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"SARCOIDOSIS"
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Sarcoidosis
Sarcoidosis is a systemic disease of unknown cause that is characterised by the formation of immune granulomas in various organs, mainly the lungs and the lymphatic system. Studies show that sarcoidosis might be the result of an exaggerated granulomatous reaction after exposure to unidentified antigens in individuals who are genetically susceptible. Several new insights have been made, particularly with regards to the diagnosis and care of some important manifestations of sarcoidosis. The indications for endobronchial ultrasound in diagnosis and for PET in the assessment of inflammatory activity are now better specified. Recognition of unexplained persistent disabling symptoms, fatigue, small-fibre neurological impairment, cognitive failure, and changes to health state and quality of life, has improved. Mortality in patients with sarcoidosis is higher than that of the general population, mainly due to pulmonary fibrosis. Predicted advances for the future are finding the cause of sarcoidosis, and the elucidation of relevant biomarkers, reliable endpoints, and new efficient treatments, particularly in patients with refractory sarcoidosis, lung fibrosis, and those with persistent disabling symptoms.
Journal Article
S78 A distinct immune regulatory receptor profile linked to altered monocyte subsets in sarcoidosis
Introduction and ObjectivesIn sarcoidosis, blood monocytes display enhanced inflammatory responses, reduced expression of the regulatory receptor CD200R, and altered subsets defined by CD14 and CD16. We investigated the relationship between sarcoidosis monocyte subsets and expression of CD200R and other regulatory receptors.MethodsWe studied live monocytes from 25 treatment-naïve patients with pulmonary sarcoidosis and age-matched healthy controls.ResultsNon-classical monocytes were expanded in sarcoidosis and exhibited significantly lower expression of CD200R, SIRP-α, and CD47 than classical or intermediate monocytes. All sarcoidosis monocyte subsets had significantly reduced CD200R and CD47 expression compared with healthy controls.ConclusionsWe provide the first description of significantly reduced regulatory receptor expression on non-classical monocytes. Expansion of these non-classical monocytes in sarcoidosis, together with reduced levels of specific regulatory molecules (CD200R and CD47) on all sarcoidosis monocyte subsets compared with controls, will favour heightened inflammatory responses. This sarcoidosis monocyte phenotype could be assessed as a prognostic or therapeutic biomarker in future studies.
Journal Article
First-Line Treatment of Pulmonary Sarcoidosis with Prednisone or Methotrexate
In this randomized trial, first-line treatment of pulmonary sarcoidosis with methotrexate was noninferior to that with prednisone regarding the change at 24 weeks in the percentage of the predicted forced vital capacity.
Journal Article
Epidemiology of Sarcoidosis 1946-2013: A Population-Based Study
To characterize the epidemiology of sarcoidosis from 1946 through 2013.
An inception cohort of patients with incident sarcoidosis from January 1, 1976, through December 31, 2013, in Olmsted County, Minnesota, was identified based on comprehensive individual medical record review. Inclusion required physician diagnosis supported by histopathologic confirmation, radiologic features of intrathoracic sarcoidosis, and a compatible clinical presentation. Data were collected on demographic characteristics, clinical presentation, laboratory investigations, and mortality. The data were augmented with a previously identified cohort of Olmsted County residents diagnosed as having sarcoidosis in 1946-1975. Incidence rates were age and sex adjusted to the 2010 US white population.
A total of 448 incident cases of sarcoidosis were identified (mean age, 44.2 years; 52% women). The annual incidence of sarcoidosis was 10.0 per 100,000 population. The incidence of sarcoidosis increased in women from 1950 to 1960, but otherwise there were no significant calendar year trends. However, the peak age at incidence for women shifted from 40 to 59 years in 1950 to 50 to 69 years in 2010. Similarly, the peak age at incidence for men shifted from 30 to 49 years in 1950 to 40 to 59 years in 2010. Ninety-seven percent of patients had intrathoracic involvement, but only 43% had respiratory symptoms. The overall mortality of patients with sarcoidosis was not different from that of the general population (standardized mortality ratio=0.90; 95% CI, 0.74-1.08).
Sarcoidosis occurred in approximately 10 persons per 100,000 per year. Most of the patients had intrathoracic involvement, although less than half had respiratory symptoms. Overall mortality was not different from that of the general population.
