sarcoidosis

Quick Overview

• What is Sarcoidosis? Inflammatory disease that accompanies the formation of nodular tissue changes. It usually affects the lungs, but can also occur in other tissues and organs.
• symptoms: depend on the organs involved and the course of the disease (acute or chronic). Typical signs of chronic sarcoidosis of the lung (most common form) are increasing irritable cough, stress-related dyspnea and swollen lymph nodes in the lung area.
• Reason: unknown. Presumably, however, several factors are involved in the pathogenesis. In addition to genetic factors are suspected harmful substances that have been inhaled.
• Therapy: Not always necessary because sarcoidosis often heals by itself (especially acute sarcoidosis). Patients should be treated for severe symptoms and / or impaired lung function. Therapy of the first choice are cortisone preparations.
• Forecast: Mostly cheap, especially in acute sarcoidosis. The more advanced the chronic sarcoidosis, the worse the chances of recovery. In some patients, pulmonary function remains permanently limited. About five percent of sarcoidosis patients die of complications.

Sarcoidosis: description

The Sarcoidosis (Boeck’s disease) is an inflammatory disease that can affect various organs and tissues in the body. That is why it is also called a multi-system disease.

In most cases, sarcoidosis affects thelung, Also Eyes, heart and skin are more likely to be affected. In principle, however, sarcoidosis can also be found in other parts of the body, for example in the area of ​​bones, kidneys, parotid and pancreas as well as in the central nervous system. Accordingly diverse are the possible symptoms of Boeck’s disease.

Sarcoidosis is one of the so-called granulomatous diseases. Their classic feature is small, nodular tissue changes, These so-called granulomas are visible under the microscope. Why they form is so far unclear. However, medical experts suspect that hereditary factors associated with certain environmental factors can cause sarcoidosis.

Sarcoidosis: frequency

In Western Europe, approximately 40 to 50 out of 100,000 people are estimated to have sarcoidosis. The highest rates of morbidity occur in Sweden and Iceland, as well as in people of dark skinned skin in the United States.

The disease occurs usually between 20 and 40 years on. Women are slightly more affected than men.

Sometimes children also get sarcoidosis. A disease up to the fourth year of life is called “early childhood sarcoidosis” referred to (early onset sarcoidosis, EOS or blue syndrome). This rare form of disease is usually based on a genetic defect.

Sarcoidosis: symptoms

After the course, doctors differentiate acute and chronic sarcoidosis, The symptoms that occur depend on the type of disease, the severity of the disease (sarcoidosis stages: see below) and the organs affected.

Acute Sarcoidosis

Acute sarcoidosis accounts for only about 10 percent of all cases. The symptoms are quite sudden here. Mostly the lungs are affected. Typical are:

• fever and fatigue
• painful, at first red, later bluish nodules under the skin (Erythema nodosum)
• painful joint swelling and inflammation (arthritis)
• lymphadenopathy in the area of ​​both lungs

The Erythema nodosum occurs in sarcoidosis preferably on the front sides of the lower leg. The sites are very sensitive to pain. In severe cases, even the weight of clothing on the affected skin can cause pain. The skin nodules are formed by inflammation of the fatty tissue directly under the skin. Possible causes of this inflammation are, in addition to sarcoidosis, for example, also infectious diseases and autoimmune diseases.

The arthritis (Arthritis) in acute sarcoidosis usually affects the hocks. The patients are especially in pain when walking. It can also inflame several joints simultaneously (polyarthritis).

Swollen lymph nodes are found in acute sarcoidosis in the area of ​​the main bronchi and the large pulmonary vessels. This area is called lung hili. The swelling usually causes no complaints, but are clearly visible on the radiograph. Lymph node swelling is a very typical feature of Boeck’s disease. In people who otherwise have no symptoms, Boeck’s disease is often caused by these alone “Bihilar lymphadenopathy” detected in the x-ray.

The three symptoms of lymph node swelling in the lung area, erythema nodosum and arthritis are also referred to as the “triad of acute sarcoidosis” or Löfgren syndrome.

