Teaching Clinic - Secodary Immunodeficiency 101 Flashcards
Female 69
Unremarkable PMH
Presented with bilateral SN hearling loss, fleeting lung shadows and postive ANCA (anti-nuclear cytoplasmic antibody)
ANA (anti-nuclear antibody)
What is ANA? How is it detected?
Anti-neutrophilic cytoplasmic Ab Ix
- These Ab stick to neutrophil detected by immunofluorescence involving neutrophils.
§ Find normal cells + fluorescent stain if light up = positive dilute until it is not lighting up = titre
· Titre dilute until its no longer detectable.
· Reflects the concentration, the higher the tire the higher the concentration
· Prone to inter-investigator variability
ANA: Immunofluoresence test, against any nuclei
Titre is more qualitative, 1:160 is significant titre. The higher the titre, the more likely there is autoimmune disease
What is ENA?
- ENA test is often used as a follow-up test after a positive ANA test to identify which autoimmune disease patient has
- ENA test can check the blood for the presence of antinuclear antibodies that are known to be markers of certain diseases
See which specific part of nucleus is detected
What is this immunofluorence pattern?
C-ANCA positive
Cytoplasm is +ve!!!
What is this immunofluorence pattern?
P-ANCA, perinuclear ANCA
What are the 3 staining patterns?
What are the small, medium and large vessels vasculitis?
Types of vasculitis
- Large vessel: Giant cell arteritis, Takayasu arteritis
- Medium vessel: Polyarteritis nodosa, Kawasaki disease
- Immune complex small vessel: Cryoglobulinemic vasculitis, IgA vasculitis
- ANCA- associated small vessel: Microscopic polyangiitis, GPA, EGPA
Giant cell arteritis
- Common presentation
- Dx
- Mx
[Large vessels]
- Older people (F~M)
Background of polymyalgia rheumatica:
o Proximal joint stiffness (girdle disease)
o Constitutional symptoms
o Almost all patients with GCA have PMR, but not a lot of PMR have GCA!!!
Signs & symptoms of GCA:
- Temporal headache, jaw claudication, scalp tenderness, amaurosis fugax
- Risk of permanent blindness
o Arteritic AION (red colour blindness/desaturation) = can be irreversible
Dx
o ESR (3 digit range, sky high), CRP
o Duplex USG of temporal vessel
o Temporal artery biopsy to confirm (page surgeon!)
Mx
o Immediate high dose steroid (AION can be irreversible, need to recognise ASAP)
[Phillip Li said this won’t be assessed]
Polyarteritis Nodosa
- Some overlap with MPA
- Affects large vessel
o Necrosis of skin or pleura
o Renal arteries renal artery stenosis
o Mononeuritis multiplex
What is Kawasaki?
Medium vessel arteritis (coronary artery)
High dose aspirin, IVIg
This is a pure paediatric immunologic condition
Medium vessel vasculitis
Strawberry tongue, peripheral oedema
[Will not be assessed]
Cryoglobulinemic vasculitis
Smokers
Complements are low
Refer to specialist
Henoch Schonlein Purpura (Paediatrics)
PAEDS
IgA-mediated vasculitis
Petechiae of lower limbs
Molecular mimicry
Self-limiting
Petechiae, joint pain
What is reno-pulmonary syndrome?
Vasculature in these structure are effected:
- Glomeruli
- Pulmonary vasculature of lung
Do not miss this in patients with haematuria and haematemesis (not true haematemesis, but rather frank haemoptysis)
FLEETING LUNG SHADOWS ON CXR in EARLY
AKI without symptoms, quickly progress to ESRF
Anti-GBM
- Hematemesis
- Crescentic glomerulonephritis
Symptoms of large vessel involvement
Symptoms of medium vessels
Symptoms of small vessel disease