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Sarcoidosis

Last updated: March 17, 2021

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Sarcoidosis is a multisystem disorder characterized by noncaseating granulomatous inflammation. It is classified as either acute or chronic; chronic sarcoidosis is not necessarily preceded by acute sarcoidosis. Acute sarcoidosis has an abrupt onset with constitutional symptoms (e.g., fever, malaise) as well as cough, dyspnea, anterior uveitis, erythema nodosum, and arthralgia, and it is self-limiting after a few years. Chronic sarcoidosis has an insidious onset and is often asymptomatic in its early stages. It primarily affects the lungs, although other systemic manifestations are also possible. The first symptoms of chronic sarcoidosis usually include exertional dyspnea and a dry cough with mild rales on pulmonary examination. Chest x-ray is the most appropriate initial test in a patient with suspected sarcoidosis. A chest x-ray may show parenchymal disease with bilateral hilar lymphadenopathy, but these features are not always evident. Biopsy is the gold standard for diagnosis. The most common histopathological finding is noncaseating granulomas with giant cells. Glucocorticoid therapy is indicated with disease progression or if certain organs, such as the eyes or heart, are affected. While spontaneous remission rates are high during the early stages of sarcoidosis, irreversible lung fibrosis may develop as the disease recurs or progresses.

  • Peak incidence: 25–35 years old with a second peak for females 50–65 years old [1]
  • Sex: > (2:1)
  • Prevalence: ∼ 10 times higher among African Americans than whites [2]

Sarcoidosis most frequently affects young African American women in the US.

Epidemiological data refers to the US, unless otherwise specified.

The cause of sarcoidosis is still unknown; . Current hypotheses suggest that the etiology is multifactorial. [3]

  • Genetic
  • Environmental agent exposure (e.g., beryllium and its salts may cause granulomas)
  • Infectious agents (e.g., mycobacteria are seen as potential etiologic agents)

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

References:[4][5]

Acute sarcoidosis and chronic sarcoidosis are two distinct manifestations of the disease, where acute sarcoidosis does not necessarily precede chronic sarcoidosis.

Acute sarcoidosis (approx. ⅓ of cases) [6]

Chronic sarcoidosis (approx. ⅔ of cases) [6]

  • In rare cases, preceded by acute sarcoidosis
  • Gradual disease course; may be recurrent or progressive

Pulmonary (most common) [7]

Extrapulmonary [8]

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

Lofgren syndrome [10]

  • Highly acute clinical presentation with fever and the following triad of symptoms

Heerfordt syndrome [11]

Jungling disease [12]

Stages of chronic sarcoidosis
Chronic sarcoidosis Chest x-ray findings
Stage 0
  • Normal findings
Stage I
Stage II
Stage III
Stage IV
* In most cases, the disease resolves spontaneously at this stage.

References:[8][13]

A chest x-ray (which may reveal parenchymal disease with hilar lymphadenopathy) is the most appropriate initial test for a patient with suspected sarcoidosis. Laboratory tests may support the diagnosis of sarcoidosis, but a biopsy is the gold standard. Additional tests can help determine the severity of the disease, possible complications, and prognosis.

Chest x-ray

  • Best initial test
  • Sarcoidosis is frequently an incidental finding detected on chest x-ray
  • Findings: hilar lymphadenopathy with or without bilateral reticular opacities
  • Chronic sarcoidosis is categorized according to chest x-ray findings (see “Stages” above).

Patients with chronic sarcoidosis often have moderate clinical manifestations but radiographic findings of extensive disease.

High-resolution CT (HRCT) [14]

Laboratory tests

Bronchoscopy [8][17]

Pulmonary function tests

Differential diagnosis of granulomatous disease [18][19]
Risk factors Clinical presentation Biopsy Other laboratory findings
Sarcoidosis
  • African American women in the US
Tuberculosis (TB)
Hodgkin lymphoma
Non-Hodgkin lymphoma
Pneumoconiosis
  • Exposure to mineral dust (e.g., silica)
Granulomatosis with polyangiitis
  • Caucasian individuals aged 65–74 years
Histoplasmosis [20]
  • AIDS
  • Exposure to bird or bat excrement

The differential diagnoses listed here are not exhaustive.

References:[6]

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

  • Increased calcium is associated with a poorer prognosis .
  • Acute sarcoidosis: spontaneous remission in 60–70% of cases [21]
  • 10–30% of cases may progress to chronic sarcoidosis. [22]
  • Chronic sarcoidosis (% remission rate) [13]
    • Type IV: Life expectancy is limited because of severely impaired lung function.
    • Type III: approx. 20%
    • Type II: approx. 50%
    • Type I: approx. 70%

The spontaneous remission rates in acute sarcoidosis are extremely high. In chronic sarcoidosis, the remission rates vary depending on the type.

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