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, 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:
- 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/
- Pasadhika, S., & Rosenbaum, J. (2015, December). Ocular sarcoidosis. Retrieved May 03, 2021, from https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4662043/
- Patompong Ungprasert, M. (2016, May 01). Clinical characteristics of parotid gland sarcoidosis. Retrieved May 03, 2021, from https://jamanetwork.com/journals/jamaotolaryngology/fullarticle/2503118
- 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/
- 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
- Tadros, M., Forouhar, F., & Wu, G. (2013, December). Hepatic sarcoidosis. Retrieved May 03, 2021, from https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4521279/
- 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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