Internal Medicine

Sarcoid

definition

Sarcoid is a disease in which very small nodules, so-called granulomas, form in the connective tissue and organ tissue of the affected patient.

These granulomas occur most frequently in the lymph nodes of the lungs and in the actual lung tissue, but can in principle 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 (Sarcoid).

Symptoms of Sarcoid

Sarcoid is a systemic connective tissue disease in which nodules develop in the connective tissue. Mostly the connective tissue of the lungs or the lymph nodes is affected, but sarcoid can occur in all organs and cause different symptoms depending on the situation.

In about 30% of the cases the sarcoid is acute. The symptoms of acute sarcoidosis are a general feeling of illness with a high fever, pain in the joints (often ankle) and erythema nodosum, a painful inflammation of the subcutaneous fat that occurs mainly on the shins. The x-ray shows enlarged lymph nodes at the root of the lung on both sides (so-called bihilar lymphadenopathy). The combination of these symptoms is known as Löfgren's syndrome.

Young women in particular are affected; the cases of the disease increase in spring and autumn. You may also experience a dry cough and shortness of breath during physical exertion. Some patients also complain of nausea, nausea, chest tightness or pressure, or weight loss. Acute sarcoid does not have to run as Löfgren's syndrome, it can occur in any other organ.

The combination of fever, anterior choroidal inflammation of the eye, inflammation of the salivary glands and unilateral facial paralysis (Facial palsy) is also a special form of acute sarcoid and is known as Heerfordt's syndrome. The acute form of sarcoid has a good prognosis: In 80-90% of cases it heals without consequences.

In approx. 70% of the cases the sarcoid is chronic. The symptoms are a dry cough that increases over several months, fatigue and weakness, a slight fever, shortness of breath during exercise and weight loss. Only sometimes do the patients also have pain in the ankle.

The chronic form of sarcoid can also take place without these symptoms and then only be discovered by chance, especially if only the lungs or lymph nodes are affected. Other organs are more often affected in chronic sarcoid than in the acute form. The cure rates for chronic sarcoidosis are lower than for the acute form.

Read more on this topic at: Symptoms of Sarcoid

Sarcoid itching

Sarcoid in some cases leads to the appearance of itching. This can occur all over the body; common areas are, for example, the face or scalp. Often the itching comes with a burning or uncomfortable feeling and redness.

Nodules or dents may form on the skin, which are often reddened, sometimes slightly bluish or brownish. Itching of the eyes can also occur. In rare cases, larger wounds can also occur.

You can read more information on this topic at: Skin itches - these are the causes

This is how the lymph nodes change in sarcoid

Sarcoid is typically an enlarged lymph node. This is also one of the typical symptoms leading to the 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 groin area. This is usually painless and can also be felt by those affected.

Symptoms of sarcoid on the lungs

In 90% of cases, someone with sarcoid will affect the lungs alone or as well. The chronic form is typically shown by a dry, tickly cough, coughing fits, shortness of breath during physical exertion and chest pain. These symptoms begin slowly and increase as the disease progresses.

Other symptoms of chronic pulmonary sarcoidosis are mild fever, fatigue, weight loss, and joint pain. However, chronic sarcoid disease of the lungs can last for a long time without symptoms.

The acute form very often affects the lungs and also manifests itself with dry, irritating cough, coughing fits, possibly shortness of breath and pain in the chest. In addition, wheezing breathing symptoms may occur.

Acute sarcoid of the lungs occurs in many cases as Löfgren's syndrome, the symptoms of the lungs are then accompanied by high fever, joint pain and erythema nodosum (Inflammation of the subcutaneous fatty tissue, especially Shins).

Symptoms of sarcoid on the joints

Symptoms in the joints are possible in both acute and chronic sarcoid. Löfgren's syndrome, a special form of acute sarcoid, manifests itself, among other things, in acute joint inflammation. In most cases the ankle joints are affected, but other joints are also possible.

Sarcoid can trigger inflammation in different parts of a joint: the joint capsule or the synovial fluid, but also the tendons or tendon sheaths of a joint can be affected by sarcoid and cause symptoms in the joints. Symptoms can appear in one or more joints.

The affected joints are swollen, painful, possibly overheated and their function is restricted. In rare cases, sarcoid affects the skeletal system. The finger bones are particularly affected: the bone substance changes into vesicle-like swellings (Youth syndrome). Bone sarcoid is often a late manifestation of an already known chronic sarcoid of other organ systems.

Symptoms of sarcoid on the skin

Sarcoid can, but does not have to, cause symptoms in the skin.

