Systemic phenotype of sarcoidosis associated with radiological stages. Analysis of 1230 patients

Pérez-Álvarez, R.; Brito-Zerón, Pilar; Kostov, Belchin; Feijoo-Massó, Carlos; Fraile, G.; Gómez-de-la-Torre, R.; De-Escalante, B.; López‐Dupla, Miguel; Alguacil, A.; Chara-Cervantes, J.; Pérez-Conesa, M.; Rascón, Javier; Garcia-Morillo, J.S.; Guerrero, P. Pérez; Fonseca‐Aizpuru, Eva; Akasbi, M.; Bonet, Mariona; Callejas‐Rubio, José Luis; De-la-Red, G.; Calvo, Eva; Soler, Cristina; Peral-Gutiérrez, E.; Gómez-Cerezo, J; Cruz-Caparrós, G.; Rodríguez-Fernández, S.; Pinilla, Blanca; Gato, A.; Chamorro, Antonio-Javier; Morcillo, César; Robles, Ángel; Ojeda, I; Vives, M.J.; Miguel‐Campo, Borja de; Penadés, M.; De-Vicente, M.; Bosch, Xavier; Pérez-de-Lis, M.; González-García, Andrés; Yllera, C.; Gracia-Tello, Borja; Perez‐Gonzalez, Alexis; Pedrosa-Aragón, M.; Tolosa, Carles; Sisó‐Almirall, Antoni; Pallarés, L; Ramos‐Casals, Manuel

doi:10.1016/j.ejim.2019.08.025

Cited by 24 publications

(26 citation statements)

References 33 publications

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“…In recent years, numerous phenotyping studies have attempted to model sarcoidosis prognosis and suggested homogeneous clusters of patients. However, many studies are still based on pulmonary involvement and fail to differentiate extrapulmonary clusters [ 7 , 33 , 34 ]. In 2018, the GenPhenReSa project proposed five clinical clusters: (1) abdominal organ involvement, (2) ocular-cardiac-cutaneous-central nervous system involvement, (3) musculoskeletal-cutaneous involvement, (4) pulmonary and intrathoracic lymph node involvement, and (5) extrapulmonary involvement [ 8 ].…”

Section: Discussionmentioning

confidence: 99%

Cardiac Sarcoidosis Is Uncommon in Patients with Isolated Sarcoid Uveitis: Outcome of 294 Cases

Richard

Jamilloux

Courand

et al. 2021

JCM

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Recently, concerns have been raised about an increased risk of cardiac sarcoidosis in patients with sarcoid uveitis. While cardiac sarcoidosis has a high mortality burden, there is still a lack of precise data on this association. The objective of this study is to describe the frequency and type of cardiac complications associated with sarcoidosis of a large cohort of patients with sarcoid uveitis. We analyzed the cardiac outcomes of a monocentric retrospective cohort of consecutive adults with a diagnosis of sarcoid uveitis between January 2004 and March 2020 in a tertiary French university hospital. A total of 294 patients with a final diagnosis of sarcoid uveitis were included. At final follow-up, seven (2.4%) patients of the cohort had cardiac sarcoidosis. Cardiac sarcoidosis was more frequent among patients with previously reported systemic sarcoidosis (p = 0.008). The prevalence of cardiac sarcoidosis among patients with sarcoid uveitis is low, but patients with previously diagnosed sarcoidosis or those who develop systemic sarcoidosis during follow-up appear to be at increased risk.

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Section: Discussionmentioning

confidence: 99%

Cardiac Sarcoidosis Is Uncommon in Patients with Isolated Sarcoid Uveitis: Outcome of 294 Cases

Richard

Jamilloux

Courand

et al. 2021

JCM

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“…6,50,51 Stages I and II are the most common radiographic manifestations in many series. 8,12,15,16 PFTs generally correlate with the overall disease process; despite this, they do not allow the differentiation of the respective influence of alveolitis, granulomas, and fibrosis. Forced vital capacity (FVC) is the simplest and most accurate parameter to reflect the impact of pulmonary sarcoidosis.…”

Section: Organ Involvementmentioning

confidence: 98%

“…In this series, the diagnosis was made at older ages in pulmonary fibrosis. 16 A sudden onset of disease is generally associated with symptoms and is followed by a prompt and spontaneous resolution, whereas insidious onset typically has nonspecific symptoms and occurs more frequently in association with features of chronic sarcoidosis. 13,18 After previous epidemiological surveys (the American AC-CESS [n ¼ 736], 30 the German-Swiss WATL [n ¼ 715] 34 and the retrospective single-center MUSC [n ¼ 1,774] 27 studies), the multicentric study GenPhenReSa (Genotype-Phenotype Relationship in Sarcoidosis) was designed to investigate the influence of genotypes on disease phenotypes, addressing this issue by the detailed characterization of 2,163 European Caucasian patients.…”