Journal Article
Diagnostic Yield and Safety of the 19-Gauge versus 22-Gauge Endobronchial Ultrasound-Guided Transbronchial Needle Aspiration Needle in Subjects with Sarcoidosis (GUESS)
Abstract
Introduction: Observational data suggest that the 19-gauge (G) needle for endobronchial ultrasound (EBUS)-guided transbronchial needle aspiration (TBNA) offers a higher diagnostic yield than the 22-G needle in sarcoidosis. No randomized trial has compared the yield of the two needles. Methods: We randomized consecutive subjects with suspected sarcoidosis and enlarged thoracic lymph nodes to undergo EBUS-TBNA with either the 19-G or the 22-G needle. We compared the study groups for diagnostic sensitivity (primary outcome) assessed by the yield of granulomas in subjects finally diagnosed with sarcoidosis. We also compared the sample adequacy, difficulty performing the needle puncture assessed on a visual analog scale (VAS), the subject’s cough intensity on an operator-rated VAS, and procedure-related complications (secondary outcomes). Results: We randomized 150 (mean age, 43.0 years; 55% women) subjects and diagnosed sarcoidosis in 116 subjects. The diagnostic sensitivity of the 19-G needle (45/60, 75.0%) was not higher (p = 0.52) than the 22-G needle (39/56, 69.6%). We obtained adequate aspirates in 90.0% and 85.7% of subjects in the respective groups (p = 0.48). The operators had greater difficulty puncturing lymph nodes with the 19-G needle (p = 0.03), while the operator-assessed cough intensity was similar in the groups (p = 0.41). Transient hypoxemia was the only complication encountered during EBUS-TBNA (two subjects in either group). Conclusion: We did not find the 19-G needle superior to the 22-G in diagnostic sensitivity, specimen adequacy, or safety of EBUS-TBNA in sarcoidosis. Puncturing the lymph nodes was more difficult with the 19-G needle.
Journal Article
The diagnostic challenge of coexistent sarcoidosis and thyroid cancer – a retrospective study
Background
Sarcoid lesions may mimic metastatic disease or recurrence in thyroid cancer (TC) patients as both diseases may affect the lungs and lymph nodes. We present the first study to systematically evaluate the clinical course of patients with (TC) after adjuvant radioactive iodine therapy (RIT) and concomitant sarcoidosis of the lung or the lymph nodes.
Methods
We screened 3285 patients and retrospectively identified 16 patients with TC (11 papillary thyroid cancer (PTC), 3 follicular thyroid cancer (FTC), 1 oncocytic PTC, 1 oncocytic FTC) and coexisting sarcoidosis of the lung and/or the lymph nodes treated at our institute. All patients had undergone thyroidectomy and initial adjuvant RIT. Challenges in diagnosing and the management of these patients were evaluated during long term follow-up (median 4.9 years (0.8–15.0 years)).
Results
Median age at first diagnosis of TC was 50.1 years (33.0–71.5 years) and of sarcoidosis 39.4 years (18.0–63.9 years). During follow-up, physicians were able to differentiate between SA and persistent or recurrent TC in 10 of 16 patients (63%). Diagnosis was complicated by initial negative thyroglobulin (Tg), positive Tg antibodies and non-specific imaging findings. Histopathology can reliably distinguish between SA and TC in patients with one suspicious lesion.
Conclusion
Physicians should be aware of the rare coexistence of sarcoidosis and TC. Lymphadenopathy and pulmonary lesions could be metastases, sarcoidosis or even a mix of both. Therefore, this rare patient group should receive a thorough work up including histopathological clarification and, if necessary, separately for each lesion.
Journal Article
Hypoxia Promotes a Mixed Inflammatory-Fibrotic Macrophages Phenotype in Active Sarcoidosis
Macrophages are pivotal cells in sarcoidosis. Monocytes-derived (MD) macrophages have recently been demonstrated to play a major role especially in pulmonary sarcoidosis. From inflammatory tissues to granulomas, they may be exposed to low oxygen tension environments. As hypoxia impact on sarcoidosis immune cells has never been addressed, we designed the present study to investigate MD-macrophages from sarcoidosis patients in this context. We hypothesized that hypoxia may induce functional changes on MD-macrophages which could have a potential impact on the course of sarcoidosis.