Erythema nodosum: Erythema nodosum is a typical symptom (especially in Löfgren's syndrome, a special form of acute sarcoid). In the case of erythema nodosum, nodules form (nodus = node) in the fatty tissue of the subcutaneous tissue, this is noticeable on the skin through reddish-purple to yellowish-green blurred spots and lumps. These areas are slightly raised and sensitive to pressure and mainly affect the shins, knees and ankles. The arms and buttocks are less affected.
Granulomas: Granulomas are painful, palpable skin nodules with a red-bluish discoloration of the affected skin area. In small-nodular sarcoid, the granulomas occur mainly on the face, but they can spread anywhere. Typical here is a rapid onset and in many cases a disappearance within weeks. In nodular sarcoid, the extremities are particularly affected by the granulomas.
Lupus pernio: Lupus pernio describes the chronic form of skin sarcoid. Symptoms are bluish swellings on the face, mostly affecting the cheeks, nose, and lips. The hands are also affected.
Scar sarcoid: Sarcoid can cause discoloration and nodule formation on old scars.

Are you more interested in this topic? You can read more detailed information in our next article: Sarcoid of the skin - causes, symptoms and therapy

Symptoms of sarcoid on the kidneys

In renal sarcoid, nodules form in the kidney tissue, which are a constant inflammatory stimulus. This inflammatory stimulus sets in motion a defense reaction of the immune system and more calcitriol is released. Calcitriol is a calcium metabolism hormone that gets mixed up due to the increased secretion.

Symptoms of chronic inflammation include frequent urination and blood in the urine. The disturbed calcium metabolism means that there is more calcium in the urine, which promotes the development of kidney stones. Symptoms are kidney and ureter colic.

Due to the increased calcium content of the urine, the renal pelvis can become filled with calcium stones (Nephrocalcinosis), which leads to renal failure. Cortisone therapy can prevent renal failure in renal sarcoid.

Symptoms of sarcoid on the heart

In sarcoidosis of the heart, nodules form in the heart muscle tissue. A slight affliction of the heart does not cause problems; a severe affection has serious complications. If the nodules form in the areas of the heart that are part of the cardiac conduction system, cardiac arrhythmias are the result. Symptoms include an irregular heartbeat or shortness of breath during exercise.

Heart muscle inflammation can develop through the nodules in the heart muscle tissue, which leads to cardiac insufficiency and heart failure. Sarcoid of the heart can lead to sudden cardiac death even if no symptoms have previously occurred.

Symptoms around the eyes

Sarcoid can cause a variety of symptoms in the eyes, most of which appear 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 skin of the eye. The latter in particular 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 sarcoid, as the optic nerve can also be affected. Under certain circumstances, this can result in permanent visual impairment.

Sarcoid therapy

A causal therapy for sarcoid, i.e. a therapy that eliminates the cause of the disease, unfortunately does not yet exist. Therefore attempts are made to limit and alleviate the symptoms as far as possible in affected patients.

In Löfgren's syndrome, this means relieving in particular the pain caused by erythema nodosum and polyarthritis and thus also reducing the functional impairment associated with the pain. The therapy is also aimed at treating general symptoms such as fever and fatigue.

Therapy is usually started with drugs from the class of "non-steroidal anti-inflammatory drugs", these are drugs such as ibuprofen and diclofenac, which have both analgesic and anti-inflammatory effects. For many patients, however, such medication is not sufficient, so cortisone is often used. Cortisone should be used as long as necessary to get the acute inflammation under control.
In the chronic form of sarcoid, the anti-inflammatory effect of cortisone is also used; immunosuppressants are often used in long-term therapy.

What role does vitamin D play in the treatment of sarcoid?

The role of vitamin D in the treatment of sarcoid is complex, as the precise relationship between the vitamin and the development of the disease is not yet ultimately understood. In any case, the vitamin D and calcium levels should be checked regularly by the doctor.

An increased level of vitamin D can have a negative effect on the body and especially the kidney function in sarcoid. Therefore, any necessary prophylaxis for osteoporosis with vitamin D and calcium must be discussed with a doctor.

Which Doctor Treats Sarcoid?

Unfortunately, there is no specialist doctor who is specifically responsible for the disease of sarcoid. Sarcoid is a systemic disease, which means that it affects or can affect the entire body. It is therefore often advisable to base the choice of doctor on the symptoms.

For example, if the sarcoid is particularly conspicuous due to symptoms of the lungs, a pulmonologist, i.e. a pulmonologist, can be consulted. A dermatologist, i.e. a dermatologist, can also be helpful if the skin is affected.

There are also a few centers in Germany that focus specifically on sarcoid disease. In addition, an ophthalmologist should always check it, as sarcoidosis can often also affect the eyes.

