What are the other Names for this Condition? (Also known as/Synonyms)

• Benign Lymphogranulomatosis of Schaumann (Sarcoidosis)
• Besnier Boeck Schaumann Syndrome (Sarcoidosis)
• Darier-Roussy Sarcoid

What is Sarcoidosis? (Definition/Background Information)

• Sarcoidosis is a systemic disease that can affect several parts of the body, leading to the growth of small lumps of inflammatory cells. It most commonly affects the lungs, skin, eyes, and lymph nodes
• In the lungs, the condition causes tiny areas of inflamed cells that appear in the air sacs (alveoli) and airways. Individuals with Sarcoidosis may feel tired, have a persistent cough, may have rashes on the skin, and eye problems
• Sarcoidosis of the lungs and chest lymph nodes is usually diagnosed by various tests, such as a chest-x-ray, computerized axial tomographic (CAT) scan, and a lung function test
• In many cases, Sarcoidosis resolves on its own. If treatment is needed, the options depend upon the severity and symptoms of the condition.
• Sarcoidosis is not a severe illness, although care must be taken and the condition monitored periodically. Rarely, Sarcoidosis can lead to debilitating involvement of the lungs and other organs such as the heart and nervous system

Who gets Sarcoidosis? (Age and Sex Distribution)

• Sarcoidosis affects individuals of all age groups and both genders. The condition is more prevalent in women than men
• It affects all racial and ethnic groups. However, the African American population is found to have an increased incidence of this condition. Also, people with Scandinavian, Asian, Puerto Rican, Irish, and German ancestry are more prone to Sarcoidosis

What are the Risk Factors for Sarcoidosis? (Predisposing Factors)

Following are the risk factors for Sarcoidosis:

• Certain populations, like African Americans and those belonging to Northern Europe, have a greater risk of the condition
• Age appears to be a factor, as the majority of cases diagnosed are found in individuals between 20-40 years of age
• More women than men are affected by this condition
• Sarcoidosis is observed to have a genetic component as well. Thus, if someone in the family has the disease, then the likelihood of getting the condition increases

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Sarcoidosis? (Etiology)

• Currently, there are no known causes for Sarcoidosis
• Although, no gene has been definitively identified to cause the disease, the predisposition of certain populations implies a genetic causative factor
• Exposure to environmental toxins may possibly lead to the disease as well
• It is likely that Sarcoidosis develops when a genetically susceptible individual is exposed to a specific, unknown environmental antigen that acts as a trigger

What are the Signs and Symptoms of Sarcoidosis?

The following are some of the signs and symptoms associated with Sarcoidosis:

• Fatigue
• Fever
• Weight loss
• Dry cough
• Chest pain
• Wheezing
• Shortness of breath
• Rashes or lesions (raised red lesions on the skin)
• Joint pains and swelling
• Hypopigmentation or hyperpigmentation of the skin
• Subcutaneous nodules
• Eye pain, irritation, redness, and sensitivity to light

How is Sarcoidosis Diagnosed?

A physician may use some of the following methods to diagnose Sarcoidosis:

• A thorough physical examination
• Checking medical, occupational, and family history
• Blood test including calcium, vitamin D levels, ACE (angiotensin converting enzyme)
• Test for checking lung function (pulmonary function test)
• Chest X-ray
• CAT scan of the chest region
• Bronchoscopy and biopsy of lungs and/or lymph nodes in the chest (done by a pulmonologist), to find sarcoid granulomas in the tissue
• Mediastinoscopy with biopsy of the lymph nodes in the chest (done by a thoracic surgeon), to find sarcoid granulomas in the tissue
• Sometimes a skin, eyelid, or nasal biopsies can lead to a diagnosis of Sarcoidosis
• Lung biopsy, if deemed necessary
• Magnetic resonance imaging (MRI) of the chest region, if other internal organs like the heart are suspected to be affected
• Positron emission tomography (PET) scan - if the heart, liver, and other organs are suspected to be affected
• An eye examination, if the patient complains about eye problems

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Sarcoidosis?

In many instances, Sarcoidosis resolves itself over time, or with proper treatment. However, the condition is known to lead to the following complications:

• Fibrosis and pulmonary hypertension affecting the lungs, if Sarcoidosis is left untreated. This can result in breathing difficulties and a need for oxygen supplementation
• Abnormal heart rhythms and conduction disorders with the involvement of the heart
• Eye and vision problems that could in severe cases lead to blindness
• In rare cases, the central nervous system could get affected if granulomas are formed in the spinal cord or brain
• Nasal, sinus, and salivary gland related health issues

How is Sarcoidosis Treated?

Presently, there is no cure for Sarcoidosis. It may go away on its own or may need medication to alleviate symptoms. It is not known why some patients recover and why the disease progresses in others. Many times, if Sarcoidosis affects multiple organs, organ-specific medication may be prescribed to restore proper function of the organs.

The following treatments may be necessary, depending on the severity of symptoms:

• Corticosteroid treatment (example: prednisone): It is the most common anti-inflammatory drug prescribed for Sarcoidosis
• Use of immunosuppressant medicines, such as methotrexate and azathioprine, when the eyes, joints, lungs, and skin, are affected
• Hydroxychloroquine and chloroquine, both anti-malarial medications, may be prescribed for Sarcoidosis affecting the skin and brain. Sarcoidosis is complicated by high calcium levels in the body
• Bronchodilators to help with breathing, if necessary
• Treatment with specific medications for associated pulmonary hypertension
• Oxygen supplementation in patients with advanced Sarcoidosis lung disease
• Lung transplantation in end-stage Sarcoidosis lung disease

How can Sarcoidosis be Prevented?

Although concrete prevention methods for Sarcoidosis do not exist, the following have been suggested to reduce the risk of the disease:

• Smoking cessation; even though Sarcoidosis is less common in smokers
• Limiting alcohol consumption
• Avoiding exposure to toxic fumes
• Vaccinations: Pneumococcal vaccine and annual flu shot recommendations, if one is not allergic to them

What is the Prognosis of Sarcoidosis? (Outcomes/Resolutions)

• Sarcoidosis does not lead to severe illness in most cases and gets better with time, without any treatment or with treatment using steroids
• In cases where the symptoms persist, medications and lifestyle changes can help bring some relief
• In addition to regularly visiting a pulmonologist, regular check-ups on blood pressure, blood sugar, and strength of bones is important, especially if the patient is on steroid treatment
• An annual eye exam (with dilation of the eyes) is important to watch-out for Sarcoidosis that affects the eye, and to also check for cataracts while on steroids

Additional and Relevant Useful Information for Sarcoidosis:

The following DoveMed website link is a useful resource for additional information:

https://www.dovemed.com/healthy-living/skin-disorders/