Chronic sarcoidosis

About 90 percent of all patients have one chronic sarcoidosis, Lungs and adjacent lymph nodes are most commonly affected. Some patients do not notice their condition. In others, the symptoms develop slowly and creepingly: increasing cough and Stress-related dyspnea, On the x-ray you can see the swollen lymph nodes on the so-called Lungenhili (bihilar lymphadenopathy). Other chronic sarcoidosis signs are:

• light fever
• weight loss
• fatigue
• Joint pain (arthritis)

In principle, Boeck’s disease can affect the entire body (extrapulmonary sarcoidosis). This results in different symptoms:

Sarcoidosis – Eyes: Different structures in the eye can be affected here. In many patients, for example, both the iris (iris) and the so-called ciliary body (on which the eye lens is suspended) are inflamed. This so-called iridocyclitis causes eye pain, which occurs especially in bright light.

Sarcoidosis – Skin: Chronic sarcoidosis in the area of ​​the skin triggers certain skin changes. These include the above-mentioned painful nodules under the skin (erythema nodosum). They form preferably on the front of the lower leg. In addition, bluish-purple skin discoloration may develop (lupus pernio), especially on the cheeks and nose.

Sarcoidosis – Heart: The heart may be severely affected by sarcoidosis. A slight infestation causes no complaints. Significant infestation can cause heart failure (heart failure) or cardiac arrhythmias. There is then the danger of serious complications!

Sarcoidosis – kidneys: If the kidneys are affected by sarcoidosis, they excrete more calcium with the urine. This favors the formation of kidney stones.

Sarcoidosis – Central nervous system (neurosarcoidosis): Sarcoidosis rarely attacks the central nervous system (brain and spinal cord). This often causes a failure of cranial nerves. If this concerns the facial nerve (facial nerve), the facial muscles are paralyzed as a result. This facial palsy usually occurs on one side. Neurosarcoidosis also often leads to meningitis (meningitis). Possible symptoms here are for example headache and vomiting.

Sarcoidosis – liver and spleen: Sarcoidosis of the liver and spleen usually causes no symptoms. The function of the two organs is practically not restricted. Only liver enzymes in the blood may be elevated as a result of sarcoidosis of the liver.

Heerfordt syndrome: In this special form of sarcoidosis, inflammation of the parotid glands and the eyes and swollen breast lymph nodes occur. In addition, a facial half can be paralyzed (facial paralysis).

Youth syndrome (Morbus Jüngling): The term refers to a chronic sarcoidosis in the area of ​​the bones. Very often the finger bones are affected.

The rare Early childhood sarcoidosis (EOS) triggers less obvious symptoms than the disease in adulthood. The possible signs range from fever, loss of appetite and tiredness to enlargement of the liver and spleen (hepatosplenomegaly).

Sarcoidosis: causes and risk factors

The exact cause of sarcoidosis is unclear. However, different factors probably play a role in the pathogenesis.

Sarcoidosis affects the lungs in most patients. Researchers therefore suspect that the Inhalation of harmful substances activates the immune system in the lungs. This could then trigger the formation of the Gewebeknötchen (granulomas). Possible harmful substances include, for example, pollen, viruses, bacteria, fungal spores, dust and chemicals.

In addition are genetic factors involved in the development of Boeck’s disease. Scientists have discovered genes in human genomes that are often altered in sarcoidosis. Some of these genetic changes (mutations) have been shown to increase the risk of sarcoidosis. Presumably, the affected genes have something to do with the function of the immune system. Their mutation is likely to make certain substances that are important to the immune system no longer or in a different form. This could trigger a miscommunication in the immune system – with the result that sarcoidosis arises.

Sarcoidosis: examinations and diagnosis

Diagnosing a sarcoidosis is often not easy. The symptoms can be so diverse that the disease often varies greatly from patient to patient. In addition, other diseases come as a cause for the various complaints. Therefore, several diagnostic steps are necessary until a sarcoidosis can be reliably detected.

The first contact for suspected cases of Boeck’s disease is usually one General practitioners, If necessary, he will refer the patient to a specialist, say one Specialist for lung diseases.

