Löfgren's Syndrome

Grünewald, Johan; Eklúnd, Anders

doi:10.1164/rccm.200807-1082oc

Cited by 199 publications

(37 citation statements)

References 30 publications

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“…[37] Earlier studies showed that symptoms of erythema nodosum were, significantly, first noticed during springtime. [36,38,39] In addition, a genetic study involving Swedish and Spanish patients confirmed previous reports that the CCR2 haplotype 2 and HLA-DRB1∗0301 were independent risk factors predisposing to Löfgren syndrome. [36,37,40] A small but significant number of patients (6.4%) presented only with ankle swelling without erythema nodosum, which is considered a variant of Löfgren syndrome.…”

Section: Discussionsupporting

confidence: 82%

“…[24] Several studies have also described a high prevalence of Löfgren syndrome in northern European countries. [35,36] It has been hypothesized that Löfgren syndrome is a consequence of a combination of environmental factors, with a genetic predisposition. [37] Earlier studies showed that symptoms of erythema nodosum were, significantly, first noticed during springtime.…”

Section: Discussionmentioning

confidence: 99%

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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: Discussionsupporting

confidence: 82%

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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“…For example, the HLA-DRB1*03 and DQB1*0201 alleles have been associated with an acute disease onset, Löfgren syndrome and resolving disease, whereas in contrast HLA-DRB1*15 and DQB1*0601 are associated with chronic sarcoidosis (24–27). It is conceivable that both resolving and persistent sarcoidosis arise due to a unique combination of a specific genetic background and exposure to one or several environmental triggers (28).…”

Section: Genetic Risk Factors In Sarcoidosismentioning

confidence: 99%

Granuloma Formation in Pulmonary Sarcoidosis

et al. 2013

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Sarcoidosis is a granulomatous disorder of unknown cause, affecting multiple organs, but mainly the lungs. The exact order of immunological events remains obscure. Reviewing current literature, combined with careful clinical observations, we propose a model for granuloma formation in pulmonary sarcoidosis. A tight collaboration between macrophages, dendritic cells, and lymphocyte subsets, initiates the first steps toward granuloma formation, orchestrated by cytokines and chemokines. In a substantial part of pulmonary sarcoidosis patients, granuloma formation becomes an on-going process, leading to debilitating disease, and sometimes death. The immunological response, determining granuloma sustainment is not well understood. An impaired immunosuppressive function of regulatory T cells has been suggested to contribute to the exaggerated response. Interestingly, therapeutical agents commonly used in sarcoidosis, such as glucocorticosteroids and anti-TNF agents, interfere with granuloma integrity and restore the immune homeostasis in autoimmune disorders. Increasing insight into their mechanisms of action may contribute to the search for new therapeutical targets in pulmonary sarcoidosis.

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“…Levin et al examine DRB1 variation in the context of disease phenotype in 1,277 African Americans patients and 1,468 controls 12 . They found that the DRB1*03:02 allele conferred a similar likelihood of resolving disease in African American sarcoidosis patients as *03:01 -positivity does in Europeans 14 . While the DRB1*03:01 allele is found less frequently in African Americans 11 , the *03:02 allele could have similar clinical implications as the *03:01 allele in sarcoidosis patients of European ancestry 23 .…”

Section: Introductionmentioning

confidence: 95%

“…For instance, variation at the HLA-DRB1 locus is associated with disease course 13, 18-22 and organ-specific involvement 8, 11 in sarcoidosis. Grunewald et al demonstrated that 95% of *03-positive patients with Löfgren's syndrome experienced disease resolution within two years, whereas disease resolved for only half of *03-negative patients 14 . Subsequent work by Grunewald and his group showed that DRB1*14 and DRB1*15 tended to increase the risk for a non-resolving disease, but DRB1*14 had a more pronounced effect on non-Löfgren syndrome patients, whereas DRB1*15 has a greater effect on Löfgren syndrome patients with DRB1*03 predominating over DRB1*15 in terms of increasing the likelihood of resolving disease 9 .…”

Section: Introductionmentioning

confidence: 99%

Granuloma genes in sarcoidosis

Fischer

Rybicki

2015

Current Opinion in Pulmonary Medicine

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Purpose of review Non-necrotizing granulomas in the affected organ are the hallmark of sarcoidosis. This review summarizes most recent genetic findings in sarcoidosis with a focus on genes that might influence granuloma formation or resolution. Specific results in multiple ethnic groups and certain clinical subphenotypes, such as extra-pulmonary organ involvement, are discussed. Recent findings Associations of genetic variants in antigen-presenting molecules (HLA-DRB1) were shown to confer risk to sarcoidosis and certain disease phenotypes in populations of different ethnic origin. Specific DRB1 alleles, such as *0301 and *0302, appear to confer protection against chronic disease, but in an ethnic-specific manner illustrating the extensive genetic heterogeneity and complexity at this locus. Mechanistic studies of putative sarcoid antigens lend further credence to a role of HLA-DRB1 in disease pathogenesis. With relevance to granuloma formation, genes involved in apoptotic processes and immune cell activation were further confirmed (ANXA11 and BTNL2) in multiple ethnicities; others were newly identified (XAF1). Linking mechanism to clinical application, a TNF variant was shown to correlate with anti-TNF response in sarcoidosis patients. Summary The investigation of known and novel risk variants for sarcoidosis and specific clinical phenotypes in various ethnicities highlights the genetic complexity of the disease. Detailed subanalysis of disease phenotypes revealed the potential for prediction of extra-pulmonary organ involvement and therapy response based on the patient's genotype.

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Löfgren's Syndrome

Cited by 199 publications

References 30 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

Granuloma Formation in Pulmonary Sarcoidosis

Granuloma genes in sarcoidosis

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