WordNet
- a pattern of symptoms indicative of some disease
- a complex of concurrent things; "every word has a syndrome of meanings"
PrepTutorEJDIC
- (疾患の徴候となる一群の)症徴候,症候群 / (事件・社会的状態などのパターンを示す)徴候形態
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出典(authority):フリー百科事典『ウィキペディア(Wikipedia)』「2015/06/17 00:42:59」(JST)
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Löfgren syndrome |
Classification and external resources |
DiseasesDB |
11797 |
Löfgren syndrome is a type of acute sarcoidosis[1] that is frequent in Scandinavian, Irish, African and Puerto Rican women.
It was characterized in 1953[2] by Sven Halvar Löfgren, a Swedish clinician.[3]
Some have considered the condition to be imprecisely defined.[4]
Contents
- 1 Presentation
- 2 Prognosis
- 3 Genetics
- 4 Treatment Options
- 5 See also
- 6 References
Presentation
It is characterized by enlargement of the lymph nodes near the inner border of the lungs (called "hilar lymphadenopathy") as seen on x-ray, and tender red nodules (erythema nodosum) are classically present on the shins, predominantly in women. It may also be accompanied by arthritis (more prominent in men) and fever. The arthritis is often acute and involves the lower extremities.[5]
Löfgren syndrome consists of the triad of erythema nodosum, bilateral hilar lymphadenopathy on chest radiograph, and arthralgia.
Prognosis
Löfgren syndrome is associated with a good prognosis,with > 90% of patients experiencing disease resolution within 2 years.
In contrast, patients with the disfiguring skin condition lupus pernio or cardiac or neurologic involvement rarely experience disease remission.
Genetics
Recent studies have demonstrated that the HLA-DQB1*0201 is strongly associated with Löfgren syndrome.
It is associated with HLA-DRB1*03.[6]
Treatment Options
NSAIDs (non steroid anti-inflammatory drug) is the usual recommended treatment for Löfgren syndrome.
See also
- List of cutaneous conditions
References
- ^ Ohta H; Tazawa R; Nakamura A et al. (2006). "Acute-onset sarcoidosis with erythema nodosum and polyarthralgia (Löfgren's syndrome) in Japan: a case report and a review of the literature". Intern. Med. 45 (9): 659–662. doi:10.2169/internalmedicine.45.1452. PMID 16755100. Retrieved 2009-06-10.
- ^ Löfgren S (1953). "Primary pulmonary sarcoidosis. I. Early signs and symptoms". Acta Med Scand 145 (6): 424–431. doi:10.1111/j.0954-6820.1953.tb07039.x. PMID 13079656.
- ^ Sven Halvar Löfgren biography
- ^ Grunewald J, Eklund A (January 2007). "Sex-specific manifestations of Löfgren's syndrome". Am. J. Respir. Crit. Care Med. 175 (1): 40–44. doi:10.1164/rccm.200608-1197OC. PMC 1899259. PMID 17023727.
- ^ Sam, Amir H.; James T.H. Teo (2010). Rapid Medicine. Wiley-Blackwell. ISBN 1405183233.
- ^ Grunewald J, Eklund A (February 2009). "Löfgren's syndrome: human leukocyte antigen strongly influences the disease course". Am. J. Respir. Crit. Care Med. 179 (4): 307–312. doi:10.1164/rccm.200807-1082OC. PMID 18996998.
Sarcoidosis (D86, 135)
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Integuement |
- Cutaneous manifestations
- Annular
- Erythrodermic
- Ichthyosiform
- Hypopigmented
- Morpheaform
- Mucosal
- Papular
- Scar
- Subcutaneous
- Ulcerative
- Lupus pernio
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Central nervous system |
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Lymph node |
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Systemic |
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Ungrouped |
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UpToDate Contents
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English Journal
- BTNL2 gene polymorphism associations with susceptibility and phenotype expression in sarcoidosis.
- Morais A, Lima B, Peixoto MJ, Alves H, Marques A, Delgado L.SourcePneumology Department, Centro Hospitalar São João, Faculdade de Medicina, Universidade do Porto, 4200-319 Porto, Portugal. Electronic address: amorais3@hotmail.com.
- Respiratory medicine.Respir Med.2012 Dec;106(12):1771-7. doi: 10.1016/j.rmed.2012.08.009. Epub 2012 Sep 25.
- A functional polymorphism within butyrophilin-like 2 (BTNL2) gene has been described as a potential risk factor for sarcoidosis. The association between chronicity and the rs2076530 SNP A allele has also been reported. This study evaluates the BTNL2 rs2076530 G/A allele associations with sarcoidosis
- PMID 23017494
- Polymerase gamma deficiency (POLG): clinical course in a child with a two stage evolution from infantile myocerebrohepatopathy spectrum to an Alpers syndrome and neuropathological findings of Leigh's encephalopathy.
- Scalais E, Francois B, Schlesser P, Stevens R, Nuttin C, Martin JJ, Van Coster R, Seneca S, Roels F, Van Goethem G, Löfgren A, De Meirleir L.SourceDivision of Paediatric Neurology, Centre Hospitalier de Luxembourg, Luxembourg. scalais.emmanuel@chl.lu
- European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology Society.Eur J Paediatr Neurol.2012 Sep;16(5):542-8. doi: 10.1016/j.ejpn.2012.01.013. Epub 2012 Feb 17.
- AIMS: Description of the clinical course in a child compound heterozygous for POLG1 mutations, neuropathology findings and results of dietary treatment based on fasting avoidance and long chain triglycerides (LCT) restriction.RESULTS: At 3(1/2) months of age the patient presented with severe hypogly
- PMID 22342071
Japanese Journal
- 野田 一成,村瀬 享子,大滝 美浩 [他]
- 日本呼吸器学会誌 = Annals of the Japanese Respiratory Society 2(5), 578-583, 2013-09-10
- NAID 40019815001
- 症例報告 Lofgren症候群の2例 : 文献報告例における欧米との比較
- 森 由弘,菊池 宏,市川 裕久 [他]
- 日本サルコイドーシス/肉芽腫性疾患学会雑誌 = The Japanese journal of sarcoidosis and other granulomatous disorders 33(1・2), 91-96, 2013
- NAID 40019835773
Related Links
- Löfgren syndrome is a type of acute sarcoidosis that is frequent in Scandinavian, Irish, African and Puerto Rican women. It was characterized in 1953 by Sven Halvar Löfgren, a Swedish clinician. Some have considered the condition to be ...
- Löfgren's syndrome is a subtype of acute sarcoidosis involving: Hilar lymphadenopathy Erythema nodosum Joint symptoms Fever It is named after Swedish...
★リンクテーブル★
[★]
- 英
- Lofgren syndrome
- 同
- Lofgren症候群
- サルコイドーシスの特殊型
- 結節性紅斑、BHL、関節痛の三徴候を伴う。急性で白人女性に多い。
[★]