Histo: Connective tissue disease, amyloid, sarcoid, Immune related multisystem disorders Pt.2 Flashcards
What is dermatomyositis?
A condition characterised by proximal muscle pain and weakness and skin changes
Which marker would be raised in the blood in dermatomyositis?
Creatine kinase
What signs would be seen on the skin in dermatomyositis?
- Gottren’s papules
- Heliotrope rash
- Facial rash
- Systemic v-shaped rash
What is sarcoidosis of the skin called?
Lupus pernio
List some features of sarcoidosis.
What is the pathological hallmark of sarcoidosis?
Non-caseating granuloma
Langhans giant cell, epitheloid macrophages, lymphocytes
What are some biochemical changes seen the blood in sarcoidosis?
- Hypergammaglobulinaemia
- Raised ACE
- Hypercalcaemia (due to 1-alpha hydroxylase expression by granuloma macrophages)
What criteria is used to classify vasculitides based on the size of the vessel?
Chapel Hill Criteria
What is polyarteritis nodosa? What are its main features?
- A necrotising arteritis which is focal and sharply demarcated
- It heals by fibrosis and mainly affects the renal and mesenteric vessels
- May present with gut ischaemia or renal impairment
- It produces a rosary beads (nodular) appearance on angiography due to multiple aneurysms
Which infection is polyarteritis nodosa associated with?
Hepatitis B
What is a characteristic feature of vasculitis?
Palpable purpuric rash
How is temporal arteritis diagnosed and treated?
Diagnosis
- Clinical
- High ESR
- Temporal artery biospy
Treatment
- High dose prednisolone
What will be seen on temporal artery biopsy in temporal arteritis?
Lymphocytic infiltration of the tunica media
What is Kawasaki disease? What are the main clinical features?
Medium vessel vasculitis
Fever lasting longer that 5 days accompanied by:
- Conjunctivitis
- Rash
- Adenopathy
- Strawberry tongue
- Hand and feet skin desquamation
What is a complication of Kawasaki’s?
Coronary artery aneurysm (screen using echocardiogram)
