Löfgren syndrome is a subtype of sarcoidosis. It is characterized by triad, that is, the simultaneous occurrence of three symptoms: erythema nodosum, bihilary lymphadenopathy, and arthritis.
What is Löfgren’s syndrome?
Löfgren syndrome is the acute form of the disease sarcoidosis. It is named after Sven Halvar Löfgren, the first person to describe it. Löfgren’s syndrome, a highly acute clinical picture, usually begins very suddenly. Visible signs of the disease include erythema nodosum and arthritis. The diagnosis of the disease is guided by bihilary lymphadenopathy, which, however, can usually only be visualized by an X-ray of the lungs. The disease primarily affects young people (more women than men), with a peak of disease observed between 20 and 30. Löfgren’s syndrome can cause numerous symptoms.
Causes
The causes of Löfgren syndrome remain unknown to date. However, with regard to the frequency of the disease, a seasonal variation has been observed (peaks in spring and autumn), but even this background could not be explained at present. All currently known findings suggest that Löfgren’s syndrome is an overreaction of the immune system. There may be several triggers for this. These include, for example, inhalative noxae, i.e. damage and impairment caused by inhalation. Furthermore, it is often observed that Löfgren’s syndrome occurs shortly after childbirth. The reason for this is thought to be a faulty adjustment of the patient’s own immune system after pregnancy. The psyche also seems to have a role, as Löfgren’s syndrome also occurs when there is a strong mental stress.
Symptoms, complaints, and signs
The symptoms caused by Löfgren’s syndrome are varied and can vary considerably between patients. The symptom pattern of the disease also depends on whether it is acute or chronic.
- Acute course form
In the acute variant, which affects about one-third of patients, the disease usually begins suddenly. Symptoms include fever (sometimes very high), night sweats, fatigue, loss of performance, nausea and nausea. However, stomach complaints, painful inflamed skin nodules (preferably on the legs), acute joint inflammation with pain in the joints (arthritis), swelling of the lymph nodes between the lungs, which can only be seen on an X-ray, and slight shortness of breath can also occur. Irritable cough or weight loss are uncommon.
- Chronic course
The chronic form begins insidiously. In addition, it lasts longer compared to the acute form. Patients often have no complaints and do not feel limited in performance. Others suffer from general symptoms of the disease, for example, a fatigue, weakness, lack of efficiency, sweating, dry irritating cough, feeling of pressure in the chest. Depending on which organs are affected by the disease, it is also possible that visual disturbances, an altered skin appearance, cardiac arrhythmia or other complaints occur. As the disease progresses, symptoms such as shortness of breath and weight loss may also occur with exertion.
Diagnosis and disease progression
In most patients suffering from Löfgren’s syndrome, the typical laboratory findings cannot be detected. As a result, the disease is not diagnosed or is diagnosed late. As a rule, the course of Löfgren’s syndrome is positive. The disease resolves completely in most patients after a few weeks or months. Acute sarcoidosis also heals without consequences in the majority if left untreated. The severe initial symptoms usually subside significantly after four to six weeks or have disappeared completely. The remaining signs of the disease may persist longer. In general, it is possible that the patient will not feel completely healthy and able to perform again until a year or even later. Only rarely does the disease cause permanent damage to various organs such as the skin, lungs or heart. In the chronic course, Löfgren’s syndrome heals without consequences in about half of the patients.
Complications
The symptoms that can occur as a result of Löfgren’s syndrome are very diverse. For this reason, a direct and quick diagnosis of this disease is not possible in most cases, so that an early treatment of the disease is usually not possible either. In most cases, those affected suffer from a severe fever. This also results in fatigue and a significantly reduced ability to cope with stress on the part of the patient. Furthermore, those affected by Löfgren’s syndrome also suffer from nausea or vomiting. The quality of life is considerably reduced and limited by this syndrome. It is not uncommon for pneumonia to occur, resulting in respiratory distress. In severe cases, this can also lead to a loss of consciousness, in which case the affected person may possibly injure himself. Due to discomfort in the heart, sudden cardiac death can also occur in the patient in the worst case. The treatment of Löfgren’s syndrome is carried out with the help of cortisone and in many cases leads to a positive course of the disease. However, early treatment is necessary in this case to prevent secondary damage or complications.
When should you go to the doctor?
