Sarcoidosis

Chest Imaging ◽  
2019 ◽  
pp. 441-446
Author(s):  
Juliana Bueno
Keyword(s):  
Chest Radiography ◽  
Asymptomatic Patient ◽  
Systemic Disease ◽  
Imaging Study ◽  
Epithelioid Cell ◽  
Unknown Etiology ◽  
Imaging Findings ◽  
Clinical Context ◽  
Pathological Confirmation ◽  
Necrotizing Granulomas

Sarcoidosis is a systemic disease of unknown etiology, characterized by noncaseating epithelioid cell granulomas. Patients with sarcoidosis are frequently asymptomatic and may be diagnosed incidentally because of an abnormal imaging study. Sarcoidosis is a diagnosis of exclusion that warrants pathological confirmation in all cases. The distribution of granulomas in the lungs is characteristic and correlates with imaging findings; non-necrotizing granulomas classically exhibit a perilymphatic distribution. On chest radiography, the presence of bilateral hilar and right paratracheal lymphadenopathy, particularly in a young asymptomatic patient, should raise the possibility of sarcoidosis. On CT, a perilymphatic distribution (peribronchovascular, subpleural, septal) of abnormalities in the appropriate clinical context is highly specific for sarcoidosis.

Sclerosing Angiomatoid Nodular Transformation of the Spleen

2013 ◽  
Vol 137 (9) ◽  
pp. 1309-1312 ◽  
Author(s):  
Dinesh Pradhan ◽  
Sambit K. Mohanty
Keyword(s):  
Current Knowledge ◽  
Benign Lesion ◽  
Good Prognosis ◽  
Unknown Etiology ◽  
Age Group ◽  
Imaging Findings ◽  
Effective Technique ◽  
Sclerosing Angiomatoid Nodular Transformation ◽  
Malignant Lesions ◽  
Mitotic Figures

Sclerosing Angiomatoid Nodular Transformation (SANT) of the spleen is a rare benign lesion of the spleen with unknown etiology. SANT is classically considered to be a female-predominant disease, with most of the patients in the 30- to 60-year age group. Most lesions are found incidentally on imaging. Although SANT has specific imaging findings, the differential diagnosis from other splenic tumors or malignant lesions is very difficult. Histopathologically, these tumors reveal multiple confluent angiomatoid nodules; these nodules are surrounded by concentric collagen fibers exhibiting an inflammatory and myofibroblastic response and are accompanied by numerous erythrocytes and siderophages. The nodules are populated by endothelial cells, phenotypically recapitulating normal splenic vasculature, such as sinusoids, capillaries, and small veins. Nuclear atypia is minimal, mitotic figures are extremely rare, and necrosis is consistently absent. This lesion has a unique immunohistochemical profile characterized by CD34−CD31+CD8+ sinusoids, CD34+CD31+CD8− capillaries, and CD34−CD31+CD8− small veins. CD68 is positive in macrophages. Splenectomy is a useful and effective technique for the management of SANT. SANT patients have a good prognosis, with no recurrence after splenectomy. In this review, we discuss the current knowledge of SANT of the spleen and its clinical relevance.

Potential use of biomarkers for the clinical evaluation of sarcoidosis

2021 ◽  
pp. jim-2020-001659
Author(s):  
Amir Mousapasandi ◽  
Cristan Herbert ◽  
Paul Thomas
Keyword(s):  
Clinical Evaluation ◽  
Clinical Presentation ◽  
Current Knowledge ◽  
Epithelioid Cell ◽  
Pulmonary Sarcoidosis ◽  
Biological Molecules ◽  
Unknown Etiology ◽  
Chronic Antigenic Stimulation ◽  
Host Genetic ◽  
Potential Use