Section: General Factorsmentioning

confidence: 99%

“…11 Therapeutic management also correlates with X-ray stages: stage IV has the highest frequency of both need for and aggressiveness of therapy, while stage I has low frequencies. 8,16 Sarcoidosis patients may suffer from a wide range of manifestations, including pulmonary symptoms (e.g., coughing, breathlessness, and dyspnea on exertion); symptoms related to other involvements (e.g., cardiac and neurological involvement); and a wide range of nonspecific manifestations, such as fatigue, fever, anorexia, symptoms suggestive of small-fiber neuropathy, arthralgia, muscle pain, general and muscle weakness, exercise limitation, and cognitive failure. 1,2,[52][53][54] These symptoms often do not correspond to objective physical evidence of disease activity of a particular organ and are responsible for significant quality of life (QOL) and functional impairment.…”

Section: Organ Involvementmentioning

confidence: 99%

“…The presence of either BHL or fibrotic lesions did not influence the systemic phenotype of patients with pulmonary involvement. 16…”

Section: Organ Involvementmentioning

confidence: 99%

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Nonlife-Threatening Sarcoidosis

Castro

Pereira

2020

Semin Respir Crit Care Med

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Sarcoidosis is a systemic granulomatous disease of unknown etiology. The outcome is quite variable and is mainly related to persistent inflammatory processes and the development of fibrosis. Many prognostic factors have been described, but the disease evolution is not yet entirely known. The nonthreatening course is characterized by spontaneous involution or stability after treatment withdrawal. Löfgren's syndrome is a subset within the spectrum of sarcoidosis phenotypes, composed of acute onset of fever, bilateral hilar lymphadenopathy, erythema nodosum and/or bilateral ankle periarticular inflammation/arthritis, specifically characterized by a self-limiting disease course. In contrast, advanced fibrotic sarcoidosis with pulmonary hypertension phenotype is correlated with a poor prognosis. Further studies are necessary to detail phenotypes to better understand the mechanisms of the disease and plan future clinical therapeutic studies.

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Diagnosis and Treatment of Pulmonary Sarcoidosis

Belperio

Shaikh

Abtin

et al. 2022

JAMA

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ImportanceSarcoidosis is an inflammatory granulomatous disease of unknown cause that affects an estimated 2 to 160 people per 100 000 worldwide and can involve virtually any organ. Approximately 10% to 30% of patients with sarcoidosis develop progressive pulmonary disease.ObservationAmong patients with pulmonary sarcoidosis, the rate of spontaneous remission without serious sequelae ranges from 10% to 82%. However, lung disease progression occurs in more than 10% of patients and can result in fibrocystic architectural distortion of the lung, which is associated with a mortality rate of 12% to 18% within 5 years. Overall, the mortality rate for sarcoidosis is approximately 7% within a 5-year follow-up period. Worldwide, more than 60% of deaths from sarcoidosis are due to pulmonary involvement; however, more than 70% of deaths from sarcoidosis are due to cardiac involvement in Japan. Up to 70% of patients with advanced pulmonary sarcoidosis develop precapillary pulmonary hypertension, which is associated with a 5-year mortality rate of approximately 40%. Patients with sarcoidosis and precapillary pulmonary hypertension should be treated with therapies such as phosphodiesterase inhibitors and prostacyclin analogues. Although optimal doses of oral glucocorticoids for pulmonary sarcoidosis are unknown, oral prednisone typically starting at a dose of 20 mg/d to 40 mg/d for 2 to 6 weeks is recommended for patients who are symptomatic (cough, dyspnea, and chest pain) and have parenchymal infiltrates and abnormal pulmonary function test results. Oral glucocorticoids can be tapered over 6 to 18 months if symptoms, pulmonary function test results, and radiographs improve. Prolonged use of oral glucocorticoids may be required to control symptoms and stabilize disease. Patients without adequate improvement while receiving a dose of prednisone of 10 mg/d or greater or those with adverse effects due to glucocorticoids may be prescribed immunosuppressive agents, such as methotrexate, azathioprine, or an anti–tumor necrosis factor medication, either alone or with glucocorticoids combined with appropriate microbial prophylaxis for Pneumocystis jiroveci and herpes zoster. Effective treatments are not available for advanced fibrocystic pulmonary disease.Conclusions and RelevanceSarcoidosis has a mortality rate of approximately 7% within a 5-year follow-up period. More than 10% of patients with pulmonary sarcoidosis develop progressive disease and more than 60% of deaths are due to advanced pulmonary sarcoidosis. Oral glucocorticoids with or without another immunosuppressive agent are the first-line therapy for symptomatic patients with abnormal pulmonary function test results and lung infiltrates. Patients with sarcoidosis and precapillary pulmonary hypertension should be treated with therapies such as phosphodiesterase inhibitors and prostacyclin analogues.

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Systemic phenotype of sarcoidosis associated with radiological stages. Analysis of 1230 patients

Cited by 24 publications

References 33 publications

Cardiac Sarcoidosis Is Uncommon in Patients with Isolated Sarcoid Uveitis: Outcome of 294 Cases

Cardiac Sarcoidosis Is Uncommon in Patients with Isolated Sarcoid Uveitis: Outcome of 294 Cases

Nonlife-Threatening Sarcoidosis

Diagnosis and Treatment of Pulmonary Sarcoidosis

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