Sarcoidosis

Definition: A systemic autoimmune disease characterized by the presence of noncaseating granulomatas. Most commonly affects the lungs, with potential additional involvement of the eyes, salivary glands, lungs, heart, liver, and spleen. Extrapulmonary disease in the absence of pulmonary involvement is rare (1)

Eyes (2)

  • Uveitis or episcleritis/scleritis
  • Complications: glaucoma or cataracts

Parotid Gland (3)

  • Generally unilateral (though may also be bilateral) painless gland swelling
  • When associated with anterior uveitis and facial nerve paralysis, the triad is known as Heerfordt Syndrome (4)

Pulmonary

  • The most common manifestation of sarcoidosis
  • Dyspnea, cough, fevers/night sweats, weight loss

Stage I: Hilar lymphadenopathy, normal lung parenchyma

Stage II: Hilar lymphadenopathy, abnormal lung parenchyma

Stage III: No lymphadenopathy, abnormal lung parenchyma

Stage IV: Parenchymal changes with fibrosis and architectural distortion

Cardiac (5)

  • Asymptomatic, heart block, arrhythmias, cardiomyopathy, or sudden cardiac death
  • Patients with known extra-cardiac sarcoidosis should be screened with an EKG and echocardiogram

Hepatic (6)

  • Liver is involved in up to 70% of patients with sarcoidosis
  • Most cases are asymptomatic, but symptoms may include RUQ pain, fatigue, pruritus, or jaundice
  • Potential complications include portal hypertension +/- cirrhosis

Spleen

  • Splenomegaly with resulting potential for anemia and thrombocytopenia

Dermatologic

  • Erythema nodosum: Painful red subcutaneous nodules, most commonly present on the shins. When present with bilateral pulmonary hilar lymphadenopathy as well as arthralgias, the triad is referred to as Löfgren’s syndrome

Diagnosis

  • Hypercalcemia, elevated serum angiotensin-converting enzyme levels suggest the diagnosis
  • Non-caseating (i.e. without a “cheese-like” necrosis) granulomatous inflammation on biopsy

Treatment (7)

Sarcoidosis is generally only treated when there is concern for developing organ damage (including respiratory failure). Often, sarcoidosis is self-limiting and granulomas may stop growing or shrink.

1st Line: Corticosteroids

2nd Line: Methotrexate (may be given with or in place of corticosteroids)

3rd Line: Azathioprine, hydroxychloroquine, or mycophenolate

For dermatologic sarcoidosis: Thalidomide or minocycline may be used

References:

  1. Sreelesh, K., Kumar, M., & Anoop, T. (2014, October). Primary splenic sarcoidosis. Retrieved May 03, 2021, from https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4255862/
  2. Pasadhika, S., & Rosenbaum, J. (2015, December). Ocular sarcoidosis. Retrieved May 03, 2021, from https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4662043/
  3. Patompong Ungprasert, M. (2016, May 01). Clinical characteristics of parotid gland sarcoidosis. Retrieved May 03, 2021, from https://jamanetwork.com/journals/jamaotolaryngology/fullarticle/2503118
  4. Fraga, R., Kakizaki, P., Valente, N., Portocarrero, L., Teixeira, M., & Senise, P. (2017). Do you know this syndrome? Heerfordt-waldenström syndrome. Retrieved May 13, 2021, from https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5595615/
  5. Cardiac sarcoidosis and sudden death risk: What is the “consensus” regarding the approach to management, diagnosis, and treatment? (n.d.). Retrieved May 03, 2021, from https://www.acc.org/latest-in-cardiology/articles/2015/03/26/07/57/cardiac-sarcoidosis-and-sudden-death-risk
  6. Tadros, M., Forouhar, F., & Wu, G. (2013, December). Hepatic sarcoidosis. Retrieved May 03, 2021, from https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4521279/
  7. Raghu, G., Berman, J.S., & Govender, P. (2018). Treatment of Sarcoidosis. Retrieved May 13, 2021, from https://www.thoracic.org/patients/patient-resources/resources/sarcoidosis-pt-2-treatment.pdf

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