Sarcoidosis Flashcards
Define Sarcoidosis?
Multisystem granulomatous inflammatory disorder
What is the aetiology of Sarcoidosis?
Transmissible agents (e.g. viruses)
Environmental triggers
Genetic factors have all suggested
What is the pathogenesis of Sarcoidosis?
An unknown antigen is presented on MHC class 2 complexes on macrophages to CD4+ T-lymphocytes These accumulate and release cytokines This leads to the formation of non-caseating granulomas in organs
What is the epidemiology of Sarcoidosis?
Most common in AFRICANS and SCANDINAVIANS
Can occur at any age but tends to be in adults > 50 yrs
What are the general symptoms of Sarcoidosis?
Fever Malaise Weight Loss Bilateral parotid swelling Lymphadenpathy Hepatosplenomegaly
What are the Pulmonary symptoms of Sarcoidosis?
Breathlessness
Dry cough
Chest discomfort
Minimal clinical signs
What are the Musculoskeletal Symptoms of Sarcoidosis?
Bone Cysts (e.g. dactylitis)
Polyarthralgia
Myopathy
What are the eye symptoms of Sarcoidosis?
Keratoconjunctivitis Sicca
Uveitis
Papilloedema
What is Keratoconjunctivitis Sicca?
Inflammation of the conjunctivitis and surroudning tissues due to the eyes being dryg
What are the Skin Symptoms of Sarcoidosis?
Lupus pernio
Erythema nodosum
Maculopapular eruptions
What is Lupus pernio?
red-blue infiltrations of the nose, cheeks, ears and terminal phalanges
What are the neurological symptoms of Sarcoidosis?
Lymphocytic meningitis Space-occupying lesions Pituitary infiltration Cerebellar ataxia Cranial nerve palsies Peripheral neuropathy
What are the cardiac symptoms of Sarcoidosis?
Arrhythmia Bundle branch block Pericarditis Cardiomyopathy Congestive cardiac failure
What investigations do you do for Sarcoidosis?
Bloods 24 hr Urine Collection CXR High-Resolution CT Scan Gallium Scan Pulmonary Function Tests Bronchoscopy and Bronchoalveolar Lavage Transbronchial Lung Biopsy (or lymph node biopsy)
What bloods do you do for Sarcoidosis?
High Serum ACE High calcium High ESR FBC Immunoglobulins LFTs
What do you see on FBC for Sarcoidosis?
WCC may be low due to lymphocyte sequestration in the lungs
Why do we investigate Immunoglobulins for Sarcoidosis?
Polyclonal hyperglobulinaemia
What LFTs do we do for Sarcoidosis?
High ALP + GGT
Why do we do a 24 hr urine collection for Sarcoidosis?
Hypercalciuria
What do we see on CXR for Sarcoidosis?
Stage 0 - may be clear
Stage 1 - bilateral hilar lymphadenopathy
Stage 2 - stage 1 with pulmonary infiltration and paratracheal node enlargement
Stage 3 - pulmonary infiltration and fibrosis
Why do we do High-Resolution CT Scan for Sarcoidosis?
Check for diffuse lung involvement
Why do we do Gallium Scan for Sarcoidosis?
Shows areas of inflammation
Why do we do for Sarcoidosis on Pulmonary Function Tests?
Low FEV1
FVC shows restrictive picture
Why do we Bronchoscopy and Bronchoalveolar Lavage for Sarcoidosis?
High lymphocytes
High CD4:CD8 ratio
Why do we do Transbronchial Lung Biopsy for Sarcoidosis?
Shows non-caseating granulomas consisting of:
- Epithelioid cells (activated macrophages)
- Multinucleate Langerhans cells
- Mononuclear cells (lymphocytes)