Sarcoidosis is a systemic granulomatous disease of unknown etiology and pathogenesis with a heterogeneous clinical presentation. In the appropriate clinical and radiological context and with the exclusion of other diagnoses, the disease is characterized by the pathological presence of non-caseating epithelioid cell granulomas. Sarcoidosis is postulated to be a multifactorial disease caused by chronic antigenic stimulation. The immunopathogenesis of sarcoidosis encompasses a complex interaction between the host, genetic factors and postulated environmental and infectious triggers, which result in granuloma development.The exact pathogenesis of the disease has yet to be elucidated, but some of the inflammatory pathways that play a key role in disease progression and outcomes are becoming apparent, and these may form the logical basis for selecting potential biomarkers.Biomarkers are biological molecules that are altered pathologically. To date, there exists no single reliable biomarker for the evaluation of sarcoidosis, either diagnostically or prognostically but new candidates are emerging. A diagnosis of sarcoidosis ideally requires a biopsy confirming non-caseating granulomas, but the likelihood of progression that requires intervention remains unpredictable. These challenging aspects could be potentially resolved by incorporating biomarkers into clinical practice for both diagnosis and monitoring disease activity.This review outlines the current knowledge on sarcoidosis with an emphasis on pulmonary sarcoidosis, and delineates the understanding surrounding the implication of biomarkers for the clinical evaluation of sarcoidosis.

scholarly journals CD39+ EXPRESSION BY REGULATORY T CELLS IN PULMONARY SARCOIDOSIS AND LOFGREN’S SYNDROME

2019 ◽  
Vol 21 (3) ◽  
pp. 467-478
Author(s):  
I. V. Kudryavtsev ◽  
N. M. Lazareva ◽  
O. P. Baranova ◽  
A. S. Golovkin ◽  
D. V. Isakov ◽  
...  
Keyword(s):  
T Cells ◽  
Regulatory T Cells ◽  
Peripheral Blood ◽  
Relative Number ◽  
Epithelioid Cell ◽  
Pulmonary Sarcoidosis ◽  
Peripheral Blood Cell ◽  
Unknown Etiology ◽  
Löfgren’S Syndrome ◽  
Acute Form

Sarcoidosis is a disorder of unknown etiology characterized by development of necrosis-free epithelioid cell granulomas in various tissues. There are two main phenotypes of pulmonary sarcoidosis (PS): Lofgren’s syndrome (LS) is an acute form with favorable outcome, while non-Lofgren’s syndrome (nLS) is a chronic type of disease that can lead to pulmonary fibrosis in 20% of cases.Our study was aimed at investigating changes in the main cell-surface differentiation antigens on peripheral blood regulatory T cells (Tregs) from the patients with first diagnosed PS without treatment (LS, n = 11) and nLS (n = 46) compared to healthy volunteers (HC, n = 26).These indexes might be used as immunological markers for predicting severity of this disorder. Flow cytometry analysis of peripheral blood cell samples demonstrated that the nLS patients had decreased relative numbers of CD3+ cells vs healthy controls, as well as diminished CD3+CD4+ cells vs HC and LS patients. Furthermore, the relative and absolute Treg numbers were also decreased in nLS group vs HC (2.83% (2.47; 3.36) vs 3.33% (2.79; 3.84), p = 0.021), and 37 (29; 52) cells vs 50 (42; 65), p = 0.004, respectively) per one microliter of peripheral blood. Relative number of CD39-positive Тregs in chronic vs acute sarcoidosis patients was associated with 51.02% (38.20; 61.62) vs 48.64% (41.46; 63.72) that was significantly (p < 0.001 and p = 0.007, respectively) higher than in HC (39.52% (11.55; 46.34). We have found that “naïve” (CD45R0-CD62L+) Тregs did not significantly differ in percentage of CD39- and CD73-positive cells in all the groups tested. Moreover, CD45R0+CD62L+ Тregs in LS and nLS patients contained significantly more CD39-positive cells (69.66% (61.92; 79.34) and 67.62% (61.92; 79.34), respectively, compared to 47.55% (15.74; 65.32) in HC (p < 0.001 and p = 0.004, respectively). In case of CD45R0 + CD62LTregs able to exit from the circulation and migrate to the site of inflammation, an increased percentage of CD39-positive subset was noted only in patients with chronic sarcoidosis and HC (61.79% (55.12; 73.09) and 57.27% (16.03; 66.98), p = 0.006). Enhanced CD39 expression on Tregs seems to be related to chronic immune response, so that antigen elimination becomes impossible due to Treg overactivation, as shown in patients with sarcoidosis and some other chronic autoimmune and infectious disorders.

scholarly journals Evaluation of nasal mucociliary clearance in rheumatoid arthritis: a comparative analysis using saccharin test

