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A. MLA Map Musculoskeletal > Sarcoidosis > Flashcards

Sarcoidosis Flashcards

1
Q

Sarcoid means flesh and osis means disorder, so sarcoidosis means disorder of the flesh. Is sarcoidosis an immunological or occupational condition?

A
  • immunological
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2
Q

In a normal immune response pathogen-associated molecular pattern molecules (PAMPs) are recognised by antigen presenting cells (APC). Which of the following is NOT an APC?

1 - macrophage
2 - T cell
3 - B cell
4 - dendritic cells

A

2 - T cell

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3
Q

Once antigen presenting cells (APC) recognise pathogen-associated molecular pattern molecules (PAMPs) they engulf the foreign body and then present it on a major histocompatibility complex class II (MHC-II) to which cells?

1 - cytotoxic T cells
2 - T helper cells
3 - B cells
4 - all of the above

A

2 - T helper cells
- once bound to the T helper cell the APC releases cytokines to activate the T helper cells

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4
Q

Which 2 cytokine released by antigen presenting cells (APC) activates T helper cells?

1 - IL-2
2 - IL-6
3 - TNF-a
4 - IL-4

A

1 - IL-2
4 - IL-4

  • once activated the T helper cells can activate B and cytotoxic T cells
  • secrete inflammatory cytokines
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5
Q

Which of the following are the functions of T helper cells?

1 - activates B cells
2 - secretes inflammatory cytokines to recruit more immune cells
3 - activates cytotoxic T cells
4 - all of the above

A

4 - all of the above

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6
Q

In sarcoidosis does the T cell activation cascade and secretion of inflammatory cytokines become under or over activated?

A
  • over activated
  • despite the lack of a specific cause being identified
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7
Q

Is the specific cause of sarcoidosis, a parenchymal lung disease known?

A
  • no
  • linked with autoimmune deficiency
  • mainly affect the lungs
  • may be genetic inheritance
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8
Q

Is sarcoidosis, a parenchymal lung disease (restrictive lung disease) permanent?

A
  • no
  • can resolve itself
  • can remain latent and present at any time
  • remains latent in granulomas of T cells and macrophages
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9
Q

Is sarcoidosis more common in men or women?

A
  • women
  • 3:1 ratio
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10
Q

What age group does sarcoidosis normally affect?

1 - 20-40 y/o
2 - 35 y/o
3 - 50-80 y/o
4 - 20-50 y/o

A

4 - 20-50 y/o

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11
Q

Sarcoidosis has an ethnicity affect. Which of the following group of patients is NOT typically as affected as the other 3 groups?

1 - Scandinavian
2 - Caucasian
3 - Afro-Caribbean
4 - African American

A

2 - Caucasian

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12
Q

What is the incidence of Sarcoidosis in the UK?

1 - 5 / 100
2 - 5 / 1000
3 - 5 / 10,000
4 - 5 / 100,000

A

4 - 5 / 100,000
- most common idiopathic pulmonary fibrosis disease in UK

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13
Q

What is the most common cause of diffuse parenchymal lung diseases (DPLDs), also known as interstitial lung disease (ILD)?

1 - pneumoconiosis
2 - sarcoidosis
3 - connective tissue disorders
4 - iatrogenic

A

2 - sarcoidosis

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14
Q

Sarcoidosis is a multisystem granulomatous disease, but which organ is most commonly affected?

1 - heart
2 - skin
3 - lungs
4 - GIT

A

3 - lungs

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15
Q

What infection of the lungs is Sarcoidosis commonly associated with?

1 - Empyema
2 - Lung abscess
3 - Tuberculosis
4 - all of the above

A

3 - Tuberculosis
- tuberculosis which has similiar symptoms

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16
Q

Which form of cancer is sarcoidosis commonly associated with?

1 - small cell lung cancer
2 - lymphoma
3 - large cell lung cancer
4 - melanoma

A

2 - lymphoma
- because this is what is most commonly affected in sarcoidosis

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17
Q

Which of the following is NOT a typical clinical sign we can see in sarcoidosis?

1 - cardiac arrhythmias
2 - GIT obstruction
3 - anterior uveitis (inflammation of middle of the eye)
4 - bilateral hilar lymphadenopathy
5 - erythema nodosum

A

2 - GIT obstruction

  • in a healthy patients bilateral hilar lymph nodes should NOT be visible on a chest X-ray
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18
Q

All of the following are the common acute symptoms of sarcoidosis. Which 2 are most common?

1 - fever/night sweats
2 - arthralgia (joint pain) and myalgia (muscle pain)
3 - anterior uveitis (inflammation of middle of the eye)
4 - breathlessness
5 - ⬇️ appetite
6 - weight loss
6 - erythema nodosum
8 - bilateral hilar lymphadenopathy

A

3 - anterior uveitis (painful red eyes)
6 - erythema nodosum (painful red skin) - 20-35% of patients with sarcoidosis have these

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19
Q

Is sarcoidosis a permanent condition?

