Diagnostics

Editorial

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2 pages, 149 KiB

Open AccessEditorial

Sarcoidosis: An Old but Always Challenging Disease

by Claudio Tana

Diagnostics 2021, 11(4), 696; https://doi.org/10.3390/diagnostics11040696 - 14 Apr 2021

Cited by 5 | Viewed by 1405

Abstract

Sarcoidosis is a granulomatous disease which can involve every organ, and can manifest with nonspecific clinical pictures that can be misdiagnosed with those observed with other disorders [...] Full article

(This article belongs to the Special Issue Diagnosis and Management of Sarcoidosis)

Research

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10 pages, 1022 KiB

Open AccessArticle

Efficacy and Tolerability of Methotrexate and Methylprednisolone in a Comparative Assessment of the Primary and Long-Term Outcomes in Patients with Pulmonary Sarcoidosis

by Volodymyr Gavrysyuk, Ievgenia Merenkova, Yaroslav Dziublyk, Nataliia Morska, Nataliia Pendalchuk, Olesia Bychenko and Nataliia Vlasova

Diagnostics 2021, 11(7), 1289; https://doi.org/10.3390/diagnostics11071289 - 19 Jul 2021

Cited by 11 | Viewed by 2061

Abstract

Background: There is insufficient information in the literature on the comparative efficacy and tolerability of methotrexate (MTX) and methylprednisolone (MP) in patients with pulmonary sarcoidosis in assessing primary outcomes and the relapse rate. Purpose: The aim of our study was to evaluate primary [...] Read more.

Background: There is insufficient information in the literature on the comparative efficacy and tolerability of methotrexate (MTX) and methylprednisolone (MP) in patients with pulmonary sarcoidosis in assessing primary outcomes and the relapse rate. Purpose: The aim of our study was to evaluate primary and long-term outcomes of using MTX and MP in patients with pulmonary sarcoidosis. Methods: A total of 143 patients with newly diagnosed pulmonary sarcoidosis, verified by high-resolution computed tomography (CT) data, were examined. Corticosteroid (CS) therapy was used in 97 patients using MP at a dose of 0.4 mg/kg body weight for 4 weeks, followed by a dose reduction to 0.1 mg/kg by the end of the sixth month. The total duration of CS therapy was 12 months on average. Forty-six patients were treated with MTX at a dose of 10 mg/week (28) and 15 mg/week (18) per os for 6 to 12 months. The study of the relapse rate was conducted within 12 months after the CT data normalization in 60 patients after CS therapy and in 24 after MTX treatment. Results: MP treatment was successfully completed in 68 (70.1%), and MTX in 29 (60.4%) patients. In five MP patients (5.2%) and in five (10.9%) MTX, treatment was discontinued due to serious side effects. In seven (7.2%) MP patients and ten (21.7%) MTX patients, treatment required additional therapy due to the lack of efficacy. Progression with MP treatment (17–17.5%) was more common than with MTX (2–4.3%; Chi square = 4.703, p = 0.031). Relapses after MP therapy were observed in 26 (43.3%) patients, and after MTX therapy in 2 (8.3%; Chi square = 9.450, p = 0.003). Conclusion: In patients with pulmonary sarcoidosis, MTX monotherapy does not differ significantly from MP monotherapy in terms of the level of efficacy and the rate of serious side effects. Increasing the MTX dose from 10 to 15 mg/week accelerates the rate of regression of sarcoidosis, improves treatment efficacy, and does not affect the rate of serious side effects. When using MTX, there is a significant decrease in the incidence of treatment resistance and the relapse rate. Full article

(This article belongs to the Special Issue Diagnosis and Management of Sarcoidosis)

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36 pages, 2836 KiB

Open AccessArticle

Challenging Mimickers in the Diagnosis of Sarcoidosis: A Case Study

by Thomas El Jammal, Yvan Jamilloux, Mathieu Gerfaud-Valentin, Gaëlle Richard-Colmant, Emmanuelle Weber, Arthur Bert, Géraldine Androdias and Pascal Sève

Diagnostics 2021, 11(7), 1240; https://doi.org/10.3390/diagnostics11071240 - 12 Jul 2021

Cited by 8 | Viewed by 5846

Abstract

Sarcoidosis is a systemic granulomatous disease of unknown cause characterized by a wide variety of presentations. Its diagnosis is based on three major criteria: a clinical presentation compatible with sarcoidosis, the presence of non-necrotizing granulomatous inflammation in one or more tissue samples, and [...] Read more.