How should you eat if you have sarcoid?

If you have sarcoid, it can be helpful to change or adjust your diet. The main focus here is on reducing inflammation in the body.

Eating a balanced diet, including fresh fruits and vegetables, is just as important as reducing fat. Therefore, lean meat and poultry should be eaten rather than red meat. The latter can promote the inflammatory processes in the body. The consumption of fish is also recommended for people with sarcoid.

Furthermore, you should ensure that you drink enough fluids, especially water. Hydration of the body is very important for strengthening the immune system. Magnesium can also have a supportive effect and is contained in corn, potatoes and bananas, for example. Ginger or turmeric are also recommended as they stimulate the body's immune system.

Caffeine, alcohol, and cigarettes should also be avoided as risk factors that can promote the progression of sarcoid disease.

You can read our next article on the subject here: Healthy eating

Can sarcoidosis be cured?

The healing of sarcoid is possible depending on the form of the sarcoid. A basic distinction is made between acute and chronic sarcoid.

Acute sarcoid, which often only affects the lungs, heals completely in around 90% of those affected. The so-called Löfgren syndrome, in which the skin and joints are affected in addition to the lungs, is part of acute sarcoid.

Chronic sarcoid, which occurs in around 2/3 of all cases, has different types. In addition to the symptoms in the lungs, other organs such as the skin or eyes are often affected. Here the healing rates are worse than with the acute form of sarcoid. Particularly in the case of an advanced infestation of the lungs with fibrosis, i.e. a conversion of the lung tissue into scar tissue, the healing rates are only approx. 20%.

Therefore, early therapy is very important to improve the chances of recovery. It should also be remembered that people with sarcoid disease are at increased risk of developing lung cancer.

Course and prognosis of sarcoid

Overall, the prognosis for sarcoid is relatively good, but it strongly depends on the stage of the disease in which the patient is.

In stage 1, in the acute course, 90% of the patients heal spontaneously, and in stage 2 there is still a relatively high rate of spontaneous healing. In stage 3, however, the use of glucocorticoids such as cortisone for therapy is recommended, and patients often have to stay in hospital for a very long time.

The course of the disease must be monitored very closely in each case; a pulmonary function test and blood tests, for example, are suitable control parameters.
The total mortality rate due to sarcoid and its consequences is estimated at around 5%.

Causes of Sarcoid

Many attempts have been made to elucidate the cause of sarcoid, but unfortunately so far in vain. Due to the increased occurrence of sarcoid in families and in identical twins, it has long been assumed that it is a genetic defect.
In 2005 a certain gene (the BTNL2 gene) discovered which in the event of a mutation, i.e. a change, increases the risk of developing sarcoidosis by at least 60%. The gene is on chromosome 6.

At the center of the disease (Sarcoid) is an abnormal and excessive immune reaction (immune system) of the body to a certain substance, a certain antigen, which is as yet unknown. Some bacteria, viruses and organic substances have already been discussed as triggers.

If such a substance gets into the body, the organism reacts excessively in some people and tries to protect itself against the substance. This takes place with the help of granuloma formation, i.e. the formation of nodules in which a kind of protective wall is built up around the substance from different cells so that it cannot spread any further.Certain cell types are involved in the construction of these granulomas, so-called epithelial cells and also lymphocytes (Sarcoid).

Not to be confused with sarcoid, the nodular appearance of the benign skin disease granuloma annulare. It manifests itself in growing, reddish, coarse papules that appear in a ring on the back of the foot and hand and over the joints.
Here you can find out more about: Granuloma annulare

Diagnosis of sarcoid

In addition to the physical examination, various imaging methods and laboratory tests are available for diagnosing sarcoid. A chest x-ray is taken as standard.

The other diagnostic methods are described in more detail below for the particularly interested reader:

A lung lavage (BAL, bronchoalveolar lavage) is carried out to examine the secretion obtained cytologically. In sarcoid, lymphocytic alveolitis is present (Inflammation of the alveoli with an increased number of lymphocytes) with increased CD4 / CD8 quotient: This means that although more T cells (Immune cells) are present, but their function is impaired.

It is also possible to take a biopsy of lymph nodes in the lungs or a biopsy of lung tissue as part of a lung specimen. The histological examination of the tissue shows non-caseating, epitheloid-cell granulomas (Connective tissue nodules) with Langhans giant cells and an edge wall of lymphocytes, monocytes and connective tissue cells.

The blood test for sarcoid shows increased inflammation and an increased rate of sedimentation (BSG). In addition, the values for antibodies and immunoglobulin G are increased.

