Sarcoidosis Flashcards

1
Q

What is sarcoidosis?

A

Systemic granulomatous disease of unknown origin that is highly associated with lung involvement but can affect all organs.

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2
Q

What is the typical age of onset for sarcoidosis?

A

Before 50 years old, with peak incidence in 20-40 years old.

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3
Q

Which racial and ethnic groups are most affected by sarcoidosis?

A

All racial and ethnic groups, but most prevalent in individuals with north-european ancestry.

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4
Q

Is sarcoidosis more common in males or females?

A

More common in females than males.

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5
Q

How does socioeconomic status relate to sarcoidosis severity?

A

Lower socioeconomic status is associated with more severe sarcoidosis.

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6
Q

What is the familial tendency of sarcoidosis?

A

Clusters in families, more likely to occur in individuals with family members who have sarcoidosis.

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7
Q

What triggers the immune response in sarcoidosis?

A

An unknown antigen presented on antigen-presenting cells (APC).

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8
Q

Which cells are primarily involved in the immune response of sarcoidosis?

A

T-helper cells and cytokines such as macrophage-derived TNF-alpha, IL-12, IL-18.

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9
Q

What forms as a result of the inflammatory response in sarcoidosis?

A

Sarcoid epithelioid granuloma.

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10
Q

What role do T17 helper cells play in sarcoidosis?

A

They produce IL-17, which inhibits T-regulatory (Treg) cells.

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11
Q

What is a Treg cell?

A

Immunosuppressive cell that prevents autoimmunity by maintaining self-tolerance.

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12
Q

Which organs are most commonly affected by sarcoidosis?

A

Lungs, eyes, and skin.

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13
Q

What are the pulmonary manifestations of sarcoidosis?

A

Mediastinal and symmetrical hilar lymphadenopathy, dry cough, dyspnea, nonspecific chest pain.

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14
Q

What is parenchymal involvement in sarcoidosis characterized by?

A

Nodules, fibrosis, and traction bronchiectasis, with crackles on auscultation.

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15
Q

What symptoms may indicate airway involvement in sarcoidosis?

A

Asthma-like symptoms.

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16
Q

What are potential cardiac manifestations of sarcoidosis?

A

Sudden death, arrhythmias, cardiomyopathy.

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17
Q

What neurological manifestations can occur with sarcoidosis?

A

Seizures, cranial nerve palsies, aseptic meningitis.

18
Q

What are the ophthalmic manifestations associated with sarcoidosis?

A

Uveitis, vision loss, dry eyes.

19
Q

What bone-related symptoms may occur in sarcoidosis?

A

Arthralgia causing difficulty walking, mostly affecting lower limb joints and fingers.

20
Q

What dermatological manifestation is commonly seen in sarcoidosis?

A

Erythema nodosum.

21
Q

What renal issue can arise from sarcoidosis?

A

Kidney stones due to hypercalcaemia.

22
Q

What is Lofgren’s syndrome?

A

Acute form of sarcoidosis presenting with classic triad of erythema nodosum, bi-hilar lymphadenopathy, and polyarthralgia with fever.

23
Q

How long does Lofgren’s syndrome typically resolve?

A

Within 6-24 months.

24
Q

What is the classification of pulmonary sarcoidosis based on chest x-ray and HRCT?

A

Pulmonary sarcoidosis is classified into stages 0 to IV based on chest x-ray and HRCT findings.

25
Q

What are the findings in Stage 0 of pulmonary sarcoidosis?

A

No abnormal findings to suggest pulmonary sarcoidosis.

26
Q

What are the findings in Stage I of pulmonary sarcoidosis?

A

Hilar and mediastinal lymphadenopathy with clear lungs.

27
Q

What are the findings in Stage II of pulmonary sarcoidosis?

A
  1. Hilar and mediastinal lymphadenopathy 2. Reticular opacities/shadowing due to parenchymal infiltration.
28
Q

What are the findings in Stage III of pulmonary sarcoidosis?

A

Lung nodules due to granulomas.

29
Q

What are the findings in Stage IV of pulmonary sarcoidosis?

A

Pulmonary fibrosis with bilateral reticular opacities, shaggy/unclear heart borders, lung volume loss, and honeycombing.

30
Q

What is the significance of reticular opacities in Stage IV?

A

Bilateral reticular opacities are most prominent around lung bases.

31
Q

What is observed in Stage IV regarding lung nodules?

A

Normal-sized nodes with lung fibrosis.

32
Q

What investigations are done for sarcoidosis?

A

FBC: Check for anaemia or lymphopenia
U&E: Check renal function
LFTS
Serum angiotensin converting enzyme (SACE): Elevated levels in granulomatous-inflammatory conditions such as sarcoidosis
Histology: Non-caseating granulomas

33
Q

What is the management approach for sarcoidosis?

A

Most patients don’t need treatment but it’s considered in patients who meet the requirements of Well’s law.

34
Q

What are the two main principles of Well’s law of sarcoidosis treatment?

A
  1. Prevent organ damage or dangerous disease
  2. To improve quality of life
35
Q

What is the first-line treatment for sarcoidosis?

A

Prednisolone

36
Q

What are the second-line treatments for sarcoidosis?

A

MTX, azathioprine

Azathioprine is indicated for neurosarcoidosis and has a higher infection rate than MTX.

37
Q

What are the side effects of second-line treatments for sarcoidosis?

A

Cytopenias, gastrointestinal effects, LFT derangement

38
Q

What are the third-line treatments for sarcoidosis?

A

Mycophenolate Mofetil, hydroxychloroquine, anti-TNF biologics

MMF is indicated for neurosarcoidosis but is better tolerated than azathioprine.

39
Q

What is the indication for hydroxychloroquine in sarcoidosis?

A

Indicated for musculoskeletal manifestations and hypercalcemia

Side effects include optic neuritis.

40
Q

What is the indication for anti-TNF biologics in sarcoidosis?

A

Indicated for ophthalmic manifestations and neurosarcoidosis

Side effects include paradoxical granulomatous disease and sepsis.