Sarcoidosis is a systemic granulomatous disease of unknown cause characterized by a wide variety of presentations. Its diagnosis is based on three major criteria: a clinical presentation compatible with sarcoidosis, the presence of non-necrotizing granulomatous inflammation in one or more tissue samples, and the exclusion of alternative causes of granulomatous disease. Many conditions may mimic a sarcoid-like granulomatous reaction. These conditions include infections, neoplasms, immunodeficiencies, and drug-induced diseases. Moreover, patients with sarcoidosis are at risk of developing opportunistic infections or lymphoma. Reliably confirming the diagnosis of sarcoidosis and better identifying new events are major clinical problems in daily practice. To address such issues, we present seven emblematic cases, seen in our department, over a ten-year period along with a literature review about case reports of conditions misdiagnosed as sarcoidosis. Full article

(This article belongs to the Special Issue Diagnosis and Management of Sarcoidosis)

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9 pages, 3662 KiB

Open AccessArticle

The Comparative Value of Serum Angiotensin Converting Enzyme (ACE) and Lysozyme and the Use of Polyclonal Antibody Activation in the Work-up of Ocular Sarcoidosis

by Ioannis Papasavvas, Béatrice Gehrig and Carl P. Herbort, Jr.

Diagnostics 2021, 11(4), 608; https://doi.org/10.3390/diagnostics11040608 - 29 Mar 2021

Cited by 9 | Viewed by 2086

Abstract

Background: The diagnosis of ocular sarcoidosis (OS) is difficult to establish in the absence of manifest systemic involvement. To help clinicians reach a diagnosis, we convened a group of experts in 2006 (International Workshop on Ocular Sarcoidosis (IWOS)) to set-up clinical criteria for [...] Read more.

Background: The diagnosis of ocular sarcoidosis (OS) is difficult to establish in the absence of manifest systemic involvement. To help clinicians reach a diagnosis, we convened a group of experts in 2006 (International Workshop on Ocular Sarcoidosis (IWOS)) to set-up clinical criteria for the diagnosis of ocular sarcoidosis. In addition, laboratory investigational tests represent a much-needed adjunct to ascertain the diagnosis. However, many of these tests have low sensitivity and specificity. Purpose: The aim of our study was to evaluate the usefulness of serum ACE, serum lysozyme and polyclonal antibody activation in the diagnosis of ocular sarcoidosis and compare the frequency of increased serum levels of lysozyme and ACE in proven ocular sarcoidosis or in suspected ocular sarcoidosis. Methods: Serum ACE and lysozyme were assessed in these two groups and their means compared to a group of non-granulomatous (i.e., non-sarcoidosis) uveitis patients. The proportion of elevated serum ACE versus lysozyme was compared in the sarcoidosis patients. Polyclonal antibody activation was measured by establishing exposition of patients to four human commensal herpesviruses (EBV, CMV, HSV and VZV) using ELISA or immunofluorescence and in parallel by performing quantitative complement fixation (CF) serologies. The ratio of elevated CF to positive ELISA/immunofluorescence serologies was calculated. The mean of ratios (polyclonal antibody activation) was compared between ocular sarcoidosis and control groups. Results: Thirty-seven patients (F24/M13) were included in our study including 17 patients with IWOS Level 1 and 2 criteria qualifying for Group 1 (proven sarcoidosis) and 20 ocular sarcoidosis suspect patients. Mean age was 54.52 ± 23.74. Mean serum levels of ACE was 49.17± 29 IU/L in the ocular sarcoidosis group versus 27.4 ± 15.34 IU/L (p ≤ 0.00018, student’s t test) in the control group. Mean serum lysozyme levels was 39.92 ± 55.5 mg/L in the ocular sarcoidosis group versus 10.5 ± 5.8 mg/L (p ≤ 0.0013) in the control group (n = 30). Both tests were elevated in 8/37 (21.6%) patients, elevated ACE and normal lysozyme was noted in 2/37 (5.4%) patients, whereas the proportion of normal ACE/elevated lysozyme was much higher, 23/37 (62.2%). In 4/37 (10.8%) patients, both tests were normal. The mean score of polyclonal activation (N of elevated CF serologies divided by number of viruses to which a patient was exposed) was 0.6 ± 0.33 in the ocular sarcoidosis group versus 0.15 ± 0.2 for the control group (n = 42) (p ≤ 0.00001). Sensitivity and specificity of ACE and lysozyme were, respectively, 27%/96.6% and 83.7%/90%. Sensitivity and specificity of polyclonal antibody activation amounted to 70%/90.4% Conclusion: Lysozyme was found to be much more useful than ACE as a laboratory test to support the diagnosis of ocular sarcoidosis. As shown in a previous study, polyclonal antibody activation appears to be another useful laboratory test supportive of the diagnosis of ocular sarcoidosis. Full article