2017 ◽  
Vol 5 (11) ◽  
pp. 5026 ◽  
Author(s):  
Shelja Deswal ◽  
Jyoti Yadav ◽  
Mohit Deswal ◽  
Harpreet Singh
Keyword(s):  
Rheumatoid Arthritis ◽  
Disease Duration ◽  
Mucociliary Clearance ◽  
Systemic Disease ◽  
Unknown Etiology ◽  
Control Group ◽  
Ciliary Activity ◽  
Multisystem Disease ◽  
Organic Particles ◽  
Nasal Mucociliary Clearance

Background: Rheumatoid arthritis (RA) is a chronic multisystem disease of unknown etiology characterized by persistent inflammatory synovitis, usually involving peripheral joints in a symmetric distribution. RA is a systemic disease often associated with cutaneous and organ-specific extra-articular manifestations the mucociliary clearance system protects the lower respiratory system by trapping and removing inhaled pathogenic viruses and bacteria, in addition to nontoxic and toxic particulates (e.g., pollen, ash, mineral dust, mold spores, and organic particles), from the lungs. Effective clearance requires both ciliary activity and the appropriate balance of periciliary fluid and mucus.Methods: This was a case control study conducted in the Department of Physiology, Pt. B.D. Sharma PGIMS, Rohtak in 50 females of age group 30-50 years. Control group comprised of 25 healthy volunteer females while study group comprised of 25 rheumatoid arthritis female patients with disease duration of more than five years. Proven cases of RA (as per 1987 ACR criteria) were taken with disease duration of more than five years from Rheumatology clinic of Pt. B.D. Sharma PGIMS, Rohtak. Nasal mucociliary clearance time was evaluated by saccharin method.Results: The results of our study showed abnormal mucociliary clearance in rheumatoid arthritis patients.Conclusions: The study shows an abnormal mucociliary clearance in rheumatoid arthritis patients. Impairment of mucociliary clearance seems to be the result of qualitative and quantitative alterations in respiratory secretions.

scholarly journals Features of cytokine profile in patients with sarcoidosis

2020 ◽  
Vol 22 (5) ◽  
pp. 993-1002
Author(s):  
N. M. Lazareva ◽  
O. P. Baranova ◽  
I. V. Kudryavtsev ◽  
N. A. Arsentieva ◽  
N. E. Liubimova ◽  
...  
Keyword(s):  
Blood Plasma ◽  
Healthy Volunteers ◽  
Peripheral Blood ◽  
Granulomatous Inflammation ◽  
Epithelioid Cell ◽  
Cytokine Profile ◽  
Unknown Etiology ◽  
Gm Csf ◽  
Peripheral Blood Plasma ◽  
Anti Inflammatory Cytokine

Sarcoidosis is an inflammatory disease of unknown etiology with damage to the lungs and other organs characterized by development of necrosis-free epithelioid cell granulomas. Granulomatous inflammation characterized by the activation of different immune systems cells, in particular T lymphocytes, and the cytokines production. Our study was aimed at investigating the characteristics of the cytokine profile of blood plasma in patients with sarcoidosis. We studied peripheral blood plasma samples of patients with sarcoidosis (n = 52). The control blood samples were taken from healthy volunteers (n = 22). The level of 46 cytokines (pg/ml) was determined, as follows: IL-1α, IL-1β, IL-2, IL-3, IL-4, IL-5, IL- 6, IL-7, IL-9, IL-12 (p40), IL-12 (p70), IL-13, IL-15, IL-17A, IFNα2, IFNγ, TNFα, TNFβ, IL- 1ra, IL-10, EGF, FGF-2, Flt3 Ligand, G-CSF, GM-CSF, PDGF-AA, PDGF-AB / BB, TGFα, VEGF-A, sCD40L, CCL2, CCL3, CCL4, CCL5, CCL7, CCL11, CCL17, CCL20, CCL22, CXCL1, CXCL8, CXCL9, CXCL10, CXCL11, CXCL13, CX3CL1. Significantly higher levels of interleukins and some proinflammatory cytokines were found in the patients with sarcoidosis, i.e., IL-3, 0.70 vs 0.20, p = 0.003; IL-4, 14.37 vs 3.15, p = 0.009; IL-5, 1.06 vs 0.89, p < 0.001; IL-12 (p70), 1.27 vs 0.56, p = 0.028; IL-17A, 1.48 vs 0.43, p < 0.001; IFNα2, 41.79 vs 25.04, p = 0.003; IFNγ, 4.13 vs 1.14, p < 0.001; TNFα, 21.67 vs 6.70, p < 0.001; anti-inflammatory cytokine IL-10, 1.03 vs 0.45, p = 0.019; growth factors: FGF-2, 40.08 vs 30.58, p = 0.008, G-CSF, 24.18 vs 8.21, p = 0.006, and VEGF-A, 42.52 vs 26.76, p = 0.048; chemokines: CCL3, 3.86 vs 1.33, p < 0,001; CCL17, 78.24 vs 26.24, p < 0.001; CCL20, 7.19 vs 5.64, p = 0.021; CCL22, 660.60 vs 405.00, p < 0,001; CXCL9, 4013 vs 1142, p < 0,001; CXCL10, 565.90 vs 196.60, p < 0.001; CXCL11, 230.20 vs 121.10, p = 0.018; CX3CL1, 56.99 vs 5.16, p < 0.001. Peripheral blood chemokine CCL11 levels were significantly lower in patients compared to the group of healthy volunteers: 77.58 vs 124.70, p = 0.022. The features of the cytokine profile in patients with sarcoidosis may indicate their important role in the processes of formation and outcomes of granulomas. These issues require an additional detailed study, comparison with phenotypes, differential course and outcomes of the disease.

