Sarcoidosis

• What causes sarcoidosis?

  Unsure but there are some factors that may trigger an immune response in susceptible people
• What are some factors that may trigger sarcoidosis?

  Infections

  Environmental and occupational exposures

  Genetics
• What ethnicity is sarcoidosis more common in?

  Black African ethnicity
• What happens in sarcoidosis?

  Exaggerated immune response -> formation of non-caseating granulomas
• What is the classification of sarcoidosis based on?

  Organ involvement
• What are the 2 classifications of sarcoidosis?

  Pulmonary

  Extrapulmonary
• Which is the most common type of sarcoidosis?

  Pulmonary
• How is pulmonary sarcoidosis characterised?

  Non-caseating granulomas in the lungs
• What are some common sites of extrapulmonary sarcoidosis?

  Skin

  Eyes

  Lymph nodes

  Liver

  Heart

  Nervous system
• What is extrapulmonary sarcoidosis characterised by?

  Affects organs other than the lungs
• What is the name of the scoring system used to radiologically stage sarcoidosis?

  Scadding staging
• What is stage 0 of the Scadding staging?

  Normal CXR
• What is stage 1 of the Scadding staging?

  Bilateral hilar lymphadenopathy alone
• What is stage 2 of the Scadding staging?

  BHL with pulmonary infiltrates
• What is stage 3 of the Scadding staging?

  Pulmonary infiltrates without BHL
• What is stage 4 of the Scadding staging?

  Pulmonary fibrosis
• How can sarcoidosis present?

  Starts with non-specific symptoms - clinical manifestations can vary as it is dependent on the organ systems involved
• What symptoms can present if the resp system is involved in sarcoidosis?

  Dry cough

  Dyspnoea on exertion

  Chest discomfort
• What symptoms can present if the skin is involved in sarcoidosis?

  Erythema nodosum - on anterior shins

  Lupus pernio - specific for sarcoidosis but not commonly seen
• What is lupus pernio characterised by?

  Violaceous skin lesions on the nose and cheeks
• What symptoms can present if the eyes are involved in sarcoidosis?

  Uveitis (most frequent)

  Conjunctivitis

  Scleritis
• What symptoms can present if the cardiovascular system is involved in sarcoidosis?

  Heart block/arrhythmias

  Left vent dysfunction

  Sudden cardiac death
• What symptoms can present if the MSK is involved in sarcoidosis?

  Lofgren's syndrome (acute sarcoid arthritis)
• How is Lofgren's syndrome characterised?

  Erythema nodosum

  Arthralgia

  BHL
• What symptoms can present if the endocrine and renal systems are involved in sarcoidosis?

  Hypercalcaemia -> nephrocalcinosis or nephrolithiasis
• Why can you get hypercalcaemia in sarcoidosis?

  Inc conversion of vit D to calcitriol (active vit D form) by mphages in granulomas
• What are the first-line investigations for sarcoidosis?

  Bloods

  Chest radiography

  Pulmonary function tests (PFTs)
• What are some further investigations for sarcoidosis?

  HRCT

  Tissue biopsy

  Organ specific investigations (dependent on symptoms)
• Investigation of sarcoidosis uses a stepwise approach: starts with firstline investigations -> further investigations if necessary
• What blood tests are done for sarcoidosis?

  FBC

  RFT

  LFT

  Serum calcium
• Which is the essential first-line imaging investigation for sarcoidosis?

  Chest X-ray
• What pulmonary function test results are typically seen in pulmonary sarcoidosis?

  Restrictive pattern + dec FVC, dec TLC, normal or inc FEV1/FVC ratio
• Why would a tissue biopsy be done for sarcoidosis?

  Confirmation of non-caseating granulomas
• What is the firstline therapy for sarcoidosis?

  Corticosteroids - prednisone
• How is sarcoidosis managed?

  Depends on severity and extent of organ involvement
• If corticosteroids are not effective or contraindicated what may be used to manage sarcoidosis?

  Immunosuppressants - methotrexate, azathioprine
• What may be used in refractory cases or severe organ involvement sarcoidosis?

  Biologic therapies - infliximab or adalimumab