First conversation

The doctor will first record your medical history in a detailed conversation (anamnese). Typical questions of the doctor in anamnesis interview are for example:

• Do you suffer from irritating cough?
• What other complaints do you have (skin changes, fever, etc.)?
• Since when do the symptoms exist?
• Have you had such symptoms before?
• Has an X-ray of the lungs been made lately?
• Was or is there any lung disease in your family?

Physical examination

After the anamnesis interview, a physical examination follows. The focus is on the lungs and skin. These two organs are most frequently affected by Boeck’s disease.

The doctor will be about yours Listen to the ribcage and tap off, This provides him with clues to possible changes in the lungs.

any skin lesions the doctor will take a closer look. If necessary, he also takes a tissue sample (biopsy) to examine under the microscope. In sarcoidosis, the sample shows the inflammatory tissue nodules (granulomas).

X-ray

Sarcoidosis almost always affects the lungs and lymph nodes in the lung area. This can be ascertained by an X-ray examination of the chest (chest X-ray): In an X-ray of the lungs, in Boeck’s disease, among other things, enlarged lymph nodes in the area of ​​the main bronchi and the large pulmonary vessels (bihilar lymphadenopathy).

Based on the radiographic findings, the doctor can also determine at what stage the sarcoidosis is located. This stage influences the prognosis:

stage	description
Type 0	Sarcoidosis signs outside the thorax, but no discernible change in the lungs.
Type I	Swelling of the lymph nodes to the Lungenhili (bihilar lymphadenopathy). The lung tissue itself shows no abnormalities. Spontaneous cure rate about 70 percent.
Type II	Swelling of the lymph nodes to the Lungenhili and Lung infection. Spontaneous cure rate about 50 percent.
Type III	No swelling of the lymph nodes to the Lungenhili, but just Lung infection. Spontaneous cure rate about 20 percent.
Type IV	X-ray pulmonary fibrosis discernible (transformation of inflamed lung tissue into scarred connective tissue). Pulmonary function is thereby irreversibly restricted. A complete healing is no longer possible.

Lung function test

Sarcoidosis of the lung can severely restrict lung function. If, for example, lung tissue has hardened as a result of the disease (pulmonary fibrosis), it is less distensible when inhaled. This can be clearly determined with a lung function test.

Lung reflection & biopsy

In the lung (bronchoscopy), the doctor introduces a flexible, thin tube into the lungs. At its top is a small camera attached. So the doctor can examine the lung tissue directly.

In addition, tiny instruments can be inserted through the tube to take a sample of the lung tissue (biopsy). This is then examined in the laboratory for the typical granulomas.

blood test

Blood tests are less likely to diagnose sarcoidosis. Different blood counts help to assess the course of the disease. In sarcoidosis patients, for example, a particular enzyme in the blood is elevated, the so-called Angiotensin Converting Enzyme (ACE), From the level of the measured value, the doctor can estimate how active the disease is. A high ACE value indicates high disease activity. If the sarcoidosis therapy strikes or the disease subsides by itself, the ACE decreases in the blood.

Besides, too inflammatory markers such as the erythrocyte sedimentation rate (BSG) or the C-reactive protein (CRP) may be elevated. This generally indicates inflammatory reactions in the body – in sarcoidosis patients, therefore, an increased disease activity.

Further investigations

Since sarcoidosis can affect virtually all organs of the body, further examinations are necessary depending on the symptoms. Some examples:

If the doctor suspects kidney involvement, he determines that Kidney levels in the blood, Besides, he can use the kidneys Ultrasonic (Sonography).

The heart function can be combined with a electrocardiogram (ECG). With the cardiac ultrasound (Echocardiography) the doctor can check heart size and function.

If there is a suspicion of neurosarcoidosis, the doctor may Sample of cerebrospinal fluid remove (CSF) and let analyze in the laboratory.

If sarcoidosis (presumably) affects the eyes, one can Examination by the ophthalmologist to be necessary.

With a Computed tomography (CT) The organs of the thorax can be displayed in much greater detail than in an X-ray examination. This allows more accurate assessment of tissue changes. Above all, however, CT helps to rule out other illnesses as the cause of the symptoms. These include, for example, lung cancer and tuberculosis.