A
  • no
  • it can resolve spontaneously, recur or could progress to pulmonary fibrosis
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20
Q

Typically how is sarcoidosis commonly identified?

1 - by GP
2 - on chest X-ray
3 - ultrasound
4 - based on clinical symptoms

A

2 - on chest X-ray
- presents with bilateral hilar lymphadenopathy

21
Q

Which of the following syndromes presents as acute sarcoidosis?

1 - down syndrome
2 - metabolic syndrome
3 - turner syndrome
4 - loefgrens syndrome

A

4 - loefgrens syndrome
- a phenotype of sarcoidosis

22
Q

Loefgrens syndrome presents as acute sarcoidosis. It typically presents with a triad of symptoms. Which of the following is NOT one of this triad?

1 - fever
2 - erythema nodosum
3 - hyperglycaemia
4 - bilateral hilar adenopathy;

A

3 - hyperglycaemia

23
Q

Patients can present with all of the following symptoms in chronic sarcoidosis when sarcoidosis is active. Which is the most common symptom?

1 - progressive worsening of breathlessness
2 - ⬇️ exercise tolerance
3 - cough
4 - ⬇️ appetite/anorexia
5 - weight loss
6 - all of the above

A

1 - progressive worsening of breathlessness
- hilar lymph nodes can compress airways
- lung tissue can become scarred and stiff, making it hard to breathe

24
Q

Sarcoidosis can be multi system. All of the following can be affected, but which 3 are the most common?

1 - Lung
2 - Skin
3 - Lymph nodes
4 - Eyes (anterior uveitis, posterior uveitis, retinal vasculitis, keratoconjunctivitis, conjunctival follicles)
5 - Joints and muscles
6 - Liver
7 - Spleen
8 - Heart

A

1 - Lung (90% have pulmonary
3 - Lymph nodes (palpable in 30%)
6 - Liver (hepatomegaly in 20%)

25
Q

What % of patients with sarcoidosis have any lung involvement, or only lung involvement?

1 - 5%
2 - 15%
3 - 50%
4 - 90%

A

3 - 50%
have ONLY lung involvement

4 - 90%
- have SOME lung involvement

26
Q

20% of patients with stage 1 sarcoidosis can present with abnormal lung function tests. What % of patients with stages 2-4 present with abnormal lung function tests?

1 - 1-20%
2 - 10-40%
3 - 11-80%
4 - 100%

A

3 - 11-80%
- baseline lung function does not predict outcome and mortality

  • TLCO is most sensitive to predicting steroid responsiveness and mortality
27
Q

Sarcoidosis is typically difficult to diagnose, and is typically once other conditions have been ruled out, we are left with sarcoidosis. Which of the following can help confirm a diagnosis of sarcoidosis though?

1 - clinical experience
2 - chest X-ray (bilateral lymphadenopathy?
3 - hypercalcaemia (due to macrophages)
4 - elevated lymphocytes (T cells)
5 - elevated angiotensin converting enzyme (secreted by T cells)
6 - affected tissue biopsy
7 - all of the above

A

7 - all of the above

  • remember CRP
  • C = clinical exam and experience
  • R = radiological
  • P = pathology (biopsy)
28
Q

All of the following tests are typically performed in an attempt to diagnose sarcoidosis, EXCEPT which one?

1 - radiography (CXR)
2- lung function
3 - ECG (arrythmias)
4 - blood and urine analysis (hypercalcaemia and T cells)
5- eye examination
6 - skin test (rule out TB)
7 - angiotensin converting enzymes (ACE)
8 - hypercalcaemia
9 - high definition CT

A

9 - high definition CT

  • remember CRP
  • C = clinical exam and experience
  • R = radiological
  • P = pathology (biopsy)
29
Q

Which blood marker is typically elevated in most patients with sarcoidosis?

1 - creatine kinase
2 - CRP
3 - T cells
4 - ESR

A

1 - creatine kinase

30
Q

Sarcoidosis is characterised by non-caseating granulomas, which means there is no tissue necrosis at the centre. Which one of the cell types below does NOT make up a non-caseating granuloma?

1 - B cells
2 - T cells (CD4 and CD8)
3 - macrophages
4 - neutrophils

A

4 - neutrophils

  • TB is caseating and thats how we can differentiate between sarcoidosis and TB
31
Q

The macrophages contained within the T cells come together and form what?

1 - megakaryocyte
2 - langerhans giant cell
3 - merkel cell
4 - giant neutrophil

A

2 - langerhans giant cell

  • granulomas eventually cause lymph nodes to enlarge causing bilateral hilar lymphadenopathy
32
Q

In addition to macrophages within the granuloma in sarcoidosis, which 2 of the following are commonly present?

1 - atheroma
2 - embolism
3 - asteroid bodies
4 - schaumann bodies

A

3 - asteroid bodies
4 - schaumann bodies

  • granulomas eventually cause lymph nodes to enlarge causing bilateral hilar lymphadenopathy
33
Q

In sarcoidosis, although the exact cause is unknown, it is hypothesised that whatever the cause is, the granuloma surround the stressor and try to kill it or contain it. Which of the following have been identified as potential causes of sarcoidosis?

