Types of Sarcoidosis

There are many types of sarcoidosis, a rare condition associated with the formation of granulomas, or clumps of immune cells, in various organs and tissues. The formation of such granulomas can prevent the normal function of organs, and cause permanent damage if left untreated.

The different types of sarcoidosis are usually identified by the part of the body they affect.

In fact, sarcoidosis can affect almost any body part. But depending on where it occurs, patients can experience very different symptoms and require different treatment methods. Additionally, a patient may experience more than one type of sarcoidosis at once. Some of the most common types are discussed here.

Pulmonary sarcoidosis

The most common form of the condition, pulmonary sarcoidosis refers to sarcoidosis affecting the lungs. The majority of people with sarcoidosis, about 90%, experience some level of lung involvement.

Granulomas can affect normal lung function and lead to shortness of breath, a persistent cough, chest pain, and wheezing.

In rare cases, the inflammation caused by sarcoidosis may lead to scarring and permanent damage to the lungs, causing complications such as bronchiectasis — a condition in which the airways become abnormally widened. Bronchiectasis leads to the buildup of mucus in the lungs, and increases the risk of infections.

Ocular sarcoidosis

When the condition affects the eyes or the surrounding structures, which occurs in up to three-quarters of patients, it is called ocular sarcoidosis. Individual symptoms vary depending on which parts of the eye are affected, but the most common is uveitis, or inflammation of the iris and surrounding tissues.

Uveitis involves redness, swelling, and pain in the eyes, and can cause vision abnormalities such as floaters. If left untreated, ocular sarcoidosis can lead to complications that can cause vision loss, such as glaucoma and cataracts. Glaucoma is a disease that damages the optic nerve, while cataracts cause cloudy patches in the eye and impair vision.

Neurosarcoidosis

Granulomas developing in and around the nervous system lead to the disease being referred to as neurosarcoidosis. This type affects about 5-15% of sarcoidosis patients, half of whom also have sarcoidosis in other organs and tissues.

Depending on the nerves affected, neurosarcoidosis can have a wide variety of symptoms, which can be extremely debilitating in some cases.

The condition most commonly affects hormone-producing regions of the brain, such as the hypothalamus and the pituitary gland. This can lead to problems such as a change in menstrual cycles in female patients, diabetes, and excessive hormone production.

Neurosarcoidosis also can affect the peripheral nerves and lead to problems with motor function, such as muscle weakness or numbness.

Cardiac sarcoidosis

When sarcoidosis affects the heart, the condition is referred to as cardiac sarcoidosis. While this type occurs in about a third of sarcoidosis patients, only about 5% will experience symptoms.

In cardiac sarcoidosis, the granulomas disrupt the electrical signals that trigger the contraction of the heart muscle. Thus, the most common manifestation of this type is an irregular heartbeat, or arrhythmia, which can feel like palpitations.

Heart failure — when the heart is unable to pump enough blood to maintain essential body functions — may occur in patients at advanced stages of cardiac sarcoidosis. The most common symptoms include a cough, shortness of breath, fatigue, and leg swelling.

Musculoskeletal sarcoidosis

Musculoskeletal sarcoidosis refers to the rare condition affecting the bones, muscles, or joints.

Sarcoidosis can cause inflammation in the bones, but this normally does not cause symptoms. However, some medications used to control the abnormal immune reactions can cause the bones to become more fragile and prone to breaking (osteoporosis).

Inflammation of the joints can cause arthritis-like symptoms, which can restrict a patient’s range of movement and cause pain. Most commonly, the feet, ankles, and knees are affected. Chronic joint problems are rare in sarcoidosis, but patients can experience acute symptoms.

Muscle sarcoidosis is rare and usually asymptomatic. In about 1% of cases, symptoms may occur. These include chronic muscle weakness, due to muscle compression by the granulomas, and muscle nodules.

Cutaneous sarcoidosis

In cutaneous sarcoidosis, a type that occurs in about 20-35% of patients, granulomas form in the skin, although some types of nonspecific skin lesions can form without granulomas.

The classification of granuloma-containing skin lesions depends on their location and appearance. Erythema nodosum, the most common skin condition in sarcoidosis, is characterized by acute painful red lumps on the lower legs or arms that are often accompanied by joint pain, fatigue, and fever.

Lupus pernio, meanwhile, is a chronic condition, usually appearing as painful blue lesions or discoloration on the face, ears, fingers, or toes. This also can manifest as plaque-like lesions that appear as purple-red or brown, and usually occur on the face, scalp, and back.

Renal sarcoidosis

About 15-40% of patients will experience renal sarcoidosis, which affects the kidneys. If left untreated, the inflammation caused by the granulomas can cause tissue scarring and permanent damage, leading to kidney failure. Up to 10% of patients with renal involvement will require permanent dialysis or a kidney transplant over the course of their disease.

Changes in calcium metabolism caused by sarcoidosis often lead to other problems, such as kidney stones, which further contribute to kidney damage and failure.

Hepatic sarcoidosis

When sarcoidosis involves the liver, which occurs in 50-80% of patients, it is called hepatic sarcoidosis. About one-third of patients have abnormal liver enzymes in routine clinical testing, but the disease rarely affects organ function and most patients show no symptoms.

In rare cases, it can cause liver disease, known as cirrhosis, or portal hypertension, which is high blood pressure in the liver. Hepatic sarcoidosis also can cause less specific symptoms such as fatigue, fever, and joint pain.

Sarcoidosis of the spleen and bone marrow

Sarcoidosis of the spleen is detected in up to three out of four sarcoidosis patients, while sarcoidosis of the bone marrow is rarer, affecting about 10% of patients.

Both can result in cytopenia, or low blood cell counts, in addition to an enlarged spleen, fatigue, weight loss, fever, night sweats, and pain.

Bone marrow is involved in the production of blood cells, so when sarcoidosis affects the bone marrow, it can lead to abnormalities such as anemia or low red blood cells. The spleen is involved in filtering out blood cells, and if is not functioning correctly, too many, or the wrong type of blood cells, can be removed.

Löfgren syndrome

An acute and milder form of sarcoidosis, Löfgren syndrome usually presents with a constellation of symptoms such as erythema nodosum, enlargement of the hilar lymph nodes (located in the center of the chest), fever, and joint pain that spreads from one joint to another. These are sometimes accompanied by uveitis.

While the symptoms are similar to those of overt sarcoidosis, people with Löfgren syndrome usually do not develop long-term or chronic sarcoidosis, and show little or no organ damage.

This syndrome is more common in females and younger people, and usually resolves within six months to two years following diagnosis, sometimes without treatment. However, some medicines may be used to reduce pain and inflammation.

 

Last updated: July 29, 2021

***

Sarcoidosis News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website.