(This article belongs to the Special Issue Diagnosis and Management of Sarcoidosis)

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Review

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14 pages, 1414 KiB

Open AccessReview

Pulmonary Sarcoidosis: Diagnosis and Differential Diagnosis

by Nicol Bernardinello, Simone Petrarulo, Elisabetta Balestro, Elisabetta Cocconcelli, Marcel Veltkamp and Paolo Spagnolo

Diagnostics 2021, 11(9), 1558; https://doi.org/10.3390/diagnostics11091558 - 28 Aug 2021

Cited by 18 | Viewed by 7821

Abstract

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 [...] Read more.

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. Full article

(This article belongs to the Special Issue Diagnosis and Management of Sarcoidosis)

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35 pages, 1270 KiB

Open AccessReview

by Lesley Ann Saketkoo, Anne-Marie Russell, Kelly Jensen, Jessica Mandizha, Jinny Tavee, Jacqui Newton, Frank Rivera, Mike Howie, Rodney Reese, Melanie Goodman, Patricia Hart, Bert Strookappe, Jolanda De Vries, Misha Rosenbach, Mary Beth Scholand, Mathew R. Lammi, Marjon Elfferich, Elyse Lower, Robert P. Baughman, Nadera Sweiss, Marc A. Judson and Marjolein Drent Show full author list Hide full author list

Diagnostics 2021, 11(6), 1089; https://doi.org/10.3390/diagnostics11061089 - 15 Jun 2021

Cited by 31 | Viewed by 9657

Abstract

Health-related quality of life (HRQoL), though rarely considered as a primary endpoint in clinical trials, may be the single outcome reflective of patient priorities when living with a health condition. HRQoL is a multi-dimensional concept that reflects the degree to which a health [...] Read more.

