Sarcoidosis Flashcards
What is sarcoidosis?
It is defined as a multisystem disorder, characterised by non-caseating granulomatous inflammation
What are granulomas?
They are nodules of inflammation, containing macrophages
What is the aetiology of sarcoidosis?
Unknown
Why do respiratory specialists manage sarcoidosis, despite being a multisystem disorder?
In most cases, sarcoidosis affects the lungs
Is sarcoidosis an obstructive or restrictive lung disease?
Restrictive lung disease
What are the three risk factors of sarcoidosis?
Female Gender
Young Age, 20 – 40 Years Old
Black Ethnicity
What are the clinical features of sarcoidosis?
It is a multisystem disorder that affects almost any organ in the body, therefore the clinical features vary significantly
In exams, what form of sarcoidosis is usually presents?
Lofgren’s syndrome
What are the five clinical features associated with Lofgren’s syndrome?
Respiratory Features
Erythema Nodosum
Lupus Pernio
Bilateral Hilar Lymphadenopathy
Polyarthralgia
What is erythema nodosum?
It is defined as tender, red nodules on the shins
What is the cause of erythema nodosum?
It is caused by inflammation of the subcutaneous fat
What is lupus pernio?
It is defined as rash, consisting of a raised purple plaque
It affects the tip of the nose, checks, lips, ears and the skin around the right nostril
What is bilateral hilar lymphadenopathy?
It s defined as bilateral enlargement of the pulmonary hilla lymph nodes
What is polyarthralgia?
It is defined as joint pain, affecting multiple joint
Which joints are affected by sarcoidosis - small or large?
Large
What four investigations are used to diagnose sarcoidosis?
Blood Tests
Chest X-Ray (CXR)
CT Scan
Tissue Biopsy
What five blood test results indicate sarcoidosis?
Increased Serum ACE Levels
Increased CRP/ESR Levels
Increased Serum Soluble Interleukin-2 Receptor Levels
Hypercalcaemia
Hyperglobulinaemia
Describe why hypercalcaemia is associated with sarcoidosis
This is due to the fact that the macrophages in the granulomas cause an increased conversion of vitamin D to its active form
What CXR feature indicates stage one sarcoidosis?
Bilateral Hilar Lymphadenopathy (BHL)
What two CXR features indicate stage two sarcoidosis?
Bilateral Hilar Lymphadenopathy (BHL)
Interstitial Pulmonary Infiltrates
What CXR feature indicates stage three sarcoidosis?
Diffuse Interstitial Infiltrates
What CXR feature indicates stage four sarcoidosis?
Diffuse Fibrosis
What fibrosis is associated with fibrosis - upper lung zone or lower lung zone?
Upper lung zone
What are the two signs of sarcoidosis on CT scan?
Bilateral Hilar Lymphadenopathy
Pulmonary Nodules
What is the gold standard investigation used to diagnose sarcoidosis?
Tissue biopsy
What tissue is biopsied in pulmonary sarcoidosis? How is this biopsy conducted?
Mediastinal lymph nodes
It is conducted by bronchoscopy, under ultrasound guidance
What histology results indicate sarcoidosis?
Non-caseating granulomas with epithelioid cells
When is no treatment of sarcoidosis recommended? Why?
In individuals who present with mild clinical features
In 60% of cases, sarcoidosis spontaneously resolves
How long does it usually take for sarcoidosis to spontaneously resolve?
6 months
What are the two pharmacological management options for sarcoidosis?
Steroids
Immunosuppressants
What is the first line pharmacological management option used to treat sarcoidosis?
Steroids
What steroid is used to treat sarcoidosis? At what dose? How long is the course?
Oral prednisolone
40mg for 4 -6 weeks
This dose is then gradually reduced over a period of 6 - 24 months
Due to the long term administration of steroids, what do we additionally prescribe patients? Why?
Bisphosphonates
To prevent the development of osteoporosis
What is the second line pharmacological management option used to treat sarcoidosis?
Immunosuppressants
What two immunosuppressants are used to treat sarcoidosis?
Methotrexate
Azathioprine
What surgical management option is used to treat sarcoidosis?
Lung transplant
When is a lung transplant recommended to treat sarcoidosis?
In severe cases of sarcoidosis, in which pulmonary fibrosis and pulmonary hypertension develop