scholarly journals Rheumatoid arthritis activity scores in patients with and without fibromyalgia syndrome

2021 ◽  
Vol 41 (4) ◽  
pp. 246-252
Author(s):  
Yunus Durmaz ◽  
Ilker Ilhanli
Keyword(s):  
Rheumatoid Arthritis ◽  
Disease Activity ◽  
Fibromyalgia Syndrome ◽  
Conflicts Of Interest ◽  
Systemic Disease ◽  
Response To Treatment ◽  
Unknown Etiology ◽  
Tender Joint Count ◽  
Tender Joint ◽  
Cross Sectional

BACKGROUND: Fibromyalgia syndrome (FM) is a systemic disease of unknown etiology, which can cause widespread musculoskeletal pain. In patients with rheumatoid arthritis (RA), FM can cause an additional symptom burden, which can affect some variables on the RA disease activity score 28 (DAS28), a tool that evaluates 28 joints in RA patients. OBJECTIVE: Compare the results of four different versions of the DAS28 and the parameters used to determine disease activity scores in RA patients with and without FM, and determine whether there are treatment differences between RA patients with and without FM. DESIGN: Retrospective, cross-sectional. SETTING: Tertiary hospital. PATIENTS AND METHODS: We identified patients diagnosed with RA between 1 September 2016 and 1 February 2020 and identified patients with and without FM. MAIN OUTCOME MEASURES: Differences between variables in the DAS28 calculations (tender joint count [TJC], patient global assessment [PGA], and others), between patients with and without FM, and differences between patients with and without FM who were using or not using biological agents. SAMPLE SIZE: 381, including 322 females (84.5%). RESULTS: The frequency of FM in RA patients was 25.7% (89 females, 24.6%). In RA patients with FM, the TJC and PGA median values were significantly higher than in patients without FM ( P <.05). The use of corticosteroids and biological therapy in patients with FM was more frequent than in patients without FM ( P <.05). Compared to patients without FM, patients with FM switched treatment more often because of non-response to treatment ( P =.01) Median values of the DAS28 scores (calculated by four different versions of the instrument) in RA patients with FM were higher than in patients without FM ( P <.05). CONCLUSION: The presence of FM in RA patients may affect the subjective variables in different versions of DAS28 scores, causing the disease activity to score higher on the instrument, erroneously indicating worse disease than is actually present. LIMITATIONS: A single center, retrospective study. CONFLICTS OF INTEREST: None.

