Sarcoidosis (RESP) Flashcards

1
Q

Define sarcoidosis.

A

Chronic granulomatous multisystem disorder of unknown aetiology

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2
Q

Which body systems are most commonly affected in sarcoidosis? (3)

A
  • lungs
  • skin
  • eyes
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3
Q

What is sarcoidosis a diagnosis of exclusion of?

A

Granulomatous lung diseases, including TB and histoplasmosis

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4
Q

What is sarcoidosis characterised by?

A

Accumulation of lymphocytes and macrophages

Formation of non-caseating granulomas (collection of histiocytes) in the lungs and other organs

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5
Q

What body systems are involved in >90% of patients with sarcoidosis?

A

Lungs and lymph nodes

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6
Q

Describe the epidemiology of sarcoidosis.

A
  • F>M
  • 10x more likely in African-Americans
  • bimodal peak incidence:
    • 25-35 years old
    • 50-65 years old
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7
Q

What is the aetiology of sarcoidosis?

A

Unknown - hypotheses suggest genetic, immunological and infectious causes

HLA-DRB1 and DQB1 alleles

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8
Q

Describe the pathogenesis of sarcoidosis.

A
  • unknown antigen presented on MHC-II complexes on macrophages to CD4+ T lymphocytes
  • these accumulate and release cytokines
  • leads to formation of non-caseating granulomas in organs
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9
Q

What are the clinical features of sarcoidosis? (12)

A
  • chronic dry cough
  • dyspnoea
  • wheezing
  • rhonchi
  • swinging fever
  • chronic fatigue
  • facial nerve (Bell’s) palsy
  • arthralgia - pain in knees, ankles and wrists
  • erythema nodosum - rash on shin
  • lupus pernio - indurated purple rash on face, nose, ears
  • lymphadenopathy - cervical and submandibular lymph nodes
  • anterior uveitis - red painful eye, photophobia, blurred vision
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10
Q

What pulmonary symptoms may be seen in sarcoidosis? (4)

A
  • chronic dry cough
  • dyspnoea (SOB)
  • wheezing
  • rhonchi
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11
Q

What skin symptoms may be seen in sarcoidosis? (2+1)

A
  • lupus pernio - purple indurated rash of face, nose and ears (and terminal phalanges)
  • erythema nodosum - rash on shin
  • (maculopapular eruptions)
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12
Q

What eye symptoms are seen in sarcoidosis? (3+3)

A

Anterior uveitis:

  • red painful eye
  • photophobia
  • blurred vision

(keratoconjunctivitis sicca, glaucoma, papilloedema)

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13
Q

What cardiac symptoms may be seen in sarcoidosis? (5)

A
  • arrhythmias
  • heart block
  • pericarditis
  • cardiomyopathy
  • congestive heart failure
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14
Q

What symptom is pathognomonic for sarcoidosis?

A

Lupus pernio - indurated purple rash on face, nose and ears

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15
Q

What electrolyte disturbance can occur in sarcoidosis and why?

A

Hypercalcaemia - granuloma produces ectopic 1-alpha-hydroxylase, macrophages inside the granulomas cause an increased conversion of vitamin D to its active form (1,25-dihydroxycholecalciferol)

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16
Q

What are some risk factors for sarcoidosis? (4)

A
  • age 20-50 years (binomial age distribution with 2 peaks 25-35 and 50-65)
  • Fx sarcoidosis
  • Scandinavian and African origin
  • female sex
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17
Q

What are the first-line investigations for sarcoidosis? (7)

A
  • CXR
  • FBC
  • serum urea
  • serum creatinine
  • PFTs
  • LFTs
  • serum calcium
18
Q

What is the best initial test for sarcoidosis?

A

CXR

19
Q

What does CXR show in sarcoidosis? (2)

A

Bilateral hilar lymphadenopathy

Bilateral pulmonary infiltrates, predominantly in upper lobes

(Pleural effusions - rare)
(Eggshell calcifications - very rare)

20
Q

What are the stages of sarcoidosis by CXR? (Correlates with likelihood of spontaneous resolution)

A
  • stage 0 - normal
  • stage I - bilateral hilar lymphadenopathy
  • stage II - bilateral hilar lymphadenopathy PLUS pulmonary infiltrates (and paratracheal node enlargement)
  • stage III - pulmonary infiltrates WITHOUT hilar lymphadenopathy
  • stage IV - extensive fibrosis with distortion (honeycombing/bulla formation, pleural involvement)
21
Q

What investigation can be done if suspicious CXR for sarcoidosis?