Fever, cough and rheumatic symptoms indicate a serious condition that needs to be diagnosed by a doctor. When these symptoms occur, it is best to see the family doctor immediately so that a diagnosis can be made quickly. People between the ages of 20 and 40 are most at risk, especially young women and middle-aged men. The disease also occurs predominantly in spring and autumn. People who belong to the above-mentioned risk groups or who suffer from an immune deficiency should see their general practitioner if they show any of the signs described. An early start of treatment significantly improves the chances of recovery. Nevertheless, regular visits to the doctor are also indicated during and after therapy. In addition to the general practitioner, Löfgren’s syndrome can be treated by dermatologists, internists and rheumatologists, depending on the type and severity of the clinical picture. In a severe course with muscle damage and neurological deficits, a physiotherapist may need to be consulted. Severely ill patients should seek placement in a specialized clinic early on in conjunction with their primary care physician.
Treatment and therapy
Treatment of Löfgren’s syndrome depends primarily on the symptoms and the resulting functional impairment. In most cases, therapy starts with cortisone-free anti-inflammatory drugs. If there is highly acute arthritis as well as pronounced inflammatory activity, cortisone is usually used. It is important that the cortisone dose be sufficiently high in the initial phase. In addition, it must not be reduced or discontinued too quickly, even during therapy, in order to avoid a relapse with even more severe symptoms. The reduction of the dose depends on how the patient responds to the initial treatment and how the laboratory values develop. The acute phase in particular should be carefully monitored. Optimal treatment requires a great deal of experience. If Löfgren’s syndrome takes a chronic course, further diagnosis and appropriate therapy are required.
Outlook and prognosis
Although the process of diagnosis as well as the healing path are complex and lengthy, the prognosis is nevertheless favorable. In principle, there is a prospect of freedom from symptoms even without seeking medical care. In most cases, those affected suffer from a variety of symptoms for several months. These lead to a severe impairment of lifestyle and thus limit the quality of life. Nevertheless, a positive course of the disease is documented in most patients. In exceptional cases, psychological problems can develop as a result of the shortness of breath. Anxiety or panic sets in, which in particularly severe cases can lead to an anxiety disorder. This circumstance must be taken into account when making an overall prognosis. Normally, the sooner the diagnosis can be made, the sooner a response can be made. There are various therapeutic approaches that can be used. Since the disease mostly occurs sporadically, this complicates the diagnosis. Since the cause of Löfgren’s syndrome has not yet been clarified, symptomatic therapy is used. The immune system is supported so that the overreaction is avoided in the future.Often the organism of the affected person is in a phase of heavy stress when the complaints occur. If stressors are reduced and lifestyle is optimized, improvements and changes in the complaints can be observed.
Prevention
Prevention of Löfgren’s syndrome is not currently possible because too little is known about the causes of the disease and how it can be influenced. In more than half of those affected, Löfgren’s syndrome disappears on its own. In other cases, it is important to follow the recommended treatment. This is to prevent the progression of the disease and possible organ damage. Some patients also receive rehabilitation to restore physical as well as mental abilities. A stay at the sea also has a positive effect due to the healing climate. This can strengthen the immune system, which supports the healing process.
Aftercare
In most cases, Löfgren’s syndrome is associated with various complications and complaints that need to be examined and treated by a doctor during follow-up care. Rehabilitation measures are aimed at restoring the patient’s usual, physical condition, which is individualized depending on the degree of the disease. Most of those affected react irritably due to the weakening or suffer from depression and other psychological upsets. Relaxing exercises such as yoga or meditation can contribute to the recovery process. In many cases, however, the life expectancy of the affected person is limited.
What you can do yourself
Löfgren’s syndrome, as a particular form of sarcoidosis, is usually benign. Spontaneous healing occurs in about 80 to 90 percent of all patients. Nevertheless, the healing process can still be accelerated by personal commitment. During the rehabilitation phase, the patient should be treated regularly in specialized clinics that deal specifically with sarcoidosis and its effects. Rehabilitation clinics with a special healing climate are recommended, if possible with a location at high altitudes or by the sea. Of particular importance is a consistent exercise therapy. Patients in the rehabilitation phase should therefore take daily walks in the fresh air to strengthen their physical defences. At the same time, exercise also ensures better tolerance of the effects of the medication. Since Löfgren’s syndrome has a wide variety of manifestations, however, there is no uniform rehabilitation concept. The use of medication depends on the severity of the disease. For many patients, however, it is also helpful to exchange information with other sufferers. For this purpose, there are, among others, self-help groups for sarcoidosis patients that regularly organize discussion meetings to exchange experiences. Although the disease usually heals on its own, the shared experiences can provide a positive force for accelerated healing. In addition to the shared experiences, those affected also get the feeling at the same time that they are not alone with their complaints and problems and that successful overcoming of the disease is possible.
