Löfgren syndrome (LS), first described by the Swedish pulmonologist Sven Löfgren in
1946, is one of the most familiar syndromes in medicine. In the original investigative
paper, LS represented a specific phenotypic presentation of sarcoidosis and was associated
with an excellent long term prognosis.
1
The onset of LS was characteristically acute with the constellation of the following
findings: bilateral hilar lymphadenopathy (BHL) on chest radiography, erythema nodosum
(EN), and arthritis or periarthritis, commonly involving the ankle joints.
2
Subsequently, the presence of EN was determined to be a conditional criterion, as
there was a significant difference in incidence between genders. Guidelines have proposed
that a confident clinical diagnosis of sarcoidosis can be made without any tissue
evidence if LS is the initial presentation.
3
However, this can be challenging, especially in areas where fungal infections are
endemic, and the availability of subspecialty expertise is few and far between. Identification
of LS is vital, as most patients can be managed without a commitment to long term
systemic steroid therapy and avoidance of complications from such treatment. Genetic
studies have further identified that a particular subgroup of patients with LS who
are HLA-DRB1×03 positive, to have an even better prognosis.To read this article in full you will need to make a payment
Purchase one-time access:
Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online accessOne-time access price info
- For academic or personal research use, select 'Academic and Personal'
- For corporate R&D use, select 'Corporate R&D Professionals'
Subscribe:
Subscribe to The American Journal of the Medical SciencesAlready a print subscriber? Claim online access
Already an online subscriber? Sign in
Register: Create an account
Institutional Access: Sign in to ScienceDirect
References
- Studies on etiology and pathogenesis in 185 adult cases.Acta Med Scand. 1946; 124: 1
- Löfgren's syndrome: human leukocyte antigen strongly influences the disease course.Am J Respir Crit Care Med. 2009; 179: 307-312
- Statement on sarcoidosis.Am J Respir Crit Care Med. 1999; 160: 736-755
- Löfgren's syndrome: diagnosis, management, and disease pathogenesis.Semin Respir Crit Care Med. 2017; 38: 463-476
- Imaging of sarcoidosis of the airways and lung parenchyma and correlation with lung function.Eur Respir J. 2012; 40: 750-765
- Erythema nodosum – review of the literature.Reumatologia. 2016; 54: 79-82
- Ultrasound findings in Löfgren's syndrome: is ankle swelling caused by arthritis, tenosynovitis or periarthritis?.J Rheumatol. 1992; 19: 38-41
- Histologic findings in siblings with acute sarcoid arthritis: association with the B8,DR3 phenotype.J Rheumatol. 1986; 13: 593-597
- Sarcoid arthritis: clinical characteristics, diagnostic aspects, and risk factors.Ann Rheum Dis. 2002 Jun; 61: 499-504
- Löfgren's syndrome revisited: a study of 186 patients.Am J Med. 1999; 107: 240-245
- Rheumatologic manifestations of histoplasmosis in the recent Indianapolis epidemic.Arthritis Rheum. 1983; 26: 1065-1070
- Diagnosis of acute pulmonary histoplasmosis by antigen detection.Clin Infect Dis Off Publ Infect Dis Soc Am. 2009; 49: 1878-1882
- Sarcoidlike manifestations of histoplasmosis.Arch Intern Med. 1989; 149: 2421-2426
Article info
Publication history
Published online: June 27, 2020
Accepted:
June 25,
2020
Received:
December 15,
2019
Identification
Copyright
© 2020 Southern Society for Clinical Investigation. Published by Elsevier Inc. All rights reserved.
