Respiratory - Sarcoidosis

Question 1

What is Sarcoidosis?

Answer 1

A granulomatous inflammatory condition.

Granulomas are nodules of inflammation full of macrophages.

Question 2

How does sarcoidosis present?

Answer 2

Usually associated with pulmonary symptoms

But has multiple extra-pulmonary manifestations

Symptoms can vary dramatically from asymptomatic (in up to 50%) to severe and life-threatening.

Question 3

Incidence and prevalence of sarcoidosis

Answer 3

Two spikes in incidence (in young adulthood and again around aged 60

Women and people of black ethnicity more commonly affected

Question 4

Organs affected by sarcoidosis

Answer 4

Most to least common
Lungs - >90% patients
Systemic symptoms
Liver - 20% 
Eyes - 20%
Skin - 15%
Heart - 5%
Kidneys - 5%
CNS - 5%
PNS - 5%
Bones - 2%

Question 5

Sarcoidosis lung problems

Answer 5

Mediastinal lymphadenopathy

Pulmonary fibrosis

Pulmonary nodules

Question 6

Systemic symptoms of sarcoid

Answer 6

Fever
Fatigue
Weight loss

Question 7

Liver problems in sarcoid

Answer 7

Liver nodules
Liver cirrhosis
Cholestasis (slowing of bile through bile duct)

Question 8

Eye problems in sarcoidosis

Answer 8

Uveitis
Conjunctivitis
Optic neuritis (also associated with MS)

Question 9

Skin problems in sarcoidosis

Answer 9

Erythema nodosum - tender red nodules on shins (due to inflammation of subcutaneous fat)

Lupus pernio - raised purple skin lesions that typically affect tip of nose and the cheeks

Granulomas that appear in scar tissue

Question 10

Heart problems in sarcoidosis

Answer 10

BBB
Heart block
Heart failure due to granulomas in cardiac muscle

Question 11

Kidney problems in sarcoidosis

Answer 11

Kidney stones due to hypercalcaemia

Nephrocalcinosis - calcium deposits in the kidneys

Interstitial nephritis

Question 12

NS problems and bone problems in sarcoidosis

Answer 12

• Nodules in the CNS
• Pituitary involvement - can lead to Diabetes insipidus
• Encephalopathy due to inflammation
• Bells palsy
• Mononeuritis multiplex
• Arthralgia
• Arthritis
• Myopathies

Question 13

What is Lofgren’s syndrome?

Answer 13

A specific presentation in sarcoid

Triad - erythema nodosum, bilateral hilar LNs raised and polyarthralgia

Question 14

Differential diagnoses

Answer 14

TB
Lymphoma
Hypersensitivity pneumonitis
HIV
Toxoplasmosis
Histoplasmosis

Question 15

Investigations for suspected sarcoid

Answer 15

Blood tests:

• Serum ACE (raised in sarcoidosis)
• Serum calcium (often raised)
• Soluble IL-2 receptors (can be raised in sarcoidosis)
• Inflammatory markers - CRP, IGs can be raised

Imaging - CXR (can show hilar lymphadenopathy),

• High resolution CT - can also show more detail and pulmonary nodules
• MRI for any CNS involvement
• PET-CT - can show active areas of inflammation

Tests for other organ involvement:

• U&Es for kidney involvement
• Urine dipstick or urine albumin-creatinine ratio to look for proteinuria indicating nephritis
• LFTs for liver involvement
• Ophthalmology review for eye involvement
• ECG and echocardiogram for heart involvement
• Ultrasound abdomen for liver and kidney involvement

Question 16

Diagnosing sarcoidosis

Answer 16

Biopsy and histology is gold standard

• Could biopsy skin if that is affected
• Bronchoscopy with US guided biopsy of mediastinal LNs

The histology shows characteristic non-caseating granulomas with epithelioid cells.

Question 17

Treatment of sarcoidosis

Answer 17

No treatment is considered as first line in patients with no or mild symptoms as the condition often resolves spontaneously.

Oral steroids are usually first line where treatment is required and are given for between 6 and 24 months. Plus bisphosphonates to protect against osteoporosis

Second line options are methotrexate or azathioprine

Lung transplant is rarely required in severe pulmonary disease

Question 18

Prognosis in sarcoidosis

Answer 18

Spontaneously resolves in roughly 60% within 6 months

Some patients progress to pulmonary fibrosis and pulmonary hypertension

Death in this disease is usually due to heart or CNS involvement