If the kidneys are involved, calcium and calcitriol are increased; further tests include creatinine, urea, and a urine test to assess kidney function.

The lung function is examined using spirometry. ACE (Angiotensin converting enzymes) and S-IL-2R (soluble IL-2 receptor) are activity markers for lung diseases, they decrease with successful therapy of sarcoid.

Further examinations for sarcoidosis include an EKG to rule out cardiac arrhythmias, a visit to the ophthalmologist (Eye involvement?) and a tuberculin test (which is negative because of the impaired T cell function).

Read more on this topic at: Diagnosis of sarcoid

Stages of sarcoid

Sarcoid is divided into stages according to the x-ray findings:

Stage 0: no changes, but another organ has sarcoid.
Stage 1: biliary lymphadenopathy (Lymph nodes enlarged on both sides at the root of the lung), approx. 70% chance of spontaneous remission.
Stage 2: Stage 1 plus nodular changes in the lungs, approx. 40% chance of spontaneous remission.
Stage 3: lung involvement without enlargement of the lymph nodes.
Stage 4: permanent damage to the lungs with fibrotic remodeling (more connective tissue). It is also possible to visualize the lung involvement in a computer tomography.

More information is available here: The pulmonary fibrosis

What types of sarcoid are there?

Basically, there are two types of sarcoid: a chronic form that slowly creeps in and an acute form of sarcoid.

In the acute form of sarcoid with a sudden onset of the disease, a distinction is made between the so-called Löfgren syndrome (after the first descriptor Sven Halvar Löfgren) and Heerfordt-Waldenström syndrome.

Usually it is the chronic form of sarcoid (95%), only rarely the acute form of sarcoid (5%).

Acute form

The acute forms of sarcoidosis, Löfgren's syndrome and Heerfordt-Waldenström syndrome, on the other hand, are typically characterized by very specific symptom complexes.

Löfgren's Syndrome

In Löfgren's syndrome (Sarcoid) one speaks of so-called symptom triad, i.e. a combination of three different symptoms:

Bihilial lymphadenopathy
Erythema nodosum
Polyarthritis

A biliary lymphadenopathy is a pathological swelling of the lung lymph nodes in the area of the lung root (hilus) in both lungs.

An erythema nodosum is also known as a nodular rose and presents as multiple granuloma formation (so pilling) in the subcutaneous fatty tissue. Ankle joints, knees and lower legs are mostly affected. Since the nodules are the expression of an inflammatory reaction, they are usually painful, reddish in color and occasionally accompanied by a fever and a general feeling of illness.

Polyarthritis is an inflammatory joint disease that affects at least five or more joints, usually symmetrically opposite joints.
A few weeks before the development of erythema nodosum and polyarthritis, flu-like symptoms such as a dry cough, impaired physical performance and a general feeling of illness occur.

At this point you can also read our main page on Löfgren Syndrome: Löfgren syndrome - what's behind it?

Heerfordt-Waldenström syndrome

The very rare Heerfordt-Waldenström syndrome, like Löfgren syndrome, is characterized by a certain complex of symptoms:

Fever,
Parotid swelling
anterior uveitis and
Fascial palsy.

Parotid swelling is swelling of the parotid gland, anterior uveitis is inflammation of the iris and ciliary muscles in the eye, and fascial palsy is a functional disorder of the facial nerve with paralysis of the facial muscles in the face area.

Without therapy, the acute symptoms last for a few weeks and then spontaneously regress completely.

Frequency of sarcoid

People between the ages of 20 and 40 are most frequently affected by sarcoid, in Germany it is assumed that there are 15-30 affected per 100,000 inhabitants. In some other countries, such as Sweden and Iceland, the proportion of new cases is significantly higher, namely around 60 cases per 100,000 inhabitants.

In Europe one speaks of a north-south divide, namely in Spain the rate of new cases of sarcoid is much lower and is around 2: 100,000. This is probably due to a genetic cause of the disease. The dark-skinned population of America is also affected more often than other population groups.

Sarcoid is generally widespread worldwide; sarcoid occurs slightly more often in women than in men. Since many diseases are asymptomatic, it is difficult to give exact numbers. In young people, sarcoid is the second most common lung disease after bronchial asthma.

What is the life expectancy with sarcoidosis?

There are no exact figures for the life expectancy with sarcoid, as this depends strongly on the severity of the disease and its symptoms.

For example, if it is an acute sarcoid, the chances of spontaneous healing are very good. Although the risk of certain types of cancer is increased, apart from this, it can be assumed that life expectancy is normal and unimpaired.

The life expectancy with sarcoid at a chronic advanced stage is correspondingly lower.