Sarcoidosis: Information & sarcoidosis specialists

Sarcoidosis - also known as Boeck's disease or granulomatosis - is a rare inflammatory disease. Accumulations of small granulomas can form in any organ of the body. The granulomas consist of a granular form of connective tissue known as granulation tissue. It is normally formed as part of the wound healing process.

Here you will find all the important information and highly qualified sarcoidosis specialists.

What is sarcoidosis?

In general, a distinction is made between the acute and chronic form of sarcoidosis. Most people affected, around 95% of cases, suffer from the chronic form. The acute form, also known as Löfgren's syndrome, mainly affects young women.

The disease is caused by a dysregulation of the immune system or an increased immune response to inflammatory processes. This leads to the formation of granulomas. For this reason, sarcoidosis is a granulomatous autoimmune disease.

The causes of the increased immune response are still unknown. A genetic predisposition is assumed due to the observed familial clustering. If certain environmental factors are added, the immune system is activated and thus triggers granuloma formation.

The risk factors under discussion include inhaled pollutants such as

• chemicals or particulate matter,
• pathogens and
• allergenic substances.

In addition, sarcoidosis occurs more frequently in nursing staff for reasons that are still unknown.

Sarcoidosis usually forms granulomas in the lungs © designua | AdobeStock

Sarcoidosis usually manifests itself between the ages of 20 and 40. In rare cases, children and infants also develop the disease (early childhood sarcoidosis). Early childhood sarcoidosis (also known as early onset sarcoidosis or Blau syndrome) manifests itself before the age of 5. It is due to a genetic defect.

In principle, Boeck's disease can affect any organ, as it is a systemic disease. In 95% of cases , however, nodular neoplasms manifest in the lungs (pulmonary manifestation).

In addition, sarcoidosis often affects the following organs outside the lungs (extrapulmonary manifestations):

• the skin (15.9 percent),
• the eyes (11.8 percent),
• the liver (11.5 percent),
• the spleen (6.7 percent),
• the nervous system (4.9 percent),
• (ear) salivary glands (3.9 percent),
• bone marrow (3.9 percent),
• Throat, nose, ears (3.0 percent),
• heart (2.3 percent),
• kidney, bones, muscles (0.4 to 0.7 percent).

What are the symptoms of sarcoidosis?

In general, the usual general symptoms of inflammatory diseases occur, such as

• general feeling of illness,
• fatigue,
• fever,
• aching limbs and
• joint inflammation

occur. In addition, the symptoms associated with the disease vary greatly. They depend on the respective patterns of infestation and types of disease.

Acute sarcoidosis

Acute sarcoidosis is characterized by the typical triad of symptoms consisting of

• Arthritis,
• erythema nodosum and
• bihilar lymphadenopathy

characterized.

Erythema nodosum is characterized by painful, inflammatory changes in the fatty tissue under the skin. It manifests itself as reddish-bluish papules. The affected areas of skin are very sensitive to pain. Typically, erythema nodosum manifests itself on the extensor sides of the lower legs.

Arthritis is the inflammation of one or more joints(polyarthritis). In many cases of acute sarcoidosis, the ankle joint is affected. Ankle arthritis in turn causes pain when walking.

Bihilar lymphadenopathy is characterized by swelling of the lymph nodes on both sides of the pulmonary hilus. The pulmonary hilus is the area of the large pulmonary vessels and main bronchi (trachea branching). Other symptoms of acute sarcoidosis are fever and cough.

Chronic sarcoidosis

At the beginning of the disease, chronic sarcoidosis is asymptomatic in 50 percent of cases. The diagnosis is therefore often made by chance during a chest X-ray examination.

A characteristic feature is the discrepancy between the comparatively good subjective condition of the affected patient and the pronounced objective findings such as the chest X-ray.

In 95 percent of cases, chronic sarcoidosis manifests itself in the lungs and the lymph nodes in the chest area. In the case of pulmonary manifestations, a chesty cough and dyspnoea on exertion (dyspnoea on exertion) may occur later in the course of the disease.

General signs of the disease such as

• mild fever,
• weight loss,
• fatigue and
• arthritis

are further signs of chronic sarcoidosis.

The chronic form is divided into stages (type 0 to IV) according to the chest X-ray findings, which provide an indication of the pattern of infestation:

• Type 0: There are signs of the disease outside the chest, with no visible involvement of the lungs
• Type I: bihilar lymphadenopathy (= enlargement of lymph nodes on both sides of the pulmonary hilus) without visible involvement of the lungs
• Type II: bihilar lymphadenopathy with lung involvement
• Type III: lung involvement without bihilar lymphadenopathy
• Type IV: pulmonary fibrosis (= remodeling of the lung tissue into connective tissue) with irreversible impairment of lung function

Early onset sarcoidosis (EOS)

EOS typically manifests itself before the age of 5 in the form of an attack on

• joints (arthritis),
• the central vascular membrane in the eye(uveitis) and
• the skin (exanthema).

