Löfgren syndrome

Löfgren 's syndrome is the name given to the acute form of sarcoidosis affecting approximately one third of all cases of disease. Is named the disease after the first describer Halvar Sven Löfgren.

The Löfgren syndrome (stage I sarcoidosis ) is in typical cases characterized by the triad of symptoms:

  • Bihiläre lymphadenopathy ( usually symmetrical enlargement of the hilar lymph nodes) in all cases ( 100%)
  • Polyarthritis ( with the hocks increased participation) 80 %
  • Erythema nodosum ( nodular subcutaneous inflammation) 70-80 % of cases

Often, polyarthritis and erythema occurs in common, but it can also occur just one of two symptoms. less frequently occur:

  • Liver involvement
  • Inflammation in the eye ( iridocyclitis, conjunctivitis )
  • Meningeal irritation symptoms (symptoms that are reminiscent of meningitis )
  • Myocarditis

Laboratory examination:

  • Accelerated erythrocyte sedimentation
  • Leukocytosis, eosinophilia
  • Increased Alphaglobuline and Betaglobluline
  • No evidence of tuberculosis pathogens
  • In the acute form is often no increase in the angiotensinase I ( ACE), which is typical of the chronic form.

Frequent misdiagnosis:

  • Rheumatic fever
  • Flu
  • Lymphangits
  • Allergy
  • Tuberculosis
  • Hodgkin's disease

Cause

There are mainly patients between 20-30 years of age affected, although women predominate. There is a seasonal variation with peaks in spring and autumn. The disease is caused by an overreaction of the immune system, which triggers are unknown.

Symptoms

The Löfgren 's syndrome is a highly acute illness of sudden onset. Often it begins with irritation of the respiratory tract, similar to an infectious disease to rheumatic complaints. In 70-80 % of cases, there are coarse - nodular erythema erythema nodosum, which are highly sensitive to pressure, over -heated, reddened and swollen. They are an expression of an acute inflammation of the subcutaneous ( under the skin ) with a diameter up to 10 cm. Affected regions are usually lower legs, feet and hands. Also general symptoms such as fever, fatigue, myalgia and flu infections are observed.

Diagnostics

For the diagnosis of X-rays of the lungs, pulmonary function tests and bronchoscopy with removal of tissue (biopsy ) may be used.

Therapy

In stage 1 only controls and nichsteroidale anti-inflammatory drugs such as aspirin, ibuprofen or diclofenac are used. In stages 2 and 3 of sarcoidosis corticosteroids are used for severe cases can be a treatment trial with cytostatics.

The prognosis is usually good, in 80-90 % of cases, spontaneous recovery is observed within one to two years. As a general rule, the younger the patient and the more acute the curve, the better the prognosis.

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