Cardiac Sarcoidosis: Cytokine Patterns in the Course of the Disease

2003 ◽  
Vol 127 (9) ◽  
pp. 1207-1210 ◽  
Author(s):  
Michael Schoppet ◽  
Sabine Pankuweit ◽  
Bernhard Maisch
Keyword(s):  
Time Course ◽  
Mononuclear Cells ◽  
Cardiac Sarcoidosis ◽  
Systemic Disease ◽  
Healing Process ◽  
Unknown Etiology ◽  
Enzyme Linked Immunosorbent Assay ◽  
Rapid Decline ◽  
Peripheral Blood Mononuclear ◽  
Multiple Organs

Abstract Sarcoidosis is a chronic systemic disease of unknown etiology, which is characterized by noncaseating epitheloid granulomas usually in multiple organs. Here we describe changes in cytokine mRNA expression by peripheral blood mononuclear cells (PBMCs) and changes of cytokine protein levels in plasma over a time course of 12 months in a patient with sarcoidosis confined to the heart as diagnosed by endomyocardial biopsy. Mitogen-stimulated PBMCs exhibited a more TH1 cytokine profile at onset of symptoms before immunosuppressive therapy was initiated, with a change to a TH0 response in the course of the disease as evidenced by multiplex-polymerase chain reaction. In plasma, high levels of interleukin-6 could be detected by an enzyme-linked immunosorbent assay system, with rapid decline correlating with immunosuppression and improving clinical course. These changes may point to a role of TH1 and TH2 cytokines in the pathogenesis and the healing process of cardiac sarcoidosis.

Anemias

2019 ◽  
pp. 21-27
Author(s):  
Kevin B. Hoover
Keyword(s):  
Iron Deficiency ◽  
Sickle Cell ◽  
Blood Cells ◽  
Imaging Study ◽  
Deficiency Anemia ◽  
Imaging Findings ◽  
Common Causes ◽  
Characteristic Imaging ◽  
The Common ◽  
Initial Imaging

Chapter 74 discusses anemia conditions including sickle cell disease and thalassemia. Anemia is a condition of decreased oxygen delivery to organs caused by a deficit of functional red blood cells. It is a very common clinical condition and has a wide variety of causes. Of the common causes, the hemoglobinopathies have the most characteristic imaging findings. There are common imaging findings secondary to the compensatory increase in red blood cell and precursor number. Radiographs are a standard initial imaging study. MRI is the most sensitive imaging study for the sequelae of anemia. Iron deficiency anemia is treated with iron supplementation, aplastic anemia is treated by removal of the inciting agent, and painful crises of sickle cell are treated symptomatically.

Imaging Modalities

Chest Imaging ◽  
2019 ◽  
pp. 7-11
Author(s):  
Melissa L. Rosado-de-Christenson
Keyword(s):  
Chest Radiography ◽  
Pulmonary Thromboembolism ◽  
Imaging Techniques ◽  
Imaging Study ◽  
Chest Ct ◽  
Imaging Modalities ◽  
Vascular Abnormalities ◽  
Contrast Enhanced ◽  
Lateral Chest ◽  
Fdg Pet Ct

The chapter titled imaging modalities describes various methods of imaging the thorax. Imaging of patients presenting with thoracic complaints typically begins with chest radiography. Ambulatory patients should undergo posteroanterior (PA) and lateral chest radiographs. Anteroposterior (AP) chest radiography should be reserved for debilitated, critically ill and traumatized patients. Special chest radiographic projections such as decubitus chest radiography may be employed for specific indications. Chest CT is the imaging study of choice for evaluating most abnormalities found on radiography. Contrast-enhanced chest CT is optimal for evaluation of vascular abnormalities, the hila and some mediastinal lesions. CT angiography is routinely employed in patients with suspected pulmonary thromboembolism or acute aortic syndromes. High-resolution chest CT is reserved for the evaluation of diffuse infiltrative lung disease and often includes expiratory and prone imaging. FDG PET/CT is increasingly employed in the assessment of patients with malignancy for the purposes of initial staging and post therapy re-staging of affected patients. Ventilation/perfusion scintigraphy is used in the assessment of pulmonary thromboembolism. Additional thoracic imaging techniques include: Fluoroscopy for evaluation of the diaphragm, and ultrasound for evaluation of the thyroid and the pleural space.

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

2020 ◽  
Vol 9 (8) ◽  
pp. 2363
Author(s):  
Francesca Polverino ◽  
Elisabetta Balestro ◽  
Paolo Spagnolo
Keyword(s):  
Clinical Course ◽  
State Of The Art ◽  
Systemic Disease ◽  
Unknown Etiology ◽  
Therapeutic Approaches ◽  
Epidemiologic Factors ◽  
Adaptive Immune ◽  
Clinical Presentations ◽  
Specific Organ ◽  
Noncaseating Granulomas

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.

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