Löfgren syndrome: symptoms, diagnosis and outlook of this form of sarcoidosis

Sarcoidosis is a rare condition in which small patches of swollen tissue called granulomas develop in the organs of the body. The NHS says it often affects the lungs and lymph nodes and can also affect the skin. Löfgren syndrome is a particular, acute pattern of sarcoidosis, first described in 1952 by the Swedish physician Sven Löfgren.

According to the Patient.info clinical reference, written by UK doctors, it involves swollen lymph glands at the roots of the lungs (hilar lymphadenopathy), a skin condition called erythema nodosum, joint symptoms and fever. These features often come together, which is why doctors can sometimes recognise the syndrome from the pattern alone.

Because it is part of sarcoidosis, the underlying cause is the same unknown mix. The NHS says the exact cause of sarcoidosis is not known and that possible causes include exposure to substances in the environment, viral or bacterial infections, genetics and the immune system attacking its own tissues. The condition is not infectious and cannot be passed from person to person.

The reference says patients typically have an acute onset, usually with fever and characteristic symptoms consisting of bilateral hilar lymphadenopathy (which can cause cough or breathlessness), erythema nodosum and bilateral ankle arthritis or periarticular inflammation. Other symptoms may include uveitis (eye inflammation), parotitis (swelling of the salivary gland), facial palsy or liver or spleen involvement. Erythema nodosum is found predominantly in women while joint problems are more common in men.

Erythema nodosum shows as tender red or purple lumps, usually on the shins. The NHS describes tender bumps or patches on the skin, particularly on the lower legs and arms, as a skin symptom of sarcoidosis, and says they can be reddish-brown or purple depending on skin colour and type of sarcoidosis.

• Tender red or purple lumps on the shins (erythema nodosum).

• Painful, swollen ankles.

• Fever and feeling unwell.

• Cough or breathlessness from swollen glands at the lung roots.

• Tiredness and general aching.

The clinical reference says Löfgren syndrome has to be distinguished from other causes of erythema nodosum, such as infection (including streptococcal infection, tuberculosis and mycoplasma), medicines, inflammatory bowel disease, lymphoma and Behçet's disease, and from other causes of hilar lymphadenopathy such as lymphoma, tuberculosis and lung cancer, particularly where the lymphadenopathy is not symmetrical.

Investigations that indicate active sarcoidosis include a chest X-ray, lung function tests, serum calcium and serum angiotensin-converting enzyme, and sometimes a lymph node biopsy. The NHS says that if symptoms suggest sarcoidosis in the lungs you may have a chest X-ray or a CT scan, and in some cases doctors may examine the airways with a flexible tube with a camera and take a small sample of lung tissue for a biopsy.

The reference notes that certain clinical features, including Löfgren syndrome, are highly specific and do not require histological confirmation. Routine biopsy is not required unless there are atypical features. An ankle MRI or ultrasound may show the typical inflammation around the joint. The NICE Clinical Knowledge Summaries says people with suspected sarcoidosis should be offered a chest X-ray and, if findings are consistent with sarcoidosis, should be referred to the appropriate specialist.

The clinical reference says that once Löfgren syndrome can be confidently diagnosed, the patient can be reassured that the condition is benign and normally self-limiting. Management is supportive, for example with non-steroidal anti-inflammatory drugs for joint pain, and prednisolone may be required in more severe cases. Follow-up should continue until the hilar lymphadenopathy has resolved.

The NHS says many people with sarcoidosis do not need treatment, and that simple lifestyle changes and over-the-counter painkillers such as paracetamol or ibuprofen are often all that is needed to control the pain of flare-ups. If treatment is recommended, steroid tablets are usually used, and the NHS notes they are only used when necessary because of side effects. Do not take anti-inflammatory painkillers if they have been advised against for you; check with a pharmacist if you have kidney problems, stomach ulcers or asthma.

The reference describes Löfgren syndrome as a benign, self-remitting disease, particularly in people carrying a certain genetic marker (HLA-DRB1*03). A chronic course of more than 2 years occurs in 8 to 22% of people with the syndrome and is associated with older age and the need for treatment.

For sarcoidosis in general, NICE Clinical Knowledge Summaries says approximately two-thirds of people experience disease remission within 2 to 5 years, with the majority requiring no treatment, and in 10 to 30% the disease becomes chronic. Löfgren syndrome sits at the more favourable end of this range.

The NHS suggests lifestyle measures for people with sarcoidosis: stop smoking if you smoke, eat a balanced diet, maintain a healthy weight, try to reduce stress, take regular exercise and get enough sleep. It lists the Sarcoidosis UK website, which has a list of support groups, and the Asthma + Lung UK website as sources of support.

Contact your doctor if breathlessness gets worse, if you develop eye pain or redness, palpitations or fainting, or if symptoms come back after they have settled, since these could reflect sarcoidosis affecting other organs. Call 999 for sudden severe chest pain or collapse.

Löfgren syndrome is usually diagnosed by a GP or in a respiratory, rheumatology or acute medical clinic on the NHS, with a chest X-ray as the key test. Because most people recover without long-term treatment, specialist follow-up is usually brief. Private consultations and scans are available, but they do not change the underlying natural history, and the follow-up chest X-ray is still the most useful way to show that the glands have settled.