Medicine:Löfgren syndrome
| Löfgren syndrome | |
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| 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.) |
Löfgren syndrome is a type of acute sarcoidosis,[1] an inflammatory disorder characterized by swollen lymph nodes in the chest, tender red nodules on the shins, fever and arthritis.[2] It is more common in women than men, and is more frequent in those of Scandinavian, Irish, African and Puerto Rican heritage. It was described in 1953[3] by Sven Halvar Löfgren, a Swedish clinician.[4] Some have considered the condition to be imprecisely defined.[5]
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, particularly the ankles.[2]
Löfgren syndrome consists of the triad of erythema nodosum, bilateral hilar lymphadenopathy on chest radiograph, and joint pain.[citation needed]
Genetics
Recent studies have demonstrated that the HLA-DRB1*03 is strongly associated with Löfgren syndrome.[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.[citation needed]
Treatment
NSAIDs (nonsteroidal anti-inflammatory drugs) are the usual recommended treatment for Löfgren syndrome.[7] 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.[citation needed]
See also
- List of cutaneous conditions
- Sarcoidosis
References
- ↑ 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.
- ↑ 2.0 2.1 Sam, Amir H.; James T.H. Teo (2010). Rapid Medicine. Wiley-Blackwell. ISBN 978-1405183239.
- ↑ 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
- ↑ "Sex-specific manifestations of Löfgren's syndrome". Am. J. Respir. Crit. Care Med. 175 (1): 40–44. January 2007. doi:10.1164/rccm.200608-1197OC. PMID 17023727.
- ↑ "Löfgren's syndrome: human leukocyte antigen strongly influences the disease course". Am. J. Respir. Crit. Care Med. 179 (4): 307–312. February 2009. doi:10.1164/rccm.200807-1082OC. PMID 18996998.
- ↑ Flores, Rui; Caridade, Sofia (January 2023). "Löfgren Syndrome: Clinical Presentation, Clinical Course, and Literature Review". Cureus 15 (1): e33651. doi:10.7759/cureus.33651. ISSN 2168-8184. PMID 36788849.
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