Sarcoidosis Flashcards Preview

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Flashcards in Sarcoidosis Deck (6)
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1

Sarcoidosis is a multisystem disorder of unknown aetiology characterised by non-caseating granulomas. It is more common in young adults and in people of African descent.

What are the clinical features of sarcoidosis?

  • Acute → erythema nodosum, bilateral hilar lymphadenopathy, swinging fever, polyarthralgia
  • Insidious → dyspnoea, non-productive cough, malaise, weight loss
  • Skin → lupus pernio
  • Hypercalcaemia → macrophages inside the granulomas cause an increased conversion of vitamin D to its active form (1,25-dihydroxycholecalciferol) 

Two spikes in incidence, in young adulthood and again around age 60. Typical MCQ exam patient is 20-40 y/o black woman presenting w/ dry cough and SoB. They may have nodules ontheir shins suggesting erythema nodosum.

2

What are symptoms affecting each particular system for sarcoidosis?

  • Lungs → mediastinal lymphadenopathy, pulm fibrosis + nodules
  • Systemic → fever, fatigue, weight loss
  • Liver → liver nodules, cirrhosis, cholestasis
  • Eyes → uveitis, conjunctivitis, optic neuritis
  • Skin → erythema nodosum, lupus pernio, granulomas
  • Heart → bundle branch block, heart block, myocardial muscle involvement
  • Kidneys → kidneys, nephrocalcinosis, interstitial nephritis
  • CNS → nodules, pituitary involvement, encephalopathy
  • PNS → facial nerve palsy, mononeuritis multiplex
  • Bones → arthralgia, arthritis, myopathy

These are in order of most common to least common (top to bottom)

3

What is Lofgren's syndrome?

There is a specific presentation of sarcoidosis, triad:

  • Erythema nodosum
  • Bilateral hilar lymphadenopathy
  • Polyarthralgia 

4

How is sarcoidosis investigated?

  • diagnosis largely clinical
  • ACE levels 60% sensitivity + 70% specificity
    • not reliable in diagnosis but role in monitoring
  • routine bloods may show hypercalcaemia + raised ESR
  • CXR may show:
    • stage 0 = normal
    • stage 1 = bilateral hilar lymphadenopathy
    • stage 2 = BHL + interstitial infiltrates
    • stage 3 = diffuse interstitial infiltrates only
    • stage 4 = diffuse fibrosis 
  • spirometry may show restrictive defect
  • tissue biopsy shows non-caseating granulomas

5

What is the gold standard for confirming diagnosis of sarcoidosis?

  • Histology from biopsy
  • Done by bronchoscopy w/ USS guided biopsy of mediastinal lymph nodes
  • Histology shows non-caseating granulomas + epithelioid cells

6

No treatment is considered as first line in patients with no or mild symptoms as the condition often resolves spontaneously.

What is the management of sarcoidosis?

Steroids indicated if:

  • Pts w/ CXR stage 2 or 3 disease who have moderate-severe or progressive symptoms. Pts w/ asymptomatic + stable stage 2 or 3 disease who have only mildly abnormal lung function do not require treatment
  • Hypercalcaemia
  • Eye, heart or neuro involvement

Give bisphosphonates for bone protection too. Second line includes methotrexate and azathioprine. Lung transplant in severe disease.