Immunology Flashcards
What are the key differences between the innate and adaptive immune systems?
Innate
- Rapid, first line of defence
- Antigen- independent (non specific)
- No immunological memory on subsequent exposure
Adaptive
- Slower, second-line of defence
- Antigen- dependent and specific
- Has immunological memory - can respond quick on subsequent exposure to a previous antigen
What are the main classifications of vasculitis and some examples of these
- Large vessel vasculitis (GCA and Takaysu arteritis)
- Medium vessel vasculitis (Polyarteritis nodosa, Kawasaki)
- Small vessel arteritis (garnulomatosis with polyangiitis, microscopic polyangiitis, eosinophilic granulomatosis polyangiitis)
What are the two types of small vessel vasculitis
ANCA and immune-mediated
Name the different types of ANCA
cANCA (against peroxidase 3) and pANCA (against myeloperoxidase)
cANCA predominant - Granulomatosis with polyangiitis
pANCA predominant - microscopic vasculitis and eosinophilic GPA
Note: cANCA vasculitis is associated with higher rates of relapse
what are the markers of activity in ANCA vasculitis
CRP and ESR
what diseases are associated with elevated ANCA levels
Very high levels: small vessel vasculitis
Higher than normal levels:
- Resp: ILD, cystic fibrosis
- Gastro: UC and, to lesser extent, Crohns
- Rheum: RA, SLE
What are the clinical features of granulomatosis polyangiitis
- ENT: chronic rhinitis/otitis, mucositis, conjuncitivitis/iritis, corneal ulcers, ulceration of nasal septum which can cause perforation “Saddle nose”, gingivitis “strawberry gums”
- RESP: