Paediatric sarcoidosis

Sarcoidosis, also called Besnier-Boeck-Schaumann disease, was first described in the 19th century as a systemic granulomatous disorder involving predominantly the lungs and the lymphatic system [1]. More than a century after, its pathogenesis remains unknown and its pathophysiology remains controversial [2,3]. The current hypothesis is a multifactorial disease associating a genetic susceptibility and an environmental exposure that triggers or enhances the inflammatory and granulomatous process. Sarcoidosis is mainly an adult disease, children being affected around 10 times less frequently.
Source: Paediatric Respiratory Reviews - Category: Respiratory Medicine Authors: Tags: Review Source Type: research

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[Enlarged mediastinal lymph nodes of a patient with malignant melanoma stage IV under pembrolizumab treatment]. Hautarzt. 2019 Mar 18;: Authors: Nikolakis G, Brunner M, Boye H, Arndt N, Altenburg A, Vaiopoulos A, Zouboulis CC Abstract A 40-year-old patient with malignant melanoma pT4bN0M1a stage IV (AJCC classification 2017) was treated with the PD-1/PD-L1 antibody pembrolizumab. Three months after treatment initiation the patient developed enlarged mediastinal lymph nodes despite the partial response of the cutaneous metastases. An elective lymph node extirpation was performed...
Source: Der Hautarzt: Zeitschrift fur Dermatologie, Venerologie, und verwandte Gebiete - Category: Dermatology Tags: Hautarzt Source Type: research
AbstractSarcoidosis is an inflammatory disease defined by the presence of non-caseating granulomas. It can affect a number of organ systems, most commonly the lungs, lymph nodes, and skin. Cutaneous manifestations of sarcoidosis can impose a significant detriment to patients ’ quality of life. The accepted first-line therapy for cutaneous sarcoidosis consists of intralesional and oral corticosteroids, but these can fail in the face of resistant disease and corticosteroid-induced adverse effects. Second-line agents include tetracyclines, hydroxychloroquine, and methotr exate. Biologics are an emerging treatment option...
Source: American Journal of Clinical Dermatology - Category: Dermatology Source Type: research
AbstractPurposeReduced physical activity in many chronic diseases is consistently associated with increased morbidity. Little is known about physical activity in sarcoidosis. The aim of this study was to objectively assess physical activity in patients with pulmonary sarcoidosis and investigate its relationship with lung function, exercise capacity, symptom burden, and health status.MethodsPhysical activity was assessed over one week in 15 patients with pulmonary sarcoidosis and 14 age-matched healthy controls with a tri-axial accelerometer (ActivPal ™) and the International Physical Activity Questionnaire (IPAQ). Al...
Source: Lung - Category: Respiratory Medicine Source Type: research
ConclusionsThe algorithm involving CHITO, hs-CRP, and ACE could be a suitable strategy for differentiation between sarcoidosis activity/remission status.
Source: Lung - Category: Respiratory Medicine Source Type: research
ConclusionEPS may help with risk stratification in patients with CS and abnormal imaging, especially those without conventional indications for ICD placement. Among patients with LVEF>35% and no history of prior VA, a negative EPS has good positive and negative predictive value for future VA events.
Source: IJC Heart and Vasculature - Category: Cardiology Source Type: research
Authors: Boutagy NE, Feher A, Alkhalil I, Umoh N, Sinusas AJ Abstract Multimodality cardiovascular imaging is routinely used to assess cardiac function, structure, and physiological parameters to facilitate the diagnosis, characterization, and phenotyping of numerous cardiovascular diseases (CVD), as well as allows for risk stratification and guidance in medical therapy decision-making. Although useful, these imaging strategies are unable to assess the underlying cellular and molecular processes that modulate pathophysiological changes. Over the last decade, there have been great advancements in imaging instrumenta...
Source: Comprehensive Physiology - Category: Physiology Tags: Compr Physiol Source Type: research
Authors: Toussirot E, Bernard C, Bossert M Abstract PMID: 30873944 [PubMed - as supplied by publisher]
Source: Clinical and Experimental Rheumatology - Category: Rheumatology Tags: Clin Exp Rheumatol Source Type: research
We present a series of patients with sarcoidosis associated PH and describe correlation between the pre-transplant hemodynamic data from a right heart catheterization (RHC) and vascular morphologic changes in the native explanted lungs.
Source: The Journal of Heart and Lung Transplantation - Category: Transplant Surgery Authors: Tags: 117 Source Type: research
Clinical findings of cardiac sarcoidosis are evident in only about 5% of patients with sarcoidosis however approximately 20% of autopsies demonstrate myocardial involvement. The epidemiology and prognosis of cardiac sarcoidosis remains poorly understood.
Source: The Journal of Heart and Lung Transplantation - Category: Transplant Surgery Authors: Tags: 488 Source Type: research
Cardiac sarcoidosis is a potentially reversible etiology of heart failure if identified and managed in a timely fashion. Given the multisystem nature of the disease and need for a high index of suspicion, diagnosis of cardiac sarcoidosis is difficult, and the underlying cause remains speculative.
Source: The Journal of Heart and Lung Transplantation - Category: Transplant Surgery Authors: Tags: 487 Source Type: research
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