Sarcoidosis

Question 1

Definition

Answer 1

Multisystem granulomatous inflammatory disorder

Rare condition that causes small patches of swollen tissue (granulomas), to develop in the organs of the body

Granulomas:
– nodules of inflammation that are full of macrophages

Question 2

Aetiology

Answer 2

Unknown aetiology

Can affect any organ, particularly in the lungs, skin and eyes

There is great geographical variation:
– much more common in Africa compared to England and Japan

Other proposed etiological factors:
– atypical mycobacteriums or fungus’
– Epstein-Barr virus
– occupational, genetic and social environment

Question 3

Epidemiology

Answer 3

Rare condition (19 in 100000 of the UK population)

Peak incidence around 20-30

Family preponderance

2 spikes in incidence- young adulthood and around 60

Fatalities only seen in 1-6% of patients with sarcoidosis

Question 4

Pathophysiology

Answer 4

Granulomas usually contain an accumulation of epithelioid cells, macrophages and lymphocytes (T cells)

Presumably the granulomatous inflammation is a reaction to an unidentified antigen

There is an increased number of cells (particularly CD4 helper cells) in the bronchoalveolar lavage

Transbronchial biopsies show infiltration of the alveolar walls and interstitial spaces with mononuclear cells before granuloma formation

There is no evidence that patients with sarcoidosis suffer from an overall defect in immunity

Question 5

Clinical presentation

Answer 5

Symptoms can vary

50% of cases are asymptomatic

Can affect any organ, particularly the lung, skin and eyes

Lungs:
– cough
– breathlessness
– wheeze
– fine crackles on examination

Skin:
– erythema nodosum
– waxy maculopapular lesions
– lupus pernio (red/blue infiltration of the nose)
– infiltration of scars by granulomas

Eyes:
– anterior and posterior uveitis
– conjunctival nodules

Bone:
– arthralgias or bone cysts

Metabolic:
– hypercalcaemia

Liver:
– granulomatous hepatitis

Neurological:
– meningeal inflammaton

Cardiac:
– rare
– ventricular arrhythmias

Question 6

Investigations

Answer 6

Blood tests:
– raised ACE and serum calcium
– inflammatory markers raised

Imaging:
– x-ray- can show hilarious lymphadenopathy

– high resolution CT of the chest- shows lymphadenopathy in more detail

– MRI- can check for the CNS involvement

PET scan- can show you active areas of inflammation

Biopsy

Question 7

Treatment

Answer 7

No treatment for pt’s with no or mild symptoms as the condition usually recovers spontaneously

Oral steroids:
– 6-24 months
– bisphosphonates to protect their bones during the long term duration of steroid treatment

Immunosuppressants

In severe pulmonary disease- lung transplant may be required