What is Sarcoidosis?

Sarcoidosis is an idiopathic, chronic multisystemic inflammatory granulomatous disorder. While Sarcoidosis can affect any organ, it mainly affects the lungs and lymph nodes. Abnormal masses, or granulomas, consisting of inflamed tissues may alter the structure or function of the organ. Risk factors for developing Sarcoidosis include female gender, African-American race, and Northern Europeans. The exact cause of Sarcoidosis is unknown. Research postulates it may be due to an autoimmune illness that causes an abnormal immune response. 

What are the symptoms of Sarcoidosis?

  • 50% of patients with Sarcoidosis will be asymptomatic
  • Lung symptoms include dry cough, shortness of breath, chest pain, and rales.
  • Tender or enlarged lymph nodes in the chest, armpits, groin, or neck. 
  • Dermatologic symptoms include erythema nodosum, lupus pernio, and a maculopapular rash. Parotid glands may be enlarged. 
  • Cardiac symptoms include restrictive cardiomyopathy, arrhythmias, and heart blocks. 
  • Red or teary eyes, blurred vision.
  • Rheumatologic symptoms include arthralgias, fever, malaise, weight loss, and hepatosplenomegaly. 
  • Neurologic symptoms can include cranial nerve palsies, diabetes insipidus, hearing loss, seizures, meningitis, and psychiatric disorders. 
  • Patients may present with none, one, or a combination of the above symptoms. Sarcoidosis is a difficult diagnosis to make because it can affect many body systems. 

How is Sarcoidosis diagnosed?

  • A diagnosis of sarcoidosis will be made based on a combination of clinical presentation, labs, and radiologic imaging.
  • The best initial test includes a chest radiograph which will show bilateral hilar lymphadenopathy. Key findings include reticular opacities, ground glass appearance, eggshell calcifications, and fibrosis. 
  • Lab abnormalities include increased ACE levels, hypercalcemia, hypercalciuria, increased vitamin D, and increased ESR.
  • Pulmonary function tests show a restrictive pattern and impaired diffusion capacity of the lungs. PFT’s are primarily used to monitor response to treatment and not to make an initial diagnosis. 
  • A tissue biopsy showing noncaseating granulomas is the most accurate way to make a diagnosis. 

Ways to treat Sarcoidosis?

  •  If patients are asymptomatic then treatment consists of observation. Some patients will have a spontaneous remission within 2 years without treatment. 
  • First line management for patients with symptoms include oral corticosteroids. 
  • Methotrexate or hydroxychloroquine can be used for severe skin lesions. 
  • Overall prognosis is optimistic. 40% of patients spontaneously resolve, 40% resolve with treatment, and only 20% progress to irreversible lung damage.

Posted in: Uncategorized