Sarcoidosis

Question 1

What is sarcoidosis?

Answer 1

• multisystem granulomatous disorder

Question 2

What are the clinical signs of Sarcoidosis?

Answer 2

• Progressive breathlessness
• Fine inspiratory crackles
• Exertional desaturations
• Clubbing
• Pulmonary hypertension
• Cor pulmonale

Question 3

What are the extrapulmonary manifestations of sarcoidosis?

Answer 3

• occular sarcoidosis (30-60%)
  
  • uveitis
  • keratojunctivitis sicca
  • Secondary glaucoma
• Cutaneous sarcoidosis
  
  • Papular sarcoidosis
  • erythema nodosum
• Hypercalcaemia
• Renal disease - interstitial nephritis
• CNS disease
  *

Question 4

What Ix would you order for sarcoidosis?

Answer 4

Bedside

• Obs
• Spirometry - restrictive
• Mantoux test

Bloods

• FBC
• eGFR
• ESR
• Bone profile
• Serum ACE (raised)

Imaging

• CXR - to stage disease

Special test

• Bronchoalveolar lavage + biopsy

Question 5

What CXR features are seen for sarcoidosis?

Answer 5

• bilateral lymphadenopathy
• reticulonodular opacities
• airspace opacities
• pulmonary fibrosis

Question 6

What features will be shown on CT?

Answer 6

• Lymphadenopathy
• Diffuse nodularity
• Ground glass opacification
• Fibrosis typically affecting

Question 7

How would you diagnose sarcoidosis?

Answer 7

1. Clinically diagnosed if
   
   • Lofgren’s syndrome
   • Long-standing pulmonary disease: with a classical initial presentation, with no suspicion of other diagnoses,
2. Tissue biopsy
   
   • Bronchoalveolar lavage + transbronchial biopsy
   • Non caseating granuloma

Question 8

What staging is used to stage pulmonary sarcoidosis based on CXR?

Answer 8

• Scadding staging

Question 9

Dsecribe the scadding staging.

*stage 0-4

Answer 9

Question 10

When should tx be started in sarcoidosis?

Answer 10

• Potential danger of a fatal outcome or permanent disability or
• Unacceptable loss of quality of life

Question 11

What are the pharmacological tx for sarcoidosis?

Answer 11

• Prednisolone (first line)
  
  • 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
• immunosuppressants (second line)
  
  • Methotrexate (weekly) + folic acid
• Biologics (third line)
  
  • Infliximab (TNF agent)

Question 12

When would you consider lung transplant in sarcoidosis?

Answer 12

• Advanced pulmonary fibrosis
• Pulmonary hypertension

Question 13

What is the prognosis for sarcoidosis?

Answer 13

• majority - spontaneous regression and resolution