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 af ...

, 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 includ ...

in the chest, tender red nodules on the shins,

fever Fever, also referred to as pyrexia, is defined as having a body temperature, temperature above the human body temperature, normal range due to an increase in the body's temperature Human body temperature#Fever, set point. There is not a single ...

and

arthritis Arthritis is a term often used to mean any disorder that affects joints. Symptoms generally include joint pain and stiffness. Other symptoms may include redness, warmth, swelling, and decreased range of motion of the affected joints. In som ...

. 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 node 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 ...

s near the inner border of the lungs (called "

hilar Hilum may refer to: * Hilum (anatomy), a part of an organ where structures such as blood vessels and nerves enter the body * Hilum (botany), a scar on a seed or spore created by detachment See also * Fovea (disambiguation), another term associat ...

lymphadenopathy Lymphadenopathy or adenopathy is a disease of the lymph nodes, in which they are abnormal in size or consistency. Lymphadenopathy of an inflammatory type (the most common type) is lymphadenitis, producing swollen or enlarged lymph nodes. In cli ...

") 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

(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 A chest radiograph, called a chest X-ray (CXR), or chest film, is a projection radiograph of the chest used to diagnose conditions affecting the chest, its contents, and nearby structures. Chest radiographs are the most common film taken in med ...

, 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 HLA class II histocompatibility antigen, DRB1 beta chain is a protein that in humans is encoded by the ''HLA-DRB1'' gene. DRB1 encodes the most prevalent beta subunit of HLA-DR. DRB1 alleles, especially those encoding amino acid sequence changes at ...

*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 drug Non-steroidal anti-inflammatory drugs (NSAID) are members of a therapeutic drug class which reduces pain, decreases inflammation, decreases fever, and prevents blood clots. Side effects depend on the specific drug, its dose and duration of ...

s) 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 ...

or low-dose

prednisone Prednisone is a glucocorticoid medication mostly used to immunosuppressive drug, 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 ...

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