Health-related quality of life (HRQoL), though rarely considered as a primary endpoint in clinical trials, may be the single outcome reflective of patient priorities when living with a health condition. HRQoL is a multi-dimensional concept that reflects the degree to which a health condition interferes with participation in and fulfillment of important life areas. HRQoL is intended to capture the composite degree of physical, physiologic, psychological, and social impairment resulting from symptom burden, patient-perceived disease severity, and treatment side effects. Diminished HRQoL expectedly correlates to worsening disability and death; but interventions addressing HRQoL are linked to increased survival. Sarcoidosis, being a multi-organ system disease, is associated with a diffuse array of manifestations resulting in multiple symptoms, complications, and medication-related side effects that are linked to reduced HRQoL. Diminished HRQoL in sarcoidosis is related to decreased physical function, pain, significant loss of income, absence from work, and strain on personal relationships. Symptom distress can result clearly from a sarcoidosis manifestation (e.g., ocular pain, breathlessness, cough) but may also be non-specific, such as pain or fatigue. More complex, a single non-specific symptom, e.g., fatigue may be directly sarcoidosis-derived (e.g., inflammatory state, neurologic, hormonal, cardiopulmonary), medication-related (e.g., anemia, sleeplessness, weight gain, sub-clinical infection), or an indirect complication (e.g., sleep apnea, physical deconditioning, depression). Identifying and distinguishing underlying causes of impaired HRQoL provides opportunity for treatment strategies that can greatly impact a patient’s function, well-being, and disease outcomes. Herein, we present a reference manual that describes the current state of knowledge in sarcoidosis-related HRQoL and distinguish between diverse causes of symptom distress and other influences on sarcoidosis-related HRQoL. We provide tools to assess, investigate, and diagnose compromised HRQoL and its influencers. Strategies to address modifiable HRQoL factors through palliation of symptoms and methods to improve the sarcoidosis health profile are outlined; as well as a proposed research agenda in sarcoidosis-related HRQoL. Full article

(This article belongs to the Special Issue Diagnosis and Management of Sarcoidosis)

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13 pages, 4182 KiB

Open AccessReview

Chest Imaging of Patients with Sarcoidosis and SARS-CoV-2 Infection. Current Evidence and Clinical Perspectives

by Claudio Tana, Cesare Mantini, Francesco Cipollone and Maria Adele Giamberardino

Diagnostics 2021, 11(2), 183; https://doi.org/10.3390/diagnostics11020183 - 27 Jan 2021

Cited by 13 | Viewed by 5682

Abstract

The recent COVID-19 pandemic has dramatically changed the world in the last months, leading to a serious global emergency related to a novel coronavirus infection that affects both sexes of all ages ubiquitously. Advanced age, cardiovascular comorbidity, and viral load have been hypothesized [...] Read more.

The recent COVID-19 pandemic has dramatically changed the world in the last months, leading to a serious global emergency related to a novel coronavirus infection that affects both sexes of all ages ubiquitously. Advanced age, cardiovascular comorbidity, and viral load have been hypothesized as some of the risk factors for severity, but their role in patients affected with other diseases, in particular immune disorders, such as sarcoidosis, and the specific interaction between these two diseases remains unclear. The two conditions might share similar imaging findings but have distinctive features that are here described. The recent development of complex imaging softwares, called deep learning techniques, opens new scenarios for the diagnosis and management. Full article

(This article belongs to the Special Issue Diagnosis and Management of Sarcoidosis)

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Other

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4 pages, 193 KiB

Open AccessCommentary

Comparative Effectiveness of Methotrexate versus Methylprednisolone in Treatment Naïve Pulmonary Sarcoidosis Patients

by Jotam G. Pasipanodya

Diagnostics 2021, 11(8), 1401; https://doi.org/10.3390/diagnostics11081401 - 03 Aug 2021

Viewed by 1422

Abstract

Among those who study granulomatous diseases, sarcoidosis is of tremendous interest, not only because its cause is unknown, but also because it is still as much an enigma today as it was 150 years ago when Jonathan Hutchinson first described the cutaneous form [...] Read more.

Among those who study granulomatous diseases, sarcoidosis is of tremendous interest, not only because its cause is unknown, but also because it is still as much an enigma today as it was 150 years ago when Jonathan Hutchinson first described the cutaneous form of the disease as “livid papillary psoriasis”. This piece editorializes a comparative effectiveness study of methotrexate versus methylprednisolone in treatment naïve pulmonary sarcoidosis patients for CT-guided clinical responses and drug-related adverse events. Full article

(This article belongs to the Special Issue Diagnosis and Management of Sarcoidosis)

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