1 - remnants of microbial organisms
2 - mycobacterium spp
3 - propionibacterium acnes
4 - herpes
5 - organic dusts
6 - beryllium (metal)
7 - all of the above

A

7 - all of the above

34
Q

There are 5 stages of sarcoidosis. Which of the following is stage 0?

1 - Nodes + upper zone parenchymal disease (25-30%)
2 - Parenchymal disease upper zones
3 - Bilateral Hilar LN (45-65%)
4 - Normal CXR (5-10%)
5 - End stage pulmonary fibrosis

A

4 - Normal CXR (5-10%)

35
Q

There are 5 stages of sarcoidosis. Which of the following is stage 1?

1 - Nodes + upper zone parenchymal disease (25-30%)
2 - Parenchymal disease upper zones
3 - Bilateral Hilar LN (45-65%)
4 - Normal CXR (5-10%)
5 - End stage pulmonary fibrosis

A

3 - Bilateral Hilar LN (45-65%)

36
Q

There are 5 stages of sarcoidosis. Which of the following is stage 2?

1 - Nodes + upper zone parenchymal disease (25-30%)
2 - Parenchymal disease upper zones
3 - Bilateral Hilar LN (45-65%)
4 - Normal CXR (5-10%)
5 - End stage pulmonary fibrosis

A

1 - Nodes + upper zone parenchymal disease (25-30%)

37
Q

There are 5 stages of sarcoidosis. Which of the following is stage 3?

1 - Nodes + upper zone parenchymal disease (25-30%)
2 - Parenchymal disease upper zones
3 - Bilateral Hilar LN (45-65%)
4 - Normal CXR (5-10%)
5 - End stage pulmonary fibrosis

A

2 - Parenchymal disease upper zones

38
Q

There are 5 stages of sarcoidosis. Which of the following is stage IV?

1 - Nodes + upper zone parenchymal disease (25-30%)
2 - Parenchymal disease upper zones
3 - Bilateral Hilar LN (45-65%)
4 - Normal CXR (5-10%)
5 - End stage pulmonary fibrosis

A

5 - End stage pulmonary fibrosis

39
Q

Is sarcoidosis only a restrictive lung disease?

A
  • no
  • can be obstructive and restrictive
  • endobronchial sarcoidosis = OBSTRUCTIVE
  • fibrosis = RESTRICTIVE
40
Q

Angiotensin converting enzymes (ACE) needs to be measured in patients with suspected sarcoidosis. Which 2 cell types secrete ACE in sarcoidosis?

1 - B cells
2 - T helper cells
3 - macrophages
4 - neutrophils

A

2 - T helper cells
3 - macrophages

  • T cells and macrophages are increased and can form granulomas
  • so if sarcoidosis is active ACE will be higher
41
Q

What is the prognosis of sarcoidosis?

A
  • stage 1 = 2/3 in remission within 10 years
  • stage 2 = 60% in remission in 2 years
  • 1/3 of patients progress to significant organ damage
42
Q

Lofgren syndrome can occur in sarcoidosis, which is an acute clinical presentation of systemic sarcoidosis,. Which of the following is NOT part of the triad that is typical in Lofgren syndrome?

1 - fever
2 - erythema nodosum
3 - uveitis
4 - bilateral hilar adenopathy

A

3 - uveitis

43
Q

What is the mortality rate in patients with sarcoidosis?

1 - 0.5-1%
2 - 1-5%
3 - 10-15%
4 - >20%

A

2 - 1-5%
- respiratory failure
- cardiac arrhythmia
- neurosarcoidosis

44
Q

What medication are patients with sarcoidosis stages 2-4 typically treated with?

1 - methotrexate
2 - allopurinol
3 - prednisolone
4 - co-amoxiclav

A

3 - prednisolone
- given orally
- 20-40mg daily, either as short course 1-2 weeks or 1-3 months

45
Q

TB and sarcoidosis can present in a similar manner. Why is it important to identify the cause of the symptoms prior to treating?

1 - medication may cause anaphylaxis
2 - steroid can worsen effects of TB
3 - steroids can worsen sarcoidosis
4 - all of the above

A

2 - steroid can worsen effects of TB
- BUT sarcoidosis responds well to steroids

46
Q

If oral steroids, specifically prednisolone do not treat sarcoidosis, what can patients be given?

1 - Azathioprine
2 - Methotrexate
3 - Mycophenolate
4 - all of the above

A

4 - all of the above

All are immunosuppressive

47
Q

Do asymptomatic or stage 1 sarcoidosis patients receive any specific treatment?

A
  • no
  • generally observation only
48
Q

Which of the following is NOT a differential for sarcoidosis?

1 - Tuberculosis
2 - Occupational lung disease
3 - Chronic asthma
4 - Fungal infection
5 - Malignancies (lymphoma, lung Ca)

A

3 - Chronic asthma

A. MLA Map Musculoskeletal (6 decks)

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