Sarcoidosis

Definition

Sarcoidosis is a disease in which very small nodules, so-called granulomas, form in the connective tissue and organ tissue of the affected patients. These granulomas occur most frequently in the lymph nodes of the lung and in the actual lung tissue, but in principle they can affect any organ. The granulomas form as an expression of an immune reaction of the body and can impair the function of the affected organ (sarcoidosis).

Symptoms of Sarcoidosis

Sarcoidosis is a systemic disease of the connective tissue, in which nodules form in the connective tissue. Mostly the connective tissue of the lung or lymph nodes is affected, but nevertheless sarcoidosis can occur in all organs and cause different symptoms depending on the organ. In about 30% of cases, sarcoidosis is acute.

The symptoms of acute sarcoidosis are a general feeling of illness with high fever, pain in the joints (often ankle) and erythema nodosum, a painful inflammation of the subcutaneous fatty tissue, which occurs mainly in the shins. X-rays show enlarged lymph nodes at the root of the lung on both sides (so-called bihilary lymphadenopathy). The combination of these symptoms is known as Löfgren’s syndrome.

Young women are particularly affected, and the incidence of the disease increases in spring and autumn. There may also be a dry cough and shortness of breath during physical exertion. Some patients also complain of nausea, nausea, anxiety or pressure in the chest or weight loss.

Acute sarcoidosis does not necessarily have to be Löfgren’s syndrome, however; it can occur in any other organ. The combination of fever, anterior choroidal inflammation of the eye, salivary gland inflammation and hemiplegia (facial nerve palsy) is also a special form of acute sarcoidosis and is known as Heerfordt’s syndrome. The acute form of sarcoidosis has a good prognosis: in 80-90% of cases it heals without consequences.

In about 70% of the cases the sarcoidosis proceeds in a chronic form. The symptoms are irritable cough that increases over several months, fatigue and weakness, slight fever, shortness of breath during physical exertion and weight loss. Only sometimes the patients also have pain in the ankle joint.

However, the chronic form of sarcoidosis can also progress without these symptoms and then be discovered only by chance, especially if only the lungs or lymph nodes are affected. In chronic sarcoidosis, other organs are also affected more frequently than in the acute form. The cure rates for chronic sarcoidosis are lower than for the acute form.

In some cases, sarcoidosis leads to itching. This can occur all over the body, frequent sites are for example the face or the scalp. The itching often occurs together with a burning or uncomfortable feeling and redness.

It can lead to the formation of nodules or dents on the skin, which are often reddened, sometimes slightly bluish or brownish. Itching may also occur in the eyes. In rare cases, larger wounds may also occur.

You can read more information on this topic at Itchy skin – These are the causesSarcoidosis typically causes an enlargement of the lymph nodes. This is also one of the typical symptoms leading to a diagnosis. The swelling of the lymph nodes is mainly found in the area of the lung root and can be shown, for example, in an X-ray of the chest.

In addition, there is often an enlargement of the lymph nodes in the area of the groin. This is usually painless and can be palpated by the patient himself. In 90% of the cases, the lungs of someone suffering from sarcoidosis are affected alone or likewise.

The chronic form is typically characterized by a dry, irritable cough, coughing fits, shortness of breath during physical exertion and chest pain. These symptoms begin insidiously and increase over the course of the disease. Other symptoms of chronic pulmonary sarcoidosis include mild fever, fatigue, weight loss and joint pain.

However, chronic pulmonary sarcoidosis can go on for a long time without symptoms. The acute form of the disease very often affects the lungs and also manifests itself with dry, irritable cough, coughing fits, possibly shortness of breath and chest pain. Whistling respiratory symptoms may also occur.

Acute sarcoidosis of the lungs often occurs as Löfgren’s syndrome, in which the symptoms in the lungs are accompanied by high fever, joint pain and erythema nodosum (inflammation of the subcutaneous fatty tissue, especially the shins). Symptoms on the joints are possible in both acute and chronic sarcoidosis.Löfgren’s syndrome, a special form of acute sarcoidosis, is characterized by acute joint inflammation, among other things. In most cases, the ankle joints are affected, but other joints may also be affected.

Sarcoidosis can trigger inflammation in various parts of a joint: the joint capsule or synovial fluid, but also tendons or tendon sheaths of a joint can be affected by sarcoidosis and cause symptoms in the joints. Symptoms can occur in one or more joints. The affected joints are swollen, painful, possibly overheated and their function is restricted.

In rare cases, sarcoidosis affects the skeletal system. Especially the phalanges are affected: The bone substance is transformed into vesicular distensions (Jüngling syndrome). Bone sarcoidosis is often a late manifestation of an already known chronic sarcoidosis of other organ systems.

Sarcoidosis can, but need not, cause symptoms on the skin. Are you more interested in this topic? You can read more detailed information in our next article: Sarcoidosis of the skin – causes, symptoms and therapy

  • Erythema nodosum: Erythema nodosum is a typical symptom (especially in Löfgren’s syndrome, a special form of acute sarcoidosis).

    In erythema nodosum, nodules (nodus = nodules) are formed in the fatty tissue of the subcutis. This is manifested on the skin by reddish-purple to yellowish-green blurred spots and nodules. These areas are slightly raised and sensitive to pressure and mainly affect shins, knees and ankle joints.

    The arms and buttocks are less frequently affected.

  • Granulomas: Granulomas are painful, palpable skin nodules with red-blue discoloration of the affected skin area. In small-nodular sarcoidosis, granulomas occur mainly on the face, but they can spread everywhere. Typical here is a rapid appearance and in many cases disappearance within weeks.

    In nodular sarcoidosis, the granulomas mainly affect the extremities.

  • Lupus pernio: Lupus pernio describes the chronic form of skin sarcoidosis. The symptoms are bluish swellings on the face, which mainly affect the cheeks, nose and lips. The hands are also affected.
  • Scar sarcoidosis: Sarcoidosis can cause discoloration and nodules on old scars.

In renal sarcoidosis, nodules form in the kidney tissue, which represent a constant inflammatory stimulus.

This inflammatory stimulus triggers a defensive reaction of the immune system and more calcitriol is released. Calcitriol is a hormone of the calcium metabolism, which gets confused by the increased release. Symptoms of chronic inflammation are frequent urination and blood in the urine.

Due to the disturbed calcium metabolism, there is more calcium in the urine, which promotes the development of kidney stones. Symptoms are colics of the kidneys and ureter. Due to the increased calcium content of the urine, the renal pelvis can be filled with calcium stones (nephrocalcinosis), which leads to renal insufficiency.

In renal sarcoidosis, cortisone therapy can prevent renal failure. In sarcoidosis of the heart, nodules form in the heart muscle tissue. Mild infestation of the heart causes no problems, while severe infestation has serious complications.

If the nodules form in the areas of the heart that belong to the cardiac conduction system, cardiac arrhythmia is the result. Symptoms are, for example, an irregular heartbeat or shortness of breath under stress. The nodules in the heart muscle tissue can cause inflammation of the heart muscle, which leads to heart weakness and heart failure.

Sarcoidosis of the heart can lead to sudden cardiac death, even if no symptoms have occurred previously. Sarcoidosis can lead to various symptoms in the eyes, which often occur in both eyes at the same time. These include nodules that can appear on the iris, i.e. the iris, and inflammation of the uvea, the middle layer of the eye.

Especially the latter are very typical for eye involvement in sarcoidosis. The eyes also often feel very dry and are more sensitive to light. A check-up by an ophthalmologist is very important when diagnosing sarcoidosis, as the optic nerve can also be affected. Under certain circumstances, this can lead to permanent impairment of vision.