Sarcoidosis: treatment

Sarcoidosis does not necessarily have to be treated. It can heal spontaneously. This is especially true for acute sarcoidosis. Even chronic sarcoidosis can heal on its own. But that depends a lot on the disease stage. The higher the stage, the lower the self-healing rates.

In addition to the course, the severity of the symptoms also influences the decision: Treatment – yes or no? If the symptoms are not severe, it can often be waited and the further course can be checked regularly. However, if you have severe discomfort, loss of lung function, and / or critical organs such as the heart, kidneys, or central nervous system, sarcoidosis should be treated. The disease can, for example, greatly increase the level of calcium in the blood. This so-called hypercalcaemia can cause kidney stones and damage the kidneys. If the heart is affected, dangerous cardiac arrhythmias can occur.

How is Sarcoidosis treated?

The treatment is based on the symptoms. If at acute sarcoidosis Violent symptoms occur primarily so-called non-steroidal anti-inflammatory drugs (NSAIDs) used. These include, for example, acetylsalicylic acid and ibuprofen. They reduce fever, slightly anti-inflammatory and analgesic. In case of severe discomfort can be too short cortisone (“Glucocorticoids” such as prednisolone) are used. It is very effective against inflammation.

at chronic sarcoidosis is cortisone the therapy of choice. It is usually applied over several months. Then the dose is slowly reduced again (“tapering” of the therapy).

Many patients are afraid of the side effects of cortisone. Thus, the active ingredient may favor bone loss (osteoporosis), among other things. Such side effects occur especially with prolonged and / or higher doses of application. The doctor will take this into account when planning therapy. He will choose for each sarcoidosis patient a dosage and duration of therapy that are as low as possible, but at the same time bring about a sufficient effect. Patients should follow these recommendations exactly.

In severe cases of sarcoidosis, treatment with other medicines may be necessary. For example, some patients receive Methotrexate or azathioprine, Both active ingredients are among the so-called immunosuppressants, They effectively suppress the immune system (other than cortisone). Thus, the inflammatory reaction arrives at a sarcoidosis to a halt.

Sometimes the doctor prescribes too chloroquine, This ingredient is normally used for the prevention and treatment of malaria. It is suitable for sarcoidosis treatment because, like immunosuppressants, it can inhibit immune reactions.

In type IV of chronic sarcoidosis (irreversible pulmonary fibrosis) may require further therapy. Depending on the severity of the respiratory distress, the doctor may prescribe other medications (such as bronchodilating substances) or oxygen therapy. Rarely, the lungs are so severely damaged that they can no longer supply the body with sufficient oxygen. Affected then helps only a lung transplantation.

Sarcoidosis: disease course and prognosis

The prognosis of sarcoidosis depends primarily on whether it is an acute or chronic form:

The Prognosis of acute sarcoidosis is good in most cases. In about 95 percent of patients, the disease heals spontaneously and without treatment within a few months.

Also at thechronic sarcoidosis spontaneous healing is possible, but less common than in the acute form of the disease. Self-healing rates depend on the stage of the disease: they are best in early stages. The more advanced the disease is, the worse the prognosis – even with therapy. About half of all patients with chronic sarcoidosis sustained permanent lung damage. However, they are often not very pronounced.

Overall, considered applies to acute and chronic sarcoidosis: In 20 to 30 percent of all patients, pulmonary function remains permanently limited. About ten percent of patients even develop pulmonary fibrosis, which means that inflamed lung tissue transforms into scarred connective tissue and thus loses its function.

About five percent of sarcoidosisPatients die from complications such as sudden cardiac death or completely impaired lung function (terminal pulmonary fibrosis).

Additional information

Books:

• Living better despite sarcoidosis (Sigi Nesterenko, Rainer Bloch Verlag, 2010)

Guideline:

• Guideline “Sarcoidosis in childhood and adolescence” of the German Society for Pediatric Rheumatology and the German Association for Pediatric and Adolescent Medicine (2013)

Self-help:

• German Sarcoidosis Association of nonprofit e.V.
• Sarcoidosis network e.V.