Sarcoidosis

Question 1

Sarcoidosis

Answer 1

Chronic granulomatous disorder of unknown cause

Question 2

Type of granulomas

Answer 2

Non-caseating granulomas

Question 3

Type of Hypersensitivity

Answer 3

IV - delayed hypersensitivity

Question 4

Common systems involved

Answer 4

Lungs, lymph nodes, joints, liver, skin, eyes

Question 5

What causes tissue damage & fibrosis?

Answer 5

Persistent immune activation

Question 6

Pathology

Answer 6

Inhalation of unknown antigen => stimulates alveolar macrophages, CD4+ & CD8+ T cells & B cells within lung parenchyma => failure to clear antigen caused persistent stimulation & granuloma formation => Persistent immune activation leads to tissue damage & fibrosis, particularly in upper lobes.

Question 7

Persons affected

Answer 7

• Adults aged 20-40.
• More common in female & Afro-Caribbean.
• Highest in Northern Europe.
• Less common in smokers.

Question 8

Symptoms of Acute Sarcoidosis

Answer 8

• Erythema nodosum.
• Bilateral hilar lymphadenopathy.
• Arthritis.
• Uveitis.
• Parotitis.
• Fever.

Question 9

Symptoms of Chronic Sarcoidosis

Answer 9

• Lung infiltrates (alveolitis).
• Skin infiltrates.
• Peripheral lymphadenopathy.
• Hypercalcaemia.

Question 10

General Symptoms

Answer 10

• Weight loss.
• Malaise.
• Polyurethaning.
• Rash.
• Bihilar lymphadenopathy.
• Polyarthiritis.
• Erythema nodosum.

Question 11

Respiratory Symptoms

Answer 11

• Dry cough.
• Progressive dyspnoea.
• Reduced exercise tolerance.
• Chest pain.

Question 12

Investigations

Answer 12

CXR, spirometry, DCLO, CT lungs, Tissue biopsy, blood test.

Question 13

CXR

Answer 13

• Bilateral hilar lymphadenopathy.

- Pulmonary infiltrates or fibrosis.

Question 14

Spirometry

Answer 14

Normal restrictive pattern

Question 15

DCLO

Answer 15

Normal or reduced

Question 16

CT Lungs

Answer 16

Restrictive defect due to lung infiltrates.

Question 17

Blood Test

Answer 17

• ACE levels as activity marker.
• Raised calcium.
• Increased inflammatory markers.

Question 18

Indications for Treatment

Answer 18

• Interstitial lung disease.
• Hypercalcemia.
• Cardiac/neurological involvement/uveitis.

Question 19

Treatment of Acute Sarcoidosis

Answer 19

• Often self-limiting: usually no treatment.

- If vital organ affected: Steroids.

Question 20

Treatment of Chronic Sarcoidosis

Answer 20

• Oral or inhaled steroids usually required.

- Immunosuppression.

Question 21

Management

Answer 21

Monitor CXR & Pulmonary function for several years.