Sarcoidosis

Question 1

What is Sarcoidosis?

Answer 1

A (non-cavitating) granulomatous inflammatory multisystem disorder.

Question 2

What is a Granuloma?

Answer 2

A nodule of inflammation full of macrophages - an aggregate of activated macrophages.

Question 3

Aetiology of Granulomas.

Answer 3

Unknown.

Question 4

What is the key difference between Granulomas in TB and Granulomas in Sarcoidosis?

Answer 4

In Sarcoidosis, the granulomas do not show caseous necrosis.

Question 5

Epidemiology of Sarcoidosis.

Answer 5

1. 2 Spikes in Incidence - Young Adulthood (20-40) and Age 60.
2. Commoner in women.
3. Commoner in Black people.

Question 6

Acute Features of Sarcoidosis (4).

Answer 6

1. Erythema Nodosum.
2. Bilateral Hilar Lymphadenopathy.
3. Swinging Fever.
4. Polyarthralgia.

Question 7

Aetiology of Hypercalcaemia.

Answer 7

Macrophages inside the Granuloma cause increased conversion of Vitamin D to its active form (1,25-Dihydroxycholecaliferol).

Question 8

Clinical Presentation of Sarcoidosis.

Answer 8

1. Variation from Asymptomatic (50%) - Severe and Life-Threatening.
2. Most Commonly Affected Organ - Lungs (90%).
3. Other : Liver (20%), Eyes (20%); Skin (15%); Heart (5%); Kidneys (5%); Nerves (5%); Bones (2%).

Question 9

Pulmonary Symptoms of Sarcoidosis (3).

Answer 9

1. Mediastinal Lymphadenopathy.
2. Pulmonary Fibrosis.
3. Pulmonary Nodules.

Question 10

Systemic Symptoms of Sarcoidosis (3).

Answer 10

1. Fever.
2. Fatigue.
3. Weight Loss.

Question 11

Hepatic Symptoms of Sarcoidosis (3).

Answer 11

1. Liver Nodules.
2. Cirrhosis.
3. Cholestasis.

Question 12

Ophthalmological Symptoms of Sarcoidosis (3).

Answer 12

1. Uveitis.
2. Conjunctivitis.
3. Optic Neuritis.

Question 13

Dermatological Symptoms of Sarcoidosis (3).

Answer 13

1. Erythema Nodosum (tender red nodules on shin, caused by inflammation of the subcutaneous fat).
2. Lupus Pernio (raised purple skin lesions commonly on cheeks and nose).
3. Granulomas develop in Scar Tissue.

Question 14

Cardiovascular Symptoms of Sarcoidosis (3).

Answer 14

1. Bundle Branch Block.
2. Heart Block.
3. Myocardial Muscle Involvement.

Question 15

Renal Symptoms of Sarcoidosis (3).

Answer 15

1. Kidney Stones - Hypercalcaemia.
2. Nephrocalcinosis.
3. Interstitial Nephritis.

Question 16

Neurological Symptoms of Sarcoidosis (2).

Answer 16

1. CNS - Nodules, Pituitary Involvement e.g. Diabetes Insipidus, Encephalopathy.
2. PNS - Facial Nerve (7) Palsy, Mononeuritis Multiplex.

Question 17

Musculoskeletal Symptoms of Sarcoidosis (3).

Answer 17

1. Arthralgia.
2. Arthritis.
3. Myopathy.

Question 18

What is Lofgren’s Syndrome?

Answer 18

A specific presentation of Sarcoidosis with an excellent prognosis - a triad of :

1. Erythema Nodosum.
2. Bilateral Hilar Lymphadenopathy.
3. Polyarthralgia.

Question 19

What is Mikulicz Syndrome?

Answer 19

Enlargement of the parotid and lacrimal glands due to sarcoidosis, tuberculosis or lymphoma.

Question 20

What is Heerfordt’s Syndrome?

Answer 20

Uveoparotid Fever - parotid enlargement, fever, uveitis (secondary to sarcoidosis).

Question 21

Differential Diagnoses of Sarcoidosis (6).

Answer 21

1. TB.
2. Lymphoma.
3. Hypersensitivity Pneumonitis.
4. HIV.
5. Toxoplasmosis.
6. Histoplasmosis.

Question 22

Investigations in Sarcoidosis (7).

Answer 22

1. Bloods : Raised ACE (screening test), Calcium, serum-soluble IL-2 Receptor, CRP, Immunoglobulins.
2. CXR : Hilar Lymphadenopathy.
3. High-Resolution CT : Hilar Lymphadenopathy and Pulmonary Nodules.
4. MRI : CNS Involvement.
5. PET : Active Inflammation in Affected Areas.
6. Histology.
7. Spirometry : Restrictive Defect.

Question 23

CXR Staging of Sarcoidosis (5).

Answer 23

1. Stage 0 = Normal.
2. Stage I - Bilateral Hilar Lymphadenopathy.
3. Stage II - BHL with Interstitial Infiltrates.
4. Stage III - Diffuse Interstitial Infiltrates Only.
5. Stage IV - Diffuse Fibrosis.

Question 24

Histology in Sarcoidosis (3).

Answer 24

1. Gold-Standard for Diagnosis.
2. Bronchoscopy with US-guided biopsy of mediastinal lymph nodes.
3. Finding : Non-Caseating Granulomas with Epithelioid Cells.

Question 25

Management of Sarcoidosis (4).

Answer 25

1. Asymptomatic/Mild - No Treatment (spontaneous resolution). 2. Oral Steroids - 1st line for 6-24 months (with Bisphosphonates). 3. 2nd Line : Methotrexate, Azathioprine. 4. Lung Transplant : Rare in Severe Pulmonary Disease.

Question 26

Indications for Steroids in Sarcoidosis (3).

Answer 26

1. CXR Stage II/III Disease - Symptomatic. 2. Hypercalcaemia. 3. Eye, Heart, Neuro Involvement.

Question 27

Prognosis of Sarcoidosis (3).

Answer 27

1. Spontaneous resolution in around 60% patients within 6 months. 2. Rarely : Progression to Pulmonary Fibrosis and Pulmonary Hypertension. 3. Death : Heart or CNS Involvement.