Severe Sarcoidosis

Kouranos, Vasilis; Jacob, Joe; Wells, Athol U.

doi:10.1016/j.ccm.2015.08.012

Cited by 26 publications

(38 citation statements)

References 73 publications

(74 reference statements)

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“…Pulmonary fibrosis from sarcoidosis is usually slowly progressive, and may range from asymptomatic to be life-threatening because of the development of respiratory failure, bronchiectasis, or pulmonary hypertension. [69–71] …”

Section: Discussionmentioning

confidence: 99%

Multidisciplinary approach and long-term follow-up in a series of 640 consecutive patients with sarcoidosis

et al. 2017

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Cohort studies of large series of patients with sarcoidosis over a long period of time are scarce. The aim of this study is to report a 40-year clinical experience of a large series of patients at Bellvitge University Hospital, a tertiary university hospital in Barcelona, Spain. Diagnosis of sarcoidosis required histological confirmation except in certain specific situations. All patients underwent a prospective study protocol. Clinical assessment and follow-up of patients were performed by a multidisciplinary team.From 1976 to 2015, 640 patients were diagnosed with sarcoidosis, 438 of them (68.4%) were female (sex ratio F/M 2:1). The mean age at diagnosis was 43.3 ± 13.8 years (range, 14–86 years), and 613 patients (95.8%) were Caucasian. At diagnosis, 584 patients (91.2%) showed intrathoracic involvement at chest radiograph, and most of the patients had normal pulmonary function. Erythema nodosum (39.8%) and specific cutaneous lesions (20.8%) were the most frequent extrapulmonary manifestations, but there was a wide range of organ involvement. A total of 492 patients (76.8%) had positive histology. Follow-up was carried out in 587 patients (91.7%), over a mean of 112.4 ± 98.3 months (range, 6.4–475 months). Corticosteroid treatment was administered in 255 patients (43.4%), and steroid-sparing agents in 49 patients (7.7%). Outcomes were as follows: 111 patients (18.9%) showed active disease at the time of closing this study, 250 (42.6%) presented spontaneous remission, 61 (10.4%) had remission under treatment, and 165 (28.1%) evolved to chronic sarcoidosis; among them, 115 (19.6%) with mild disease and 50 (8.5%) with moderate to severe organ damage. A multivariate analysis showed that at diagnosis, age more than 40 years, the presence of pulmonary involvement on chest radiograph, splenic involvement, and the need of treatment, was associated with chronic sarcoidosis, whereas Löfgren syndrome and mediastinal lymphadenopathy on chest radiograph were indicators of good outcome.Sarcoidosis is a multisystem disease with protean clinical-radiographic manifestations. Although almost half of patients follow a spontaneous resolution or under treatment, a significant number of them may have several degrees of organ damage. This study emphasizes the value of a multidisciplinary approach and long-term follow-up by specialized teams in sarcoidosis.

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Section: Discussionmentioning

confidence: 99%

Multidisciplinary approach and long-term follow-up in a series of 640 consecutive patients with sarcoidosis

et al. 2017

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“…Fibrocavitary parenchymal lung disease results in chronic pulmonary symptoms and exercise limitation and may cause respiratory insufficiency associated with airflow obstruction, bronchial stenosis, and bronchiectasis. Airflow obstruction may be caused by granulomatous airway inflammation or, in patients with advanced sarcoidosis, by parenchymal fibrosis (3). Other potential complications of fibrotic sarcoidosis include pulmonary hypertension (PH), pulmonary aspergillus syndromes including aspergilloma and chronic aspergillus infection, and opportunistic infection.…”

Section: Part Iii: Clinical Management Of Fibrotic Sarcoidosismentioning

confidence: 99%

“…However, a third of patients experience persistent or recurrent active disease. This is variably referred to as chronic, severe, advanced, refractory or progressive sarcoidosis (3–6). Regardless of the nomenclature, patients with long-standing disease are most at risk of developing fibrosis.…”

