Sarcoidosis: symptoms, diagnosis, and treatment

What is Sarcoidosis?

Sarcoidosis is a multisystem disorder of unknown etiology characterized by noncaseating granulomas in organs. This condition mostly affects young adults and characteristically presents with reticular opacities in the lungs and bilateral hilar lymphadenopathy. Other involved sites include eyes, skin, joints, and in some cases, the reticuloendothelial system, musculoskeletal system, exocrine glands, heart, kidney, and central nervous system. 

How common is sarcoidosis?

The incidence is 11 cases per 100,000 in whites but 34 cases per 100,000 in African Americans, with a lifetime risk of 2.4 percent in the United States. Extrapulmonary sarcoid is seen in up to 25 to 30 percent of patients. Cardiac involvement is seen more commonly in males, while skin and eye features are more prominent in women. Extrapulmonary features can differ in age of presentation, gender, and ethnicity. (https://www.ncbi.nlm.nih.gov/books/NBK430687/)

What causes Sarcoidosis?

Research suggests that a combination of genetics and environmental factors cause sarcoidosis, but its exact cause is unknown.

People with overreactive immune system tend to get sarcoidosis with triggers like bacteria or viral infections. Due to it`s unknown etiology, it is impossible to predict who will get sarcoidosis. 

What are the symptoms of sarcoidosis?

General symptoms of sarcoidosis may include:

• Fever
• Fatigue
• Joint pain
• Muscle aches or weakness
• Night sweats or swollen lymph nodes
• Unexplained weight loss
• Kidney stones 

Symptoms of sarcoidosis in lungs include:

• Cough
• Shortness of breath (dyspnea)
• Chest pain
• Wheezing

Symptoms of sarcoidosis in eye include:

• Blurred vision or loss of vision (optic neuritis)
• Eye pain
• Red or swollen eye (uveitis or conjunctivitis)
• Sensitivity to light

Symptoms of sarcoidosis of skin: 

• Growths under your skin around scars or tattoos
• Raised, reddish-purple sores or rash across your nose or cheeks (lupus pernio)
• Red, tender bumps on the shin (erythema nodosum)

Light or dark patches of skin

Symptoms of sarcoidosis of heart:

• Chest pain
• Palpitations
• Irregular heart beats (arrythmia)
• Heart failure
• Shortness of breath

Symptoms of sarcoidosis of nervous system:

• Increased thirst or urination (Diabetes insipidus)
• Weak or paralyzed facial muscles (Bell`s palsy)
• Headaches
• Seizures 

What are the stages of pulmonary sarcoidosis?

Pulmonary (lung) sarcoidosis is sometimes described in stages called the Siltzbach classification system. They’re based on your lungs’ appearance on a chest X-ray and mostly describe where the granulomas are located. 

What is Löfgren syndrome?

Löfgren syndrome is a sudden onset of sarcoidosis defined by a group of symptoms affecting several parts of your body:

• Arthritis in multiple joints
• Fever
• Red, tender bumps on shin (erythema nodosum)
• Swollen lymph nodes in chest

Löfgren syndrome usually resolves in six months to two years. (https://my.clevelandclinic.org/health/diseases/11863-sarcoidosis) 

How is sarcoidosis diagnosed?

History and physical examination of various systems supported with following tests to rule out/in the diagnosis:

• Blood and urine tests to check overall health and how well your kidneys and liver are working.
• Chest X-rays to check lungs and heart
• Computerized tomography (CT) scan of the chest to look at your lungs.
• Lung function tests, also called pulmonary function tests, to measure how much air you can breathe in and out and how much oxygen your lungs send to your blood.
• Electrocardiogram (ECG or EKG) and cardiac ultrasound to check for heart problems and look at your heart's health.
• Eye exam to check for vision problems that may be caused by sarcoidosis.
• Positron emission tomography (PET) scan or magnetic resonance imaging (MRI) if sarcoidosis may be affecting your heart or central nervous system. 
• Tissue sample is used to look for the granulomas commonly seen with the condition. For example, biopsies can be taken from your skin, lungs and lymph nodes.

What is the treatment for sarcoidosis?

Treatment for sarcoidosis depends on the severity of symptoms. Treatment can lessen the body's immune system response and inflammation.

• Medicines like corticosteroids, methotrexate (Trexall) and azathioprine (Azasan, Imuran) help to lessen the inflammation. 
  • Hydroxychloroquine (Plaquenil) may be helpful for skin lesions and high blood-calcium levels.
  • Tumor necrosis factor-alpha (TNF-alpha) inhibitors are commonly used to treat inflammation from rheumatoid arthritis. They also can be helpful in treating sarcoidosis that hasn't responded to other treatments.
• Physical therapy to lessen tiredness and strengthen muscles.
• Pulmonary rehabilitation to help ease breathing and do more activities.
• Implanted cardiac pacemaker or defibrillator for heart rhythm problems. (https://www.mayoclinic.org/diseases-conditions/sarcoidosis/diagnosis-treatment/drc-20350363)