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Year 3: Respiratory > Respiratory: Sarcoidosis > Flashcards

Respiratory: Sarcoidosis Flashcards

1
Q

Define what is meant by sarcoidosis [1]

A

A chronic granulomatous disorder of unknown aetiology, commonly affecting the lungs, skin, and eyes.

It is characterised by accumulation of lymphocytes and macrophages and the formation of non-caseating granulomas in the lungs and other organs.

Although lungs and lymph nodes are involved in more than 90% of patients, virtually any organ can be involved.

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2
Q

Describe the aetiology of sarcoidosis

A

Unknown aeitology:

Infectious agents
- (mycobacteria and propionibacteria)

Environmental and occupational exposures:
- inorganic particles, organic antigens, and mould spores
- exposure to dust, metals, or pesticides

Genetic predisposition

Ethnicity
- Sarcoidosis is around 3 times more common in people of Black African ethnicity.

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3
Q

Describe the pathophysiology of sarcoidosis

A

Sarcoidosis is characterized by an exaggerated immune response, leading to the formation of non-caseating granulomas.:

Antigen presentation
- In genetically susceptible individuals, exposure to a yet unidentified antigenic stimulus triggers the activation of antigen-presenting cells (APCs), such as dendritic cells and macrophages, which present the antigen to CD4+ T-helper cells.

T cell activation:
- APCS & CD4+ Thelpers interacting causes differentation of T helpers into Th1 and Th17s
- Th1 cells: secrete IFN-y & IL-2
- Th17 cells secrete IL-17; IL2-21 & IL-22

Granuloma formation:
- All the cytokines recruit macrophages
- Macrophages transfrom into multinucleated giant cells and create non-causeating granulomas
- The granulomas isolate the antigenic stimulus; limiting tissue damage

Fibrosis:
- In severe cases, sarcoid granulomas lead to fibrosis and therefore organ failure and dysfunction

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4
Q

TOM TIP: The typical MCQ exam patient for a presentation of sarcoidisis consistents of what presentation? [4]

A

20-40 y/o black women
Dry cough
SOB
Nodules on shin (erythema nodosum)

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5
Q

Which organs are most commonly impacted by sarcoidosis? [3]

A

Red eyes - uveitis
Skin lesions - waxy lumps
Lungs - BHL, pulmonary infiltrates

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6
Q

State what is meant by Lofgren’s syndrome [1]

How does Lofgren’s syndrome usually present? [4]

A

Lofgren’s syndrome is an acute form of the disease characterised by:
- bilateral hilar lymphadenopathy (BHL)
- erythema nodosum
- fever
- polyarthralgia.

It usually carries an excellent prognosis

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7
Q

Explain what is meant by Heerford’ts syndrome [1]

What is the classical presentation? [3]

A

Heerfordt’s syndrome (uveoparotid fever) there is parotid enlargement, fever and uveitis secondary to sarcoidosis

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8
Q

Describe the clinical features of sarcoidosis if each of the following are effected:

  • Skin [3]
  • Lungs [3]
  • Systemically [3]
A

Skin:
- Erythema nodosum - raised, red, tender painful subcut nodules across both shins. Over time they appear as bruises
- Papular sarcoidosis: multiple papules develop, generally on the head and neck or areas of trauma.
- Lupus pernio: specific to sarcoidosis and presents with raised purple skin lesions, often on the cheeks and nose.

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9
Q

What is the name for this symptom of sarcoidosis

Papular sarcoidosis
Lupus pernio
Erythema nodosum
Lofgrens syndrome

A

What is the name for this symptom of sarcoidosis

Papular sarcoidosis
Lupus pernio
Erythema nodosum
Lofgrens syndrome

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10
Q

What is the name for this symptom of sarcoidosis

Papular sarcoidosis
Lupus pernio
Erythema nodosum
Lofgrens syndrome

A

What is the name for this symptom of sarcoidosis

Papular sarcoidosis
Lupus pernio
Erythema nodosum
Lofgrens syndrome

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11
Q

What is the name for this symptom of sarcoidosis

Papular sarcoidosis
Lupus pernio
Erythema nodosum
Lofgrens syndrome

A

What is the name for this symptom of sarcoidosis

Papular sarcoidosis
Lupus pernio
Erythema nodosum
Lofgrens syndrome

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12
Q

What is the name for this symptom of sarcoidosis

Papular sarcoidosis
Lupus pernio
Erythema nodosum
Lofgrens syndrome

A

What is the name for this symptom of sarcoidosis

Papular sarcoidosis
Lupus pernio
Erythema nodosum
Lofgrens syndrome

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13
Q

Describe the manifestation of sarcoidosis if the effects the following systems:

Pulmonary [3]
Liver [5]
Systemic [3]

