Sarcoidosis

Question 1

What is Sarcoidosis?

Answer 1

Is a chronic, multisystemic, immunological granulomatous disorder of unknown aetiology, commoly affecting the

1. Lungs, skin and eyes

Question 2

What are the pathological characteristics of sarcoidosis?

Answer 2

• Accumulation of lymphocytes and macrophages
  
  • Formation of non-caseating granulomas in lung + other organs
• Affected site
  
  • Lung and lymph nodes (90% of patient)
  • Any other organ can be involved
• Clincal course often heterogeneous and unpredictable

Question 3

What is the aetiology of Sarcoidosis?

Answer 3

Unknown

• immunological disease
  *

Question 4

What are risk factors for the development of sarcoidosis?

Answer 4

• Age 20-40
• FHx of sarcoidosis
• Scandinavian origin, African American
• (Female sex,

Question 5

What is the epidemiology of Sarcoidosis?

Question 6

What is the pathophysiological process in sarcoidosis?

Answer 6

Non-caseating (no necrosis at centre of granuloma) granulomas with multinucleated giant cells (fusion of several macrophages) in the centre

• CD4+ und CD8+, CD4 secrete interleukins, maintain and promote granulomas
• Mostly form in hilar lymph nodes
  
  • Bilateral hilar lymphadenopathy
• Also other involvements:
  
  1. Erythema nodosum
  2. Uveitis
  3. Affecting heart (and other sites)

Question 7

What is Acute sarcoidosis?

Answer 7

(onset within 2 years)

Question 8

What is chronic sarcoidosis?

Answer 8

Chronic (gradual disease course, recurrent or progressive)

Question 9

What are the signs and symptoms of acute sarcoidosis?

Answer 9

1. Systemic:
   
   1. Fever, lethargy, loss of appetite, weight loss
2. Pulmonary:
   
   1. Dyspnoea, non-productive cough, thoracic pain
   2. Bilateral hilar lymphadenopathy
3. Polyarthritis
4. Erythema nodosum
5. Anterior uveitis

Question 10

What are the signs and symptoms of chronic sarcoidosis?

Answer 10

• Dependant on affected organ
• Lung
  
  • Non-productive cough
  • Gradual onset SOB on exertion
  • Wheezing and rhonchi (bronchospasm, airway hyper-responsiveness)
• Extrapulmonary sarcoidosis
  
  • Peripheral lymph node involvement, non-tender (in 40% )
  • Eye: Conjunctivitis/Uveiits (photobia/blurred vision)
  • Skin:
    
    • Lupus Pernio
    • Scar Sarcoidosis
• Cardiac involvement
  
  • Restrictive cardiomyopathy, pericardial effusion, AV block, sudden cardiac death
• Joint involvement : arthralgia (no swelling, knee ankle and wrist, similar to RA)
• Nervous system
  
  • Cranial Nerve Palsy
  • Diabetis Insipidus
  • Meningitis
  • Hypopituitarism
• Liver
  
  • Hepatic granulomas, hepatomegaly (30%9
  • Splenomegaly (30%)
• Kidneys
  
  • Related to calcium metabolism

Question 11

What is a mnemonic for remembering signs and symptoms of Sarcoidosis?

Question 12

Which invesitigationsn would you do in a patient with suspeced sarcoidosis?

Answer 12

• Diagnosis of exclusion:
  
  • Imaging
    
    • • Bloods
    • Hypercalcaemia due to increased VItD produced by macrophages)
    • Increase in ACE
    • Increased inflamatory markers
    • Increased IgG
    • Organ involvement (Liver funtion: elevated ALP, U&E: renal involvement)
  • Broncheoalveolar lavage
    
    • Increased T-cells
  • Biopsy
  • Pulmonary function test

Question 13

Which CXR finding would you suspect in a patient with suspected sarcoidosis?

Answer 13

Though systemic disease, 90% pulmonary involvement

• Bilateral Hilar Lymphadenopathy