Löfgren syndrome is a type of acute

sarcoidosis Sarcoidosis (; also known as Besnier–Boeck–Schaumann disease) is a disease involving abnormal collections of White blood cell, inflammatory cells that form lumps known as granulomata. The disease usually begins in the lungs, skin, or lymph n ...

, an inflammatory disorder characterized by swollen

lymph nodes A lymph node, or lymph gland, is a kidney-shaped Organ (anatomy), 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 lymphoc ...

in the chest, tender red nodules on the shins,

fever Fever or pyrexia in humans is a symptom of an anti-infection defense mechanism that appears with Human body temperature, body temperature exceeding the normal range caused by an increase in the body's temperature Human body temperature#Fever, s ...

and

arthritis Arthritis is a general medical term used to describe a disorder that affects joints. Symptoms generally include joint pain and stiffness. Other symptoms may include redness, warmth, Joint effusion, swelling, and decreased range of motion of ...

. It is more common in women than men, and is more frequent in those of Scandinavian, Irish,

Africa Africa is the world's second-largest and second-most populous continent after Asia. At about 30.3 million km2 (11.7 million square miles) including adjacent islands, it covers 20% of Earth's land area and 6% of its total surfac ...

n and Puerto Rican heritage. It was described in 1953 by Sven Halvar Löfgren, a Swedish clinician. 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 includ ...

s near the inner border of the lungs (called " hilar

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 c ...

") as seen on

x-ray An X-ray (also known in many languages as Röntgen radiation) is a form of high-energy electromagnetic radiation with a wavelength shorter than those of ultraviolet rays and longer than those of gamma rays. Roughly, X-rays have a wavelength ran ...

, and tender red nodules ( erythema nodosum) 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 erythema nodosum, bilateral hilar lymphadenopathy on

chest radiograph A chest radiograph, 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 medicine. L ...

, and

joint pain Arthralgia () literally means 'joint pain'. Specifically, arthralgia is a symptom of injury, infection, illness (in particular arthritis), or an allergic reaction to medication Medication (also called medicament, medicine, pharmaceutic ...

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 drug Non-steroidal anti-inflammatory drugs (NSAID) are members of a Indication (medicine), therapeutic drug class which Analgesic, reduces pain, Anti-inflammatory, decreases inflammation, Antipyretic, decreases fever, and Antithrombotic, prevents bl ...

s) are the usual recommended treatment for Löfgren syndrome.

Colchicine Colchicine is a medication used to prevent and treat gout, to treat familial Mediterranean fever and Behçet's disease, and to reduce the risk of myocardial infarction. The American College of Rheumatology recommends colchicine, nonstero ...

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. Treatment with NSAIDs or corticosteroids leads to symptomatic relief of Löfgren syndrome 90% of the time within six weeks of symptom onset. Rarely, long-term joint damage (chronic sarcoid arthropathy) may develop. Disease modifying antirheumatic drugs, also known as DMARDs, are used for such cases.

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.

References

External links

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