Histopathlogy 5: Connective Tissue Disorders Flashcards
List 3 auto-antibodies seen in SLE ?
- Anti-dsDNA
- Anti-Smith (most specific but not very sensitive - only 30% with SLE have)
- Anti-histone (drug related)
NB ANA (Anti-nuclear antibody) is just a screening test which tells you that the patient has antibodies that bind to nuclear antigens (it does NOT tell you which antigens it binds to)
What type of hypersensitivity reaction is seen in SLE ?
Type 3 hypersensitivity reaction
What is the HLA association with SLE ?
HLA DR3
Name the heart condition that can be caused by SLE ?
- Libman-sack endocarditis
- This is a NON-INFECTIVE form of endocarditis (inflammatory form of endocarditis)
List the signs and symptoms of SLE using the mnemonic SOAP BRAIN MD
Serositis (inflam of serous tissue - surround organs)
Oral ulcers
Arthritis
Photosensitivity
Blood disorders- AIHA, ITP
Renal involvement - Type 2 RPGN
ANA +ve
Immune- anti-dsDNA, anti-smith
Neuro symptoms
Malar rash
Discoid rash
Which auto antibodies are seen in Diffuse scleroderma ?
Anti-scl70 - this is the antibody to DNA topoisomerase
Which autoantibodies are seen in limited scleroderma (CREST) ?
Anti-centromere (ANCA)
Which HLA is associated with scleroderma ?
HLA DR5
List the signs and symptoms of limited scleroderma (CREST) ?
- Calcinosis
- Raynauds phenomenon
- Esophageal dysmotility
- Sclerodactily
- Telangiectasia
No involvement of the trunk
Aetiology of scleroderma effect in skin
- EXCESS deposition of collagen
- resulting in reduced skin elasticity
Which autoantibodies are seen in Polymyositis and dermatomyositis ?
anti- Jo1
Patient has proximal muscle weakness. Their bloods show increased CK and abnormal EMG. No rash is seen.
Most likely diagnosis?
Polymyositis
Patient has proximal muscle weakness. Their bloods show increased CK and abnormal EMG. A heliotrope rash is seen on the eyelids. There are Gottron papules on the knuckles
Most likely diagnosis?
Dermatomyositis
Japanese lady with absence of pulse and Claudication (pain in muscles).
The Rheumatologist says that its a large vessel arthritis.
Most likely diagnosis ?
Takayasu’s arteritis
Elderly man presents with a temporal headache, jaw claudication and scalp tenderness.
Most likely diagnosis?
Temporal arteritis
(Large vessel vasculitis)
A man presents with abdo pain and signs of renal impairment. Angiogram shows “rosary beads” because of multiple aneurysms.
Most likely diagnosis?
Associated with which disease?
Polyartheritis nodosa (medium vessel arteritis) Hepatitis B
A heavy smoker with ulceration of the toes and fingers has an angiogram which shows a “corkscrew appearance”.
Most likely diagnosis?
Buerger’s disease
List the triad of features seen in Polyangitis with granulomatosis ?
Saddle nose
Pulmonary haemorrhage
Glomerulonephritis (Crescentic)