5% is considered effective. Full article

(This article belongs to the Special Issue Advances in the Diagnosis and Treatment of Pulmonary Sarcoidosis)

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16 pages, 1810 KiB

Open AccessReview

The Symptoms of Pulmonary Sarcoidosis

by Marc A. Judson

J. Clin. Med. 2023, 12(18), 6088; https://doi.org/10.3390/jcm12186088 - 20 Sep 2023

Cited by 3 | Viewed by 3197

Abstract

The aim of this manuscript is to provide a comprehensive review of the etiology, measurement, and treatment of common pulmonary symptoms associated with sarcoidosis. The assessment of symptoms associated with pulmonary sarcoidosis is an important component of disease management. Some symptoms of pulmonary [...] Read more.

The aim of this manuscript is to provide a comprehensive review of the etiology, measurement, and treatment of common pulmonary symptoms associated with sarcoidosis. The assessment of symptoms associated with pulmonary sarcoidosis is an important component of disease management. Some symptoms of pulmonary sarcoidosis are sensitive but nonspecific markers of disease activity, and the absence of such symptoms provides evidence that the disease is quiescent. Although quantifiable objective measurements of pulmonary physiology and chest imaging are important in the assessment of pulmonary sarcoidosis, they correlate poorly with the patient’s quality of life. Because the symptoms of pulmonary sarcoidosis directly relate to how the patient feels, they are reasonable endpoints in terms of clinical research and individual patient care. Recently, the symptoms of pulmonary sarcoidosis are capable of being quantified via patient-reported outcome measures and electronic devices. We conclude that a thorough assessment of the symptoms associated with pulmonary sarcoidosis improves patient care because it is a useful screen for manifestations of the disease, provides insight into the pathophysiology of manifestations of sarcoidosis, and may assist in optimizing treatment. Full article

(This article belongs to the Special Issue Advances in the Diagnosis and Treatment of Pulmonary Sarcoidosis)

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