A

Lungs:
* Mediastinal lymphadenopathy
* Pulmonary fibrosis
* Pulmonary nodules

Liver:
* Liver nodules
* Cirrhosis
* Pruritis
* Jaundice
* Cholestasis

Systemic Symptoms:
* Fever
* Fatigue
* Weight loss

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14
Q

Describe the manifestation of sarcoidosis if the effects the following systems:

CNS [3]
Eyes [3]
Heart [3]

A

Eyes:
* Uveitis
* Conjunctivitis
* Optic neuritis

Heart:
* Bundle branch block
* Heart block
* Myocardial muscle involvement

Central nervous system:
* Nodules
* Pituitary involvement (diabetes insipidus)
* Encephalopathy

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15
Q

Describe the manifestation of sarcoidosis if the effects the following systems:

PNS [2]
Bones [3]
Kindey [3]

A

Kidneys:
* Kidney stones (due to hypercalcaemia)
* Nephrocalcinosis
* Interstitial nephritis

Peripheral Nervous System:
* Facial nerve palsy
* Mononeuritis multiplex

Bones:
* Arthralgia
* Arthritis
* Myopathy

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16
Q

Which population is more at risk of suffering from sarcoidosis? [1]

A

if from Scandinavian origin.

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17
Q

Which symptoms would indicate that a patient is sufferinf from uveitis? [3]

A

Photophobia
Red painful eye
Blurred vision

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18
Q

Describe the CXR that would suggest sarcoidosis [5]

A
  • bilateral hilar lymphadenopathy
  • right paratracheal adenopathy
  • bilateral pulmonary infiltrates, predominantly in the upper lobes
  • pleural effusions (rare)
  • egg shell calcifications (very rare)

Chest x-ray demonstrates upper zone reticulonodular opacities with extensive mediastinal and hilar nodal enlargement. Stage II sarcoidosis with both nodal and parenchymal disease.

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19
Q

What are the red circles outlining in this CXR of a ptx with sarcoidosis? [1]

A

Egg shell calcifications

Figure. PA and lateral chest x-rays demonstrate bilateral egg shell calcification of the hilar lymph nodes. See red circles.

20
Q

State the 5 different CXR stages of sarcoidosis [5]

A

A chest x-ray may show the following changes:

  • stage 0 = normal
  • stage 1 = bilateral hilar lymphadenopathy (BHL)
  • stage 2 = BHL + interstitial infiltrates (granulomas in the lungs)
  • stage 3 = diffuse interstitial infiltrates only
  • stage 4 = diffuse fibrosis
21
Q

Which stage of sarcoidosis is depicted in this CXR?

Stage 0
Stage 1
Stage 2
Stage 3
Stage 4

A
  • stage 0 = normal
  • stage 1 = bilateral hilar lymphadenopathy (BHL)
  • stage 2 = BHL + interstitial infiltrates
  • stage 3 = diffuse interstitial infiltrates only
  • stage 4 = diffuse fibrosis
22
Q

Which stage of sarcoidosis is depicted in this CXR?

Stage 0
Stage 1
Stage 2
Stage 3
Stage 4

A

stage 1: bilateral hilar lymphadenopathy
Bilateral hilar lymphadenopathy. No lung infiltrations are shown. No fibrosis.

23
Q

Which stage of sarcoidosis is depicted in this CXR?

Stage 0
Stage 1
Stage 2
Stage 3
Stage 4

A

Which stage of sarcoidosis is depicted in this CXR?

Stage 0
Stage 1
Stage 2
Stage 3
Stage 4

24
Q

Which stage of sarcoidosis is depicted in this CXR?

Stage 0
Stage 1
Stage 2
Stage 3
Stage 4

A

Which stage of sarcoidosis is depicted in this CXR?

Stage 0
Stage 1
Stage 2
Stage 3
Stage 4

There is diffuse reticulonodular
pattern, the lung parenchyma is distorted by fibrosis, and
the right hilum is retracted due to right upper lobe fibrosis

25
Q

Which stage of sarcoidosis is depicted in this CXR?

Stage 0
Stage 1
Stage 2
Stage 3
Stage 4

A

Which stage of sarcoidosis is depicted in this CXR?

Stage 0
Stage 1
Stage 2
Stage 3
Stage 4

Stage 2 sarcoidosis with bilateral hilar and right
paratracheal adenopathy and diffuse reticulonodular disease
pattern in the lungs. The appearance is of fine lines and tiny
nodules.

26
Q

Which stage of sarcoidosis is depicted in this CXR?

Stage 0
Stage 1
Stage 2
Stage 3
Stage 4

A

Which stage of sarcoidosis is depicted in this CXR?

