Last updated: September 11, 2023

Summarytoggle arrow icon

Sarcoidosis is a multisystem inflammatory disorder characterized by noncaseating granuloma formation, most commonly in the lungs and hilar lymph nodes. Many patients are asymptomatic, especially in the early stages of disease. Symptomatic patients typically present with constitutional symptoms, cough, and dyspnea. The cause of sarcoidosis is unknown but is thought to be related to an environmental exposure in a genetically predisposed individual. Chest x-ray is the preferred initial test for individuals with suspected sarcoidosis since bilateral hilar lymphadenopathy and/or pulmonary involvement is present in ∼ 90% of affected patients. Sarcoidosis is a diagnosis of exclusion that typically requires characteristic clinical features and/or findings on chest imaging, noncaseating granulomas on biopsy, and exclusion of more common causes of granulomatous disease or lymphadenopathy. While most patients with pulmonary sarcoidosis do not require immunosuppressive therapy, patients with symptomatic or progressive disease are typically treated with glucocorticoids. Spontaneous remission without complications is common in patients with early-stage pulmonary disease, but the risk of irreversible lung fibrosis increases with disease recurrence and progression.

Epidemiologytoggle arrow icon

  • Average age of onset: 30–55 years [1][2]
  • Sex: : > (2:1)
  • Prevalence
    • Highest in African American and Scandinavian populations
    • 2–4 times higher in Black individuals than in non-Hispanic white individuals [3][4]

Epidemiological data refers to the US, unless otherwise specified.

Etiologytoggle arrow icon

The cause of sarcoidosis is unknown, but genetic predisposition (e.g., HLA-linked), exposure to substances associated with granuloma formation (e.g., beryllium and its salts), and infectious agents (e.g., mycobacteria) are believed to play a role. [5]

Pathophysiologytoggle arrow icon

Sarcoidosis is a systemic disorder characterized by widespread, immune-mediated formation of noncaseating granulomas.

Clinical featurestoggle arrow icon


  • Sarcoidosis is a disease with highly variable manifestations, ranging from asymptomatic to progressive, relapsing disease.
    • Typically sudden onset with spontaneous remission in approximately two-thirds of patients [3]
    • Approx. one-third of patients develop progressive chronic disease.
  • Most common presenting signs and symptoms

Pulmonary sarcoidosis

Extrapulmonary sarcoidosis [7]

  • Occurs in approx. 30% of affected individuals
  • Extrapulmonary findings usually involve the skin, eyes, heart, and/or the renal and central nervous systems.

Cutaneous sarcoidosis [8]

Lymph node findings

  • Most commonly affected extrapulmonary site
  • Occurs in 70–90% of affected individuals
  • Mediastinal lymph nodes: bilateral, hilar, and/or paratracheal mediastinal adenopathies (approx. 90% of affected individuals)
  • Intraabdominal lymphadenopathy [7]

Ocular sarcoidosis

Musculoskeletal findings


Other findings [7]

Features of sarcoidosis are GRUELING: Granulomas, aRthritis, Uveitis, Erythema nodosum, Lymphadenopathy, Interstitial fibrosis, Negative TB test, and Gammaglobulinemia.

Subtypes and variantstoggle arrow icon

Lofgren syndrome [9]

  • An acute clinical manifestation with fever and the following triad of symptoms:
  • Prognosis is good (spontaneous remission occurs in 70–80% of affected individuals). [9]

Heerfordt syndrome

Diagnosticstoggle arrow icon

General principles [7][10][11]

  • Sarcoidosis is a diagnosis of exclusion supported by:
  • Obtain initial studies to:
    • Rule out differential diagnoses (e.g., other granulomatous diseases).
    • Assess pulmonary and extrapulmonary disease (including ophthalmological evaluation).
  • Consider additional studies (e.g., biopsy) under specialist guidance based on suspected organ involvement.

Pulmonary involvement and/or hilar lymphadenopathy are present in ∼ 90% of patients. Obtain chest imaging in all patients. [7]

The eye and the skin are the most common sites of extrathoracic disease. [7]

Initial studies [9][11]

Increased ACE levels may be seen. However, they are not specific for sarcoidosis and should not be used in isolation for diagnosis or monitoring. [9]

Cardiac sarcoidosis can lead to sudden cardiac death. Perform an ECG in all patients with suspected sarcoidosis regardless of symptoms. [10]


Radiographic stages of pulmonary sarcoidosis [12]
Stages Chest x-ray findings
Stage 0
  • Normal
Stage I
  • Bilateral hilar lymphadenopathy: bilateral enlargement of the lymph nodes of the pulmonary hila without pulmonary infiltrates
Stage II
Stage III
Stage IV

Stage I pulmonary sarcoidosis is often an incidental finding detected on chest imaging. [10]

Patients with pulmonary sarcoidosis may have minimal to no symptoms despite significant radiographic abnormalities.

Pulmonary function testing (PFTs) [12]

Obtain PFTs to assess disease severity in patients with pulmonary sarcoidosis.

PFTs show an early reduction of DLCO and lung compliance.