Section: Introductionmentioning

confidence: 99%

From granuloma to fibrosis

Bonham

Strek²,

Patterson³

2016

Current Opinion in Pulmonary Medicine

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Purpose of review Up to twenty percent of patients with sarcoidosis develop pulmonary fibrosis, transforming an often benign disease into a highly morbid and potentially fatal one. We highlight the fibrotic pulmonary sarcoidosis phenotype as an area of intense clinical and translational investigation, review recent developments in treatment, and provide a roadmap for future research in sarcoidosis associated pulmonary fibrosis. Recent findings Granulomatous inflammation in a lymphatic distribution is the hallmark finding of pulmonary sarcoidosis and the nidus for fibrosis. Recent research demonstrates that fibrotic sarcoidosis begins in the setting of persistent, uncontrolled inflammation, and is aided by pro-fibrotic genetic features and immune responses. Comparison to other fibrotic lung diseases also reveals key features that inform our understanding of common pathways in fibrosis. Summary Understanding the mechanisms of fibrotic transformation in sarcoidosis enhances clinical care and facilitates development of novel therapeutic options. The impact of these findings in fibrotic sarcoidosis may be amplified through application to other interstitial lung diseases marked by inflammatory to fibrotic transformation. Important aspects of clinical management of fibrotic sarcoidosis include surveillance for co-morbidities, such as pulmonary hypertension, airway disease, and infection, and assessment for pulmonary disease activity that may benefit from immunosuppression.

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“…Lipid metabolism alteration plays a role in the early phase of sarcoid formation [4,5] . It damages the plasma membrane and bronchial and lung capillary endothelial cells, as recently demonstrated by immunohistochemistry [5] .…”

Section: Lipid Metabolism and Alteration Of Capillary Endothelial Cellsmentioning

confidence: 99%

“…Symptoms, if any, are usually few (dry cough, dyspnea, chest pain, and rarely a low fever). In progressive advanced forms with fibrotic lung involvement there may be severe hypoxemia and pulmonary hypertension [1][2][3][4] .…”

mentioning

confidence: 99%

Increased Risk of Atherosclerosis in Patients with Sarcoidosis

Bargagli¹,

Rosi²,

Lavorini³

et al. 2017

Pathobiology

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Sarcoidosis is a systemic granulomatous disease of unknown etiology. Recent studies demonstrated that its pathogenesis is related with enhanced oxidative stress (protein carbonylation and lipid peroxidation) and alterations in the circulating lipid profile. Alterations of lipid metabolism (including the reduction in high-density lipoprotein cholesterol levels and apolipoprotein A1 concentrations) induce plasma membrane, bronchial and lung capillary endothelial cell damage in sarcoidosis patients. Dyslipidemia is associated with increased oxidative stress, diminished overall antioxidative protection and increased risk for atherosclerosis. Very recently increased cardiovascular biomarkers (in particular alterations of lipoprotein A and d-dimer concentrations) were observed in sarcoidosis patients, mainly in those with a high risk of atherosclerosis. Chitotriosidase, a biomarker of sarcoidosis activity and macrophage activation, is increased in serum and bronchoalveolar lavage fluid of patients with sarcoidosis as well as in patients with atherosclerosis. Lipidomics and other recent methodologies allowed the discovery of proteins involved in lipid metabolism and sarcoidosis pathogenesis, such as serum amyloid A, a biomarker of sarcoidosis activity, involved in innate immune response, inflammation and apolipoprotein metabolism. In this review lipid metabolism alteration and atherosclerosis risk in sarcoidosis patients were discussed in order to contribute to this novel and interesting research topic.

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Severe Sarcoidosis

Cited by 26 publications

References 73 publications

Multidisciplinary approach and long-term follow-up in a series of 640 consecutive patients with sarcoidosis

Multidisciplinary approach and long-term follow-up in a series of 640 consecutive patients with sarcoidosis

From granuloma to fibrosis

Increased Risk of Atherosclerosis in Patients with Sarcoidosis

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