Stage 0
Stage 1
Stage 2
Stage 3
Stage 4

27
Q

Describe why sarcoidosis may lead to hypercalcaemia [1]

A

Hypercalcaemia is seen in around 15% of cases.:
* This occurs due to the uncontrolled synthesis of 1,25-dihydroxyvitamin D3 by macrophages in causing 1-α hydroxylation of 25-hydroxyvitamin D and so increases levels of activated vitamin D
* This leads to increased levels of calcium.

28
Q

Explain change in spirometry results in a patient with sarcoidosis

A

In cases of pulmonary sarcoidosis with pulmonary infiltrates and fibrosis a restrictive lung disease pattern is seen:

  • FVC: reduced
  • FEV1: reduced
  • FEV1/FVC: > 80%
29
Q
A
30
Q

What are two specific blood tests would undertake for sarcoidosis patients? [2]

A
  • Raised angiotensin-converting enzyme (ACE)(often used as a screening test)
  • Raised calcium (hypercalcaemia)
31
Q

What is the definitive investigation for sarcoidosis? [1]

A

Lavage or biopsy

Bronchoalveolar Lavage or BAL is a minimally invasive procedure that involves instillation of sterile normal saline into a subsegment of the lung, followed by suction and collection of the instillation for analysis.

32
Q

Describe the histological structure of a noncaseating granuloma

A

These are collections of macrophages, epithelioid cells, t-lymphocytes (normally CD4 +ve) and giant cells that are noncaseating (or non-necrotising) - that is the centre has not undergone caseating necrosis. This is then surrounded by both T- and B- lymphocytes, mast cells and fibroblasts.

33
Q

Describe the treatment plan for sarcoidosis for asymptomatic patients / mild disease [1]

A

Asymptomatic patients / mild disease:
- may not require treatment, as spontaneous remission is common

34
Q

Describe the treatment plan for sarcoidosis for symptomatic patients / moderate & severe disease [4]

A

First line: Prednisolone:
- High-dose induction: typically 20 - 40mg each day for 4 - 6 weeks
- Dose tapering: the initial dose is gradually reduced (e.g. 5mg every two weeks)
- Maintenance dose: typically 5 - 10mg each day

Second-line agents: classical immunosuppressants
- Methotrexate (first line)
- Azathioprine
- Mycophenolate mofetil.

Third line / refractory disease: biologic therapies
- infliximab
- adalimumab

Lung transplantation

35
Q

What treatment might be given to for treating skin sarcoid? [1]

A

Hydroxychloroquine

36
Q

Why must you screen for latent TB if giving infliximab when treating sarcoidoisis? [1]

A

They are known to trigger the reactivation of latent TB

37
Q

Severe sarcoidosis can lead to which two lung pathologies? [2]

What would indicate lung transplantation for sarcoidosis ptx? [2]

A

Advanced pulmonary fibrosis
Pulmonary hypertension

38
Q

Which patient populations have a higher risk of mortality from sarcoidosis?

A

people of Black African ethnicity

39
Q

What would you prescribe alongside long-term corticosteroids to avoid unwanted side effects of the steroids? [1]

A

Bisphosphonates protect against osteoporosis whilst on long-term steroids.

40
Q

Describe a GUM implication of sarcoidosis [1]

A

Polyuria and polydipsia: increased macrophages caused increaesd Ca2+

41
Q

What serum test may indicate sarcoidosis? [1]

A

Serum ACE (SACE)

42
Q

Describe the diagnosis pathway for sarcoidosis

A
43
Q

Which investigational modality is increasingly used for sarcoidosis? [1]

A

PET scanning

44
Q

A 36-year-old male call centre operator attends his routine outpatient clinic appointment. He has a 5-year history of sarcoidosis and admits to increasing shortness of breath over the past four weeks. This is his fourth episode of this nature since his diagnosis. He has previously responded well to tapered doses of oral steroids.

What initial test would be most helpful before prescribing steroids to assess his current pulmonary status objectively?

Chest X-ray

Pulmonary function tests with transfer factor

Arterial blood gas

Serum angiotensin-converting enzyme (ACE) level

High-resolution computed tomography (HRCT) of the chest

A

Pulmonary function tests with transfer factor

The pulmonary function picture is typically a restrictive pattern (interstitial fibrosis is the underlying pathological process) with a reduced transfer factor. The level of transfer factor is sensitive to exacerbations and treatment of the disease.

45
Q

Raised serum ACE is indicative of sarcoidosis. Name 4 other pathological states where you might see a raised serum ACE [4]

A

Raised serum ACE may also be moderate in human immunodeficiency virus (HIV), histoplasmosis, diabetes mellitus, hyperthyroidism, tuberculosis and leprosy.

46
Q

If a patient is suffering from pneumonia, where would consolidation be to suggest it is aspirational pneumonia? [1]

A

right lower zone of the lung.

47
Q
A

a prolonged PR interval

Year 3: Respiratory (42 decks)

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