Sarcoidosis

Question 1

What is sarcoidosis?

Answer 1

Sarcoidosis is a multi-system disease characterised by granuloma formation, resulting in widespread inflammatory changes and complications.

Question 2

What organs are affected?

Answer 2

Sarcoidosis can affect almost any organ in the body - mostly lungs

Question 3

Lung symtoms and features

Answer 3

Lungs (affecting over 90%)

Mediastinal lymphadenopathy
Pulmonary fibrosis
Pulmonary nodules

Systemic Symptoms

Fever
Fatigue
Weight loss
Dry cough
Dyspnoea
Reduced exercise tolerance

Examination may reveal crepitations (crackling)

Question 4

Cardiac symptoms

Answer 4

Arrhythmias, restrictive cardiomyopathy.

Question 5

Dermatological symptoms

Answer 5

Erythema nodosum, lupus pernio.

Question 6

What is Lofregen’s Syndrome?

Answer 6

This is a specific presentation of sarcoidosis. It is characteristic by a triad of:

Erythema nodosum
Bilateral hilar lymphadenopathy
Polyarthralgia (joint pain in multiple joints)

Question 7

Blood test results

Answer 7

Raised serum ACE. This is often used as a screening test.

Raised calcium (hypercalcaemia) is a key finding.

Raised serum soluble interleukin-2 receptor

Raised CRP

Raised immunoglobulins

Question 8

Blood test results

Answer 8

Raised serum ACE. This is often used as a screening test.
Hypercalcaemia (rasied calcium) is a key finding.
Raised serum soluble interleukin-2 receptor
Raised CRP
Raised immunoglobulins

Question 9

What does imaging show?

Answer 9

Chest xray shows hilar lymphadenopathy
High-resolution CT thorax shows hilar lymphadenopathy and pulmonary nodules
MRI can show CNS involvement
PET scan can show active inflammation in affected areas

Question 10

Gold standard investigation

Answer 10

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

Question 11

Stages - CXR or CT

Answer 11

Chest x-ray or CT:

Stage 1 - bilateral hilar lymphadenopathy (BHL)
Stage 2 - BHL with peripheral infiltrates
Stage 3 - peripheral infiltrates alone
Stage 4 - pulmonary fibrosis

Question 12

Management

Answer 12

No or mild sympt - nothing as first line

Oral steroids are usually first line where treatment is required and are given for between 6 and 24 months.
Patients should be given bisphosphonates to protect against osteoporosis whilst on such long term steroids.

Second line options are methotrexate or azathioprine

Question 13

Prognosis

Answer 13

Sarcoidosis spontaneously resolves within 6 months in around 60% of patients.

In a small number of patients it progresses with pulmonary fibrosis and pulmonary hypertension, potentially requiring a lung transplant.

Death in sarcoidosis is usually when it affects the heart (causing arrhythmias) or the central nervous system.