Sarcoidosis Flashcards
Sarcoidosis
Multisystem disorder of unknown aetiology
Non-caseating granulomas
More common in young adults and people of African descent
Women affected more
Most commonly affected organ
Lungs
Lung features
Mediastinal lymphadenopathy
Pulmonary fibrosis
Pulmonary nodules
Systemic symptoms
Fever
Fatigue
Weight loss
Liver features
Liver nodules
Cirrhosis
Cholestasis
Eye features
Uveitis
Conjunctivitis
Optic neuritis
Skin features
Erythema nodosum (tender red nodules on shins)
Lupus pernio (raised purple lesions on cheeks and nose)
Granulomas develop in scar tissue
Heart features
Bundle branch block
Heart block
Myocardial muscle involvement
Kidney features
Kidney stones (due to hypercalcaemia)
Nephrocalcinosis
Interstitial nephritis
CNS features
Nodules
Pituitary involvement (diabetes insipidus)
Encephalopathy
PNS features
Facial nerve palsy
Mononeuritis multiplex
Bones
Arthralgia
Arthritis
Myopathy
Lofgren’s syndrome
Triad of
- erythema nodosum
- bilateral hilar lymphadeonopathy
- polyarthralgia
Factors associated with poor prognosis
Insidious onset (symptoms >6 months)
Absence of erythema nodosum
Extrapulmonary mainfestations
CXR: stage III-IV features
Chest xray stages
0- normal
1- bilateral hilar lymphadenopathy
2- BHL and interstitial infiltrates
3- diffuse interstitial infiltrates only
4- diffuse fibrosis
Other investigations
Spirometry: restrictive defect
Tissue biopsy: non-caseating granulomas
Blood tests
Raised serum ACE (screening)
Hypercalcaemia is key finding
Raised serum soluble IL-2 receptor
Raised CRP
Raised immunoglobulins
Histology
Gold standard for confirming diagnosis
Bronchoscopy with US guided biopsy of mediastinal lymph nodes
Shows non-caseating granulomas with epitheloid cells
Treatment
No treatment in no/mild symptoms as condition often resolves spontaneously
Oral steroids 6-24 months (and bisphosphonates for bone protection)
Second line methotrexate or azathioprine
Lung transplant rarely required in severe disease
Prognosis
Resolves within 6 months in 60% patients
Small number progress with pulmonary fibrosis and pulmonary hypertension (potentially requiring lung transplant)
Death usually when heart or CNS affected
