Friday 8 October 2010

MRCP revision battle 27.2: Sarcoidosis

Sarcoidosis is a multi-system granulomatous disorder of unknown cause.


It is asymptomatic in up to 40% of cases, being discovered incidentally on a routine CXR.


Features of sarcoidosis may be divided into pulmonary and non-pulmonary:

Pulmonary features of sarcoidosis:
  • dry cough
  • progressive dyspnoea
  • chest pain

Non-pulmonary features of sarcoidosis include:
  • lymphadenopathy (commonest in non-whites)
  • erythema nodosum (commonest in white females)
  • polyarthralgia
  • hepatomegaly
  • splenomegaly (around 25%)
  • anterior uveitis (around 25%)
  • glaucoma
  • Bells palsy
  • lupus pernio
  • hypercalcaemia
  • renal stones
  • pituitary dysfunction
  • cardiomyopathy...


90% of patients with sarcoid will have an abnormal CXR.
Staging of CXR in sarcoid is:
  • 0 = clear CXR
  • 1 = bilateral hilar lymphadenopathy
  • 2 = bilateral hilar lymphadenopathy plus pulmonary infiltration
  • 3 = pulmonary infiltration only
  • 4 = fibrosis, honeycombing, pleural involvement


Investigations:
  • raised ESR
  • lymphopenia
  • raised LFTs
  • raised serum ACE
  • raised calcium (5%)
  • tuberculin skin test is positive in around a third

Management:
  • BHL alone doesn't need treatment
  • acute sarcoidosis : NSAIDs and bed rest
  • prednisolone ( 40mg OD for 4-6 weeks then reducing dose over one year) is indicated if:
    • parachymal lung disease
    • uveitis
    • hypercalcaemia
    • neurological/cardiac involvement

Lets move on to briefly consider lofgrens syndrome...