Additional studies [11]

Consider the following studies under specialist guidance based on suspected organ involvement:

Biopsy [10][11]

Histopathological confirmation is not required in patients with pathognomonic presentations, i.e., Lofgren syndrome, Heerfordt syndrome, and lupus pernio. [10]

Differential diagnosestoggle arrow icon

Differential diagnosis of granulomatous disease

Overview of granulomatous diseases [13][14]
Risk factors/prevalence Clinical features Lung biopsy Other laboratory findings
  • Highest in African American and Scandinavian populations
  • 2–4 times higher in Black individuals than in non-Hispanic white individuals
Tuberculosis (TB)
Hodgkin lymphoma
Non-Hodgkin lymphoma
  • Exposure to mineral dust (e.g., silica)
Granulomatosis with polyangiitis
  • Prevalence in the US is highest among White individuals aged 65–74 years
Histoplasmosis [15]
  • AIDS
  • Exposure to bird or bat excrement

The differential diagnoses listed here are not exhaustive.

Treatmenttoggle arrow icon

General principles [7][10]

Immunosuppressive therapy [10][12]

Supportive therapy [11]

Complicationstoggle arrow icon

We list the most important complications. The selection is not exhaustive.

Prognosistoggle arrow icon

Referencestoggle arrow icon

  1. Grunewald J, Grutters JC, Arkema EV, Saketkoo LA, Moller DR, Müller-Quernheim J. Sarcoidosis. Nat Rev Dis Primers. 2019; 5 (1).doi: 10.1038/s41572-019-0096-x . | Open in Read by QxMD
  2. Arkema EV, Cozier YC. Epidemiology of sarcoidosis: current findings and future directions. Therapeutic Advances in Chronic Disease. 2018; 9 (11): p.227-240.doi: 10.1177/2040622318790197 . | Open in Read by QxMD
  3. Dumas O, Abramovitz L, Wiley AS, Cozier YC, Camargo CA Jr. Epidemiology of Sarcoidosis in a Prospective Cohort Study of U.S. Women.. Annals of the American Thoracic Society. 2016; 13 (1): p.67-71.doi: 10.1513/AnnalsATS.201508-568BC . | Open in Read by QxMD
  4. Soto-Gomez N, Peters JI, Nambiar AM. Diagnosis and Management of Sarcoidosis.. Am Fam Physician. 2016; 93 (10): p.840-8.
  5. Hena KM. Sarcoidosis Epidemiology: Race Matters. Frontiers in Immunology. 2020; 11.doi: 10.3389/fimmu.2020.537382 . | Open in Read by QxMD
  6. Chen ES, Moller DR. Etiologies of Sarcoidosis. Clin Rev Allergy Immunol. 2015; 49 (1): p.6-18.doi: 10.1007/s12016-015-8481-z . | Open in Read by QxMD
  7. Gwadera Ł, Białas AJ, Iwański MA, Górski P, Piotrowski WJ. Sarcoidosis and calcium homeostasis disturbances—Do we know where we stand?. Chron Respir Dis. 2019; 16: p.147997311987871.doi: 10.1177/1479973119878713 . | Open in Read by QxMD
  8. Ungprasert P, Ryu JH, Matteson EL. Clinical Manifestations, Diagnosis, and Treatment of Sarcoidosis. Mayo Clinic Proceedings: Innovations, Quality & Outcomes. 2019; 3 (3): p.358-375.doi: 10.1016/j.mayocpiqo.2019.04.006 . | Open in Read by QxMD
  9. Katta R. Cutaneous sarcoidosis: a dermatologic masquerader.. Am Fam Physician. 2002; 65 (8): p.1581-4.
  10. Crouser ED, Maier LA, Wilson KC, et al. Diagnosis and Detection of Sarcoidosis. An Official American Thoracic Society Clinical Practice Guideline. Am J Respir Crit Care Med. 2020; 201 (8): p.e26-e51.doi: 10.1164/rccm.202002-0251st . | Open in Read by QxMD
  11. Drent M, Crouser ED, Grunewald J. Challenges of Sarcoidosis and Its Management. N Engl J Med. 2021; 385 (11): p.1018-1032.doi: 10.1056/nejmra2101555 . | Open in Read by QxMD
  12. Belperio JA, Shaikh F, Abtin FG, et al. Diagnosis and Treatment of Pulmonary Sarcoidosis. JAMA. 2022; 327 (9): p.856.doi: 10.1001/jama.2022.1570 . | Open in Read by QxMD
  13. Wheat J, Kauffman CA. Diagnosis and treatment of disseminated histoplasmosis in HIV-uninfected patients. In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. Last updated: July 18, 2016. Accessed: February 28, 2017.
  14. Kauffman CA. Pathogenesis and Clinical Features of Pulmonary Histoplasmosis. In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. Last updated: August 10, 2018. Accessed: January 13, 2019.
  15. Kurowski R, Ostapchuk M. Overview of Histoplasmosis. Am Fam Physician. 2002; 66 (12): p.2247-2253.
  16. Inoue Y, Inui N, Hashimoto D, et al. Cumulative Incidence and Predictors of Progression in Corticosteroid-Naïve Patients with Sarcoidosis. PLoS ONE. 2015; 10 (11): p.e0143371.doi: 10.1371/journal.pone.0143371 . | Open in Read by QxMD

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