Sarcoidosis presenting with and without Löfgren's syndrome: Clinical, radiological and behavioral differences observed in a group of 691patients.

Just a few series of Löfgren's syndrome have been reported. Our aim was to describe the epidemiology and clinical profile of sarcoidosis patients presenting with Löfgren's syndrome vs. non-Löfgren's syndrome. Retrospective cohort study of 691consecutive patients with sarcoidosis diagnosed at the Bellvitge University Hospital in Barcelona, Spain, between 1976 and 2018. Three hundred and nine patients (44.7%) were diagnosed with Löfgren's syndrome and 382with non-Löfgren's syndrome (55.3%). The mean age at diagnosis was 39.8years-old (SD 11.7) vs. 46.6 (SD 14.5) (P<0.001). 249 patients (80.6%) vs. 218 (57.1%) were female (P<0.001), and mostly Caucasians (304, 98.4% vs. 351, 91.9%, P=0.002). Out of the total 309, Löfgren's syndrome patients developed more frequently fever and articular involvement, and 45 (14.6%) presented with isolated periarticular ankle inflammation. When compared, radiological stages at diagnosis were more advanced in non-Löfgren's syndrome patients: stage 0 (2.9% vs. 14.7%), stage I (82.5% vs. 41.4%), stage II (14.6% vs. 29.3%), and stage III/IV (0 vs. 14.7%) (P<0.001). Chronic trend>2years was more prevalent in non-Löfgren's syndrome (66, 22.6% vs. 233, 67.4%; P<0.001), as well as the proportion of patients in whom treatment was needed (58, 18.8% vs. 224, 58.6%; P<0.001). Risk factors related to chronic trend>2 years were older age, stage II at diagnosis and the need of treatment. Löfgren's syndrome is a well-differentiated form of sarcoidosis with persuasive different epidemiological, clinical, radiological and prognostic features.

Rubio-Rivas M ，Franco J ，Corbella X 《-》

Löfgren's syndrome revisited: a study of 186 patients.

Mañá J ，Gómez-Vaquero C ，Montero A ，Salazar A ，Marcoval J ，Valverde J ，Manresa F ，Pujol R ... -  《AMERICAN JOURNAL OF MEDICINE》

Macrophage migration inhibitory factor (MIF) -173 polymorphism is associated with clinical erythema nodosum in Löfgren's syndrome.

Macrophage migration inhibitory factor (MIF) has been shown to be a key regulator in innate and adaptive immune responses. A single nucleotide polymorphism in the 5' region of the MIF gene, MIF -173∗G/C, is associated with increased MIF protein production, in vivo and in vitro. Associations have been shown between the minor MIF -173C allele and sarcoidosis patients with erythema nodosum (EN). Löfgren's syndrome is an acute and usually self-remitting phenotype of sarcoidosis. It is defined as having an acute onset with bilateral hilar lymphadenopathy (BHL), fever, erythema nodosum (EN) and/or arthritis. The aim of this study was to investigate whether MIF -173G/C associates with the susceptibility to and the clinical manifestations, i.e. arthritis or EN, of Löfgren's syndrome. A total of 171 patients with Löfgren's syndrome and 313 controls were genotyped for a single nucleotide polymorphism at position -173 of the MIF gene (SNP rs755622), using a PCR and a restriction enzyme technique. There were no significant differences found in the MIF -173C allele frequencies between patients with Löfgren's syndrome and controls. In patients with Löfgren's syndrome with only EN, a significantly increased frequency of the C minor allele was observed compared to patients with arthritis only (p=0.0095; OR 3.08, CI: 1.28-7.39). Patients with only EN compared to patients with EN and arthritis showed a significantly increased frequency of the minor C allele (p=0.044; OR 1.97, CI: 1.01-3.85). But patients with only arthritis compared to patients with EN and arthritis did not show a significant difference in C allele frequency (p=0.270; OR 0.64, CI: 0.29-1.42). The MIF -173C allele is associated with erythema nodosum in Löfgren's syndrome, but not with susceptibility to sarcoidosis. This indicates a role for MIF after antigen presenting to the T cell has taken place and the sarcoid inflammatory response has begun.

Karakaya B ，van Moorsel CH ，van der Helm-van Mil AH ，Huizinga TW ，Ruven HJ ，van der Vis JJ ，Grutters JC ... -  《-》

A Japanese patient with Löfgren's syndrome with an HLA-DR12 allele and review of literature on Japanese patients.

Ishimatsu Y ，Koyama H ，Tomonaga M ，Harada T ，Nakashima S ，Hara A ，Hara S ，Kakugawa T ，Sakamoto N ，Hayashi T ，Kohno S ... -  《-》

A common haplotype of the C-C chemokine receptor 2 gene and HLA-DRB1*0301 are independent genetic risk factors for Löfgren's syndrome.

Spagnolo P ，Sato H ，Grunewald J ，Brynedal B ，Hillert J ，Mañá J ，Wells AU ，Eklund A ，Welsh KI ，du Bois RM ... -  《-》