Sarcoidosis Flashcards

1
Q

What is Sarcoidosis?

A

A (non-cavitating) granulomatous inflammatory multisystem disorder.

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2
Q

What is a Granuloma?

A

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

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3
Q

Aetiology of Granulomas.

A

Unknown.

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4
Q

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

A

In Sarcoidosis, the granulomas do not show caseous necrosis.

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5
Q

Epidemiology of Sarcoidosis.

A
  1. 2 Spikes in Incidence - Young Adulthood (20-40) and Age 60.
  2. Commoner in women.
  3. Commoner in Black people.
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6
Q

Acute Features of Sarcoidosis (4).

A
  1. Erythema Nodosum.
  2. Bilateral Hilar Lymphadenopathy.
  3. Swinging Fever.
  4. Polyarthralgia.
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7
Q

Aetiology of Hypercalcaemia.

A

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

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8
Q

Clinical Presentation of Sarcoidosis.

A
  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%).
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9
Q

Pulmonary Symptoms of Sarcoidosis (3).

A
  1. Mediastinal Lymphadenopathy.
  2. Pulmonary Fibrosis.
  3. Pulmonary Nodules.
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10
Q

Systemic Symptoms of Sarcoidosis (3).

A
  1. Fever.
  2. Fatigue.
  3. Weight Loss.
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11
Q

Hepatic Symptoms of Sarcoidosis (3).

A
  1. Liver Nodules.
  2. Cirrhosis.
  3. Cholestasis.
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12
Q

Ophthalmological Symptoms of Sarcoidosis (3).

A
  1. Uveitis.
  2. Conjunctivitis.
  3. Optic Neuritis.
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13
Q

Dermatological Symptoms of Sarcoidosis (3).

A
  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.
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14
Q

Cardiovascular Symptoms of Sarcoidosis (3).

A
  1. Bundle Branch Block.
  2. Heart Block.
  3. Myocardial Muscle Involvement.
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15
Q

Renal Symptoms of Sarcoidosis (3).

A
  1. Kidney Stones - Hypercalcaemia.
  2. Nephrocalcinosis.
  3. Interstitial Nephritis.
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16
Q

Neurological Symptoms of Sarcoidosis (2).

A
  1. CNS - Nodules, Pituitary Involvement e.g. Diabetes Insipidus, Encephalopathy.
  2. PNS - Facial Nerve (7) Palsy, Mononeuritis Multiplex.
17
Q

Musculoskeletal Symptoms of Sarcoidosis (3).

A
  1. Arthralgia.
  2. Arthritis.
  3. Myopathy.
18
Q

What is Lofgren’s Syndrome?

A

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

  1. Erythema Nodosum.
  2. Bilateral Hilar Lymphadenopathy.
  3. Polyarthralgia.
19
Q

What is Mikulicz Syndrome?

A

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

20
Q

What is Heerfordt’s Syndrome?

A

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

21
Q

Differential Diagnoses of Sarcoidosis (6).

A
  1. TB.
  2. Lymphoma.
  3. Hypersensitivity Pneumonitis.
  4. HIV.
  5. Toxoplasmosis.
  6. Histoplasmosis.
22
Q

Investigations in Sarcoidosis (7).

A
  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.
23
Q

CXR Staging of Sarcoidosis (5).

A
  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.
24
Q

Histology in Sarcoidosis (3).

A
  1. Gold-Standard for Diagnosis.
  2. Bronchoscopy with US-guided biopsy of mediastinal lymph nodes.
  3. Finding : Non-Caseating Granulomas with Epithelioid Cells.
25
Q

Management of Sarcoidosis (4).

A
  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.
26
Q

Indications for Steroids in Sarcoidosis (3).

A
  1. CXR Stage II/III Disease - Symptomatic.
  2. Hypercalcaemia.
  3. Eye, Heart, Neuro Involvement.
27
Q

Prognosis of Sarcoidosis (3).

A
  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.