Further symptoms are

• Fatigue,
• anorexia (loss of appetite),
• fever and
• enlargement of the liver and spleen.

Extrapulmonary manifestations of Boeck's disease

If sarcoidosis does not occur in the lungs, it is referred to as an extrapulmonary manifestation. These affect, among other things

1. the skin:

• by reddish-brown papules of varying size,
• extensive livid discoloration of the skin on the cheeks and nose (lupus pernio),
• yellow-brownish plaques in the area of existing scars (cicatricial sarcoidosis),
• subcutaneous blue-reddish nodules in the area of the extensor sides of the lower legs, which react painfully to pressure (erythema nodosum).

2. the eyes:

• Due to inflammation of the iris and the ciliary body attached to the lens of the eye (iridocyclitis),
• inflammation of the middle vascular membrane, known as the uvea (uveitis),
• due to calcium deposits in the conjunctiva and cornea,
• infestation of the lacrimal glands.

3. the parotid gland:

• Inflammation of the parotid gland (parotitis) in combination with uveitis and facial nerve palsy(paralysis of the facial nerves) in Heerfordt's syndrome.

4. the bones:

• Due to cystic changes on the phalanges of the fingers(ostitis multiplex cystoides or Jüngling syndrome).

5. the nervous system (neurosarcoidosis):

• Due to facial nerve palsy,
• Diabetes insipidus (accompanied by greatly increased urination and thirst),
• anterior pituitary insufficiency (failure of the endocrine functions of part of the pituitary gland),
• granulomatous meningitis (inflammation of the meninges).

6. the heart (cardiac sarcoidosis):

• due to absence of symptoms with mild infestation,
• by arrhythmia, left ventricular failure, pericardial effusion (accumulation of fluid in the pericardium) in the case of pronounced infestation.

By NHLBI authors. - National Heart, Lung, and Blood Institute: DCI Home: Lung Diseases: Sarcoidosis: Signs & Symptoms Retrieved on May 9, 2009, Public Domain, https://commons.wikimedia.org/w/index.php?curid=6737947

How is sarcoidosis treated?

The therapeutic measures depend on the type of disease present and the pattern of infection.

The standard treatment for sarcoidosis is corticosteroids (cortisone). They are associated with considerable side effects. For this reason, sarcoidosis types with a high spontaneous self-healing rate are not treated in this way. These include acute sarcoidosis and chronic sarcoidosis types 0 and I.

Treatment with corticosteroids is recommended from type II onwards if lung function is impaired. An elevated calcium level increases the risk of urinary stone formation. Treatment is therefore also used in the presence of hypercalcemia. Urinary stones can cause kidney damage.

There is also an indication for the use of corticosteroids in the case of involvement of the

• eyes,
• liver,
• nervous system,
• myocardium (heart muscle) and
• skin and
• in the case of severe general symptoms in acute sarcoidosis.

If corticosteroids are not effective enough, a combination of prednisolone and immunosuppressants is indicated. In addition to systemic corticosteroids, local steroid therapy is recommended for uveitis and skin lesions.

Arthritic pain in acute sarcoidosis can be treated with NSAIDs (= non-steroidal anti-inflammatory drugs) such as ibuprofen.

Depending on the possible involvement of other organs , additional therapeutic measures may be indicated. For example, cardiac sarcoidosis may require a pacemaker or defibrillator.

In the case of advanced involvement of an organ, transplantation of the organ in question is indicated as a last resort in the final stage.

What are the chances of recovery from sarcoidosis?

In 95 percent of cases, acute sarcoidosis heals spontaneously within 2 years. It therefore has a very good prognosis.

In chronic sarcoidosis, the chances of recovery and prognosis depend on the stage of the disease. Type 1 sarcoidosis still shows a spontaneous healing rate of 70 percent within 1 to 3 years. For type II, this is around 50 percent and for type III around 20 percent.

About one fifth of patients have a permanent impairment of lung function. The mortality rate is 5 percent.

Sarcoidosis specialists - training and further education

If you have lung complaints, a lung specialist, a so-called pulmonologist, is the right contact person for you.

After studying medicine, they have completed training as a specialist in internal medicine and pneumology. This lasts a total of 72 months and covers all relevant diagnostic and therapeutic procedures for the treatment of lung diseases.