Löfgren syndrome

Löfgren syndrome is a type of acute sarcoidosis[1] that is frequent in Scandinavian, Irish, African and Puerto Rican women. It was described in 1953[2] by Sven Halvar Löfgren, a Swedish clinician.[3] Some have considered the condition to be imprecisely defined.[4]

Löfgren syndrome
Löfgren syndrome includes some of the same symptoms as traditional sarcoidosis, and presents with erythema nodosum (especially of the lower extremities), bilateral arthritis of the ankle joints, and hilar lymphadenopathy. (Note: Other symptoms are classically not present in Löfgren syndrome.)

Signs and symptoms

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 joint pain.

Genetics

Recent studies have demonstrated that the HLA-DQB1*03 is strongly associated with Löfgren syndrome. It is associated with HLA-DRB1*03.[6]

Diagnosis

The triad of erythema nodosum, acute arthritis, and bilateral hilar lymphadenopathy is highly specific (>95%) for the diagnosis of Löfgren syndrome. When the triad is present, further testing with additional imaging and laboratory testing is unnecessary.

Treatment

NSAIDs (nonsteroidal anti-inflammatory drugs) are the usual recommended treatment for Löfgren syndrome. Colchicine or low-dose prednisone may also be used.

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.

See also

References
  1. Ohta H, Tazawa R, Nakamura A, et al. (2006). "Acute-onset sarcoidosis with erythema nodosum and polyarthralgia (Lofgren'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.
  2. 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.
  3. Sven Halvar Löfgren biography
  4. 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.
  5. Sam, Amir H.; James T.H. Teo (2010). Rapid Medicine. Wiley-Blackwell. ISBN 978-1405183239.
  6. 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.
Classification
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