Löfgren syndrome is a type of acute

sarcoidosis Sarcoidosis (also known as ''Besnier-Boeck-Schaumann disease'') is a disease involving abnormal collections of inflammatory cells that form lumps known as granulomata. The disease usually begins in the lungs, skin, or lymph nodes. Less commonly a ...

, an inflammatory disorder characterized by swollen

lymph nodes A lymph node, or lymph gland, is a kidney-shaped organ of the lymphatic system and the adaptive immune system. A large number of lymph nodes are linked throughout the body by the lymphatic vessels. They are major sites of lymphocytes that inclu ...

in the chest, tender red nodules on the shins,

fever Fever, also referred to as pyrexia, is defined as having a temperature above the normal range due to an increase in the body's temperature set point. There is not a single agreed-upon upper limit for normal temperature with sources using val ...

and arthritis. 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 by Sven Halvar Löfgren, a Swedish clinician.Sven Halvar Löfgren biography
/ref> Some have considered the condition to be imprecisely defined.

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 An X-ray, or, much less commonly, X-radiation, is a penetrating form of high-energy electromagnetic radiation. Most X-rays have a wavelength ranging from 10 picometers to 10 nanometers, corresponding to frequencies in the range 30&nb ...

, and tender red nodules (

erythema nodosum Erythema nodosum (EN) is an inflammatory condition characterized by inflammation of the fat cells under the skin, resulting in tender red nodules or lumps that are usually seen on both shins. It can be caused by a variety of conditions, and typi ...

) are classically present on the shins, predominantly in women. It may also be accompanied by arthritis (more prominent in men) and

. The arthritis is often acute and involves the lower extremities, particularly the ankles. Löfgren syndrome consists of the triad of

, bilateral hilar lymphadenopathy on chest radiograph, and

joint pain Arthralgia (from Greek ''arthro-'', joint + ''-algos'', pain) literally means ''joint pain''. Specifically, arthralgia is a symptom of injury, infection, illness (in particular arthritis), or an allergic reaction to medication. According to MeSH, ...

Genetics

Recent studies have demonstrated that the HLA-DRB1*03 is strongly associated with Löfgren syndrome.

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 Colchicine is a medication used to treat gout and Behçet's disease. In gout, it is less preferred to NSAIDs or steroids. Other uses for colchicine include the management of pericarditis and familial Mediterranean fever. Colchicine is taken b ...

or low-dose

prednisone Prednisone is a glucocorticoid medication mostly used to suppress the immune system and decrease inflammation in conditions such as asthma, COPD, and rheumatologic diseases. It is also used to treat high blood calcium due to cancer and ad ...

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 Lupus pernio is a Chronic (medicine), chronic raised indurated (hardened) lesion of the skin, often purplish in color. It is seen on the Human nose, nose, ears, cheeks, lips, and forehead. It is pathognomonic of sarcoidosis. The name "lupus pernio" ...

or cardiac or neurologic involvement rarely experience disease remission.

References

External links

{{DEFAULTSORT:Lofgren syndrome Syndromes affecting the respiratory system Monocyte- and macrophage-related cutaneous conditions Autoimmune diseases Medical triads