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Löfgren's syndrome is a subtype of acute sarcoidosis involving:[1]

  • Hilar lymphadenopathy
  • Erythema nodosum
  • Joint symptoms
  • Fever

It is named after Swedish researcher, Sven Löfgren (1910-1978), who worked on sarcoidosis in the 1940s and 1950s.

Patients with Löfgren's syndrome generally have a good prognosis, are unlikely to develop chronic disease (sarcoidosis is considered chronic when symptoms last for more than three years) and most patients with Löfgren's can expect a self-limiting illness and spontaneous remission.[2]As with other forms of sarcoidosis, aetiology is unknown - the interaction of an unidentified environmental trigger and a genetically susceptible host is likely. 'A case-control etiologic study of sarcoidosis' (ACCESS) - a large US-based case-control study - failed to find any single environmental or occupational causative factor.[3, 4]

What differentiates individuals who develop Löfgren's syndrome from other forms of sarcoidosis is also unclear: the effects of different polymorphisms in the the CR2 gene on chromosome 3 are being investigated, one particular haplotype of which appears to be associated with an increased risk of Löfgren's syndrome.[5, 6]

  • Incidence varies widely around the world, with some populations (notably the Irish and Nordic people) more prone to developing Löfgren's syndrome.
  • In Europe and the USA, sarcoidosis initially presents with Löfgren's syndrome in 10% of patients.[7]
  • A study in Catalonia suggested an incidence of 0.65 per 100,000 per annum. It is much rarer in other areas of the world, such as Japan.[8]

Risk factors

  • Strong female predominance.
  • Young to middle-age (mean age of onset - 35 years).
  • There is a strong association with human leukocyte antigen (HLA)-DRB1 alleles. The association with HLA-DRB1*03 is particularly striking and is a very strong marker for a good prognosis.[9, 10]
  • Seasonality - presentation is more common in spring months (northern hemisphere).[11]


  • Arthralgia.
  • Cough or dyspnoea.
  • Fever or malaise.


  • Erythema nodosum.
  • Periarticular ankle inflammation/bilateral ankle arthritis.
  • Bilateral Achilles tendonitis (rare).[12]
  • Uveitis.

Presentation appears to differ between men and women, with a predominance of erythema nodosum amongst women and bilateral ankle arthritis in men.[13]

Löfgren's syndrome needs to be distinguished from other causes of:

Investigations indicating active sarcoidosis include:[1]

  • CXR (abnormalities include mediastinal lymphadenopathy or pulmonary infiltration).
  • Gallium-67 scan may be used when CXR is normal; shows increased hilar or paratracheal uptake).
  • Lung function tests (decreased forced vital capacity).
  • Serum calcium level (may be elevated).
  • Serum angiotensin-converting enzyme (may be increased).
  • Lymph node biopsy.
Always remember to perform CXR in those presenting with periarticular ankle signs.

The ankle arthritis may best be demonstrated by MRI scan.[15]

  • Once Löfgren's syndrome can be confidently diagnosed, the patient can be reassured that the condition is benign and normally self-limiting.
  • Routine biopsy is not required to confirm the diagnosis unless there are atypical features.
  • Management is supportive - eg, non-steroidal anti-inflammatory drugs for arthralgia.
  • Prednisolone may be required for more severe cases.
  • Follow-up should continue until any hilar lymphadenopathy has resolved.

Prognosis is usually very good, typically resolving within one year. In a minority, disease remains active (8%) or relapses (6%), sometimes after a long period.[1]Good prognostic markers include:

  • Normal serum angiotensin-converting enzyme levels at diagnosis.
  • Particular HLA types - being HLA-DRB1*03 negative increases the risk of non-resolving disease.[16]

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Further reading and references

  1. Byun CW, Yang SN, Yoon JS, et al; Lofgren's Syndrome-Acute Onset Sarcoidosis and Polyarthralgia: A Case Report. Ann Rehabil Med. 2013 Apr37(2):295-9. doi: 10.5535/arm.2013.37.2.295. Epub 2013 Apr 30.

  2. Mana J, Marcoval J; Skin manifestations of sarcoidosis. Presse Med. 2012 Jun41(6 Pt 2):e355-74. doi: 10.1016/j.lpm.2012.02.046. Epub 2012 May 9.

  3. Newman LS, Rose CS, Bresnitz EA, et al; A case control etiologic study of sarcoidosis: environmental and occupational risk factors. Am J Respir Crit Care Med. 2004 Dec 15170(12):1324-30. Epub 2004 Sep 3.

  4. Rossman MD, Kreider ME; Lesson learned from ACCESS (A Case Controlled Etiologic Study of Sarcoidosis). Proc Am Thorac Soc. 2007 Aug 154(5):453-6.

  5. Spagnolo P, Renzoni EA, Wells AU, et al; C-C chemokine receptor 2 and sarcoidosis: association with Lofgren's syndrome. Am J Respir Crit Care Med. 2003 Nov 15168(10):1162-6. Epub 2003 Jul 25.

  6. Valentonyte R, Hampe J, Croucher PJ, et al; Study of C-C chemokine receptor 2 alleles in sarcoidosis, with emphasis on family-based analysis. Am J Respir Crit Care Med. 2005 May 15171(10):1136-41. Epub 2005 Mar 4.

  7. Kobak S, Yalcin M, Sever F, et al; Sarcoidosis Presenting as Lofgren's Syndrome with Myopathy. Case Rep Rheumatol. 20132013:125251. doi: 10.1155/2013/125251. Epub 2013 Apr 3.

  8. Ohta H, Tazawa R, Nakamura A, et al; Acute-onset sarcoidosis with erythema nodosum and polyarthralgia (Lofgren's syndrome) in Japan: a case report and a review of the literature. Intern Med. 200645(9):659-62. Epub 2006 Jun 1.

  9. Grunewald J; HLA associations and Lofgren's syndrome. Expert Rev Clin Immunol. 2012 Jan8(1):55-62. doi: 10.1586/eci.11.76.

  10. Darlington P, Gabrielsen A, Sorensson P, et al; HLA-alleles associated with increased risk for extra-pulmonary involvement in sarcoidosis. Tissue Antigens. 2014 Apr83(4):267-72. doi: 10.1111/tan.12326.

  11. Sipahi Demirkok S, Basaranoglu M, Dervis E, et al; Analysis of 87 patients with Lofgren's syndrome and the pattern of seasonality of subacute sarcoidosis. Respirology. 2006 Jul11(4):456-61.

  12. Ortiz V, Holgado S, Olive A, et al; Achilles tendinitis as the presentation form of Lofgren's syndrome. Clin Rheumatol. 200019(2):169-70.

  13. Grunewald J, Eklund A; Sex-specific manifestations of Lofgren's syndrome. Am J Respir Crit Care Med. 2007 Jan 1175(1):40-4. Epub 2006 Oct 5.

  14. Kumar G, Kumar N; All red is not always bacterial cellulitis: a case of Lofgren's syndrome. WMJ. 2010 Feb109(1):31-3.

  15. Anandacoomarasamy A, Peduto A, Howe G, et al; Magnetic resonance imaging in Lofgren's syndrome: demonstration of periarthritis. Clin Rheumatol. 2007 Apr26(4):572-5. Epub 2006 Aug 2.

  16. Grunewald J, Eklund A; Lofgren's syndrome: human leukocyte antigen strongly influences the disease Am J Respir Crit Care Med. 2009 Feb 15179(4):307-12. Epub 2008 Nov 7.