We studied MD-macrophages, from high active sarcoidosis (AS) (n=26), low active or inactive sarcoidosis (IS) (n=24) and healthy controls (n=34) exposed 24 hours to normoxia (21% O
) or hypoxia (1.5% O
). Different macrophage functions were explored: hypoxia-inducible factor-1α (HIF-1α) and nuclear factor-kappa B (NF-κB) activation, cytokines secretion, phagocytosis, CD80/CD86/HLA-DR expression, profibrotic response.
We observed that hypoxia, with a significantly more pronounced effect in AS compared with controls and IS, increased the HIF-1α trans-activity, promoted a proinflammatory response (TNFα, IL1ß) without activating NF-κB pathway and a profibrotic response (TGFß1, PDGF-BB) with PAI-1 secretion associated with human lung fibroblast migration inhibition. These results were confirmed by immunodetection of HIF-1α and PAI-1 in granulomas observed in pulmonary biopsies from patients with sarcoidosis. Hypoxia also decreased the expression of CD80/CD86 and HLA-DR on MD-macrophages in the three groups while it did not impair phagocytosis and the expression of CD36 expression on cells in AS and IS at variance with controls.
Hypoxia had a significant impact on MD-macrophages from sarcoidosis patients, with the strongest effect seen in patients with high active disease. Therefore, hypoxia could play a significant role in sarcoidosis pathogenesis by increasing the macrophage proinflammatory response, maintaining phagocytosis and reducing antigen presentation, leading to a deficient T cell response. In addition, hypoxia could favor fibrosis by promoting profibrotic cytokines response and by sequestering fibroblasts in the vicinity of granulomas.
Journal Article
79 Papilledema – don’t forget the sarcoid!
Sarcoidosis is a chronic granulomatous disorder of unknown aetiology that can affect any organ, commonly the lungs, and is characterised by the presence of non-caseating granulomas. Neurosarcoidosis can affect the nervous system in 10% of cases and ocular involvement can range from 25% - 60%. We present the case of a 37 year old female who presented with incidental severe bilateral papilledema and a left lower motor nerve seventh palsy. The rest of her examination was unremarkable. CT, CT venogram and MRI head showed no abnormality. CT TAP revealed left hilar lymphadenopathy and endoscopic bronchial ultrasound (EBUS) with histology revealed non caseation granulomas cinching the diagnosis of sarcoidosis. CSF was also supportive showing lymphocytic pleocytosis. After extensive workup she was treated as neurosarcoid with ocular involvement with high dose steroids and had complete resolution of her facial nerve palsy and improvement in her optic disc swelling bilaterally. Our case highlights sarcoidosis as an unusual but treatable cause for papilledema.justine.edwards1@nhs.net
Journal Article
77 Sarcoid myopathy and inclusion body myositis – a coexistence or continuum of a spectrum
Sarcoidosis and inclusion body myositis are idiopathic inflammatory diseases which are rarely reported to coexist. We report a series of two patients with these conditions and describe their clinical and pathophysiological findings.Case 1 is a 65-year-old male who had insidious onset proximal muscle weakness on a background of sarcoidosis. Neurophysiology showed involvement of the tibialis anterior, quadriceps and long finger extensors. Muscle biopsy revealed non-caseating granulomas consistent with sarcoid myopathy as well as vacuoles with infiltrates as seen in inclusion body myositis.Case 2 is a 64-year-old female who presented with proximal muscle weakness along with swallowing difficulty with past history of sarcoidosis. Clinically there was weakness of small muscle of hands and quadriceps. Electrophysiology and muscle biopsy confirmed inclusion body myositis along with some non-caseating granuloma suggestive of combined pathology.As reported in literature, these cases may represent either a dual pathology or more likely development of inclusion body myositis in sarcoid myopathy. Treatment strategies are conflicting; unlike sarcoidosis, inclusion body myositis does not respond to treatment with steroids and immunosuppressants. These cases highlight the importance of reviewing patients clinically and pathologically for possible coexisting disease as the treatment strategy would need to be tailored accordingly.drzaibwazir@gmail.com
Journal Article