A

High resolution CT (check for diffuse lung involvement)

22
Q

What is the gold-standard investigation for sarcoidosis diagnosis?

A

Bronchoscopy with lung biopsy –> showing non-caseating granulomas

23
Q

What does FBC show in sarcoidosis? (2)

A
  • anaemia
  • leukopenia - due to lymphocyte sequestration in lungs
24
Q

What would U&Es show in sarcoidosis? (2)

A
  • hypercalcaemia (macrophages in granuloma increase 1,25-dihydroxycholecalciferol as granulomatous tissue produces ectopic 1a-hydroxylase)
  • urea and creatinine elevated if renal involvement
25
Q

What does spirometry/PFTs show in sarcoidosis?

A

Restrictive defect

  • low FEV1
  • FVC shows restrictive/obstructive/mixed picture
  • persistent decline in FVC indicates disease progression
26
Q

What do LFTs show in sarcoidosis?

A

High ALP and GGT

27
Q

What enzyme is raised in sarcoidosis?

A

Raised serum ACE

28
Q

What are some differential diagnoses for sarcoidosis? (6)

A
  • TB - caseating granulomas
  • histoplasmosis - fungus isolation on silver stain from lung biopsy
  • NSCLC - smoking Hx
  • lymphoma - extra-thoracic involvement
  • berylliosis - exposure in nuclear and aerospace industries
  • hypersensitivity pneumonitis
29
Q

What syndromes are associated with sarcoidosis? (3)

A
  • Lofgren’s syndrome - acute form of disease characterised by bilateral hilar lymphadenopathy, erythema nodosum, fever and polyarthralgia
  • Mikulicz syndrome - enlarged parotid and lacrimal glands due to sarcoidosis, TB or lymphoma
  • Heerfordt’s syndrome (uveoparotid fever) - parotid enlargement, fever and uveitis secondary to sarcoidosis
30
Q

What are the indications for treatment of sarcoidosis? (4)

A
  • CXR stage 2/3+ and symptomatic
  • hypercalcaemia
  • eye, heart or neurological involvement
  • deteriorating PFTs/CXR changes
31
Q

Give an example of a patient we would not give corticosteroid treatment to with sarcoidosis?

A

Patient with asymptomatic bilateral hilar lymphadenopathy

32
Q

What is the first-line treatment for symptomatic sarcoidosis?

A

Corticosteroids - prednisolone, (hydrocortisone)

33
Q

What other medications can we give for sarcoidosis, alongside corticosteroids? (2)

A
  • immunosuppressive therapy: methotrexate, azathioprine (check thiopurine methyltransferase [TPMT ] deficiency first)
  • antimalarial drugs - chloroquine, hydroxychloroquine
34
Q

What is given for symptomatic relief in sarcoidosis?

A

NSAIDs

35
Q

What is the last resort in severe sarcoidosis?

A

Lung transplant

36
Q

How do we manage the different stages of pulmonary disease in sarcoidosis?

A
  • stage I asymptomatic - no treatment
  • stage I symptomatic - oral/inhaled prednisolone
  • stage II/III/IV - oral/inhaled prednisolone, methotrexate (cytotoxic), oxygen
  • end stage - lung transplant
37
Q

How do we manage lupus pernio with scarring in sarcoidosis?

A

Prednisolone + methotrexate/azathioprine

38
Q

How do we manage acute respiratory failure in sarcoidosis?

A

IV/oral methylprednisolone + ventilatory support and oxygen

39
Q

What are some complications of sarcoidosis?

A
  • increased risk of malignancy
  • bronchiectasis
  • lung fibrosis
  • chronic renal failure
  • haemoptysis
  • glaucoma
  • corticosteroid-related conditions (hyperglycaemia, osteoporosis, infection)
  • cytotoxic-related conditions (hepatic toxicity, marrow toxicity)
40
Q

What indicates poorer prognosis of sarcoidosis?

A

Chronic hypercalcaemia (renal failure), chronic pulmonary involvement, lupus pernio, black ancestry

41
Q

Describe the % of spontaneous remissions in different stages of sarcoidosis.

A
  • stage I 55-90%
  • stage II 40-70%
  • stage III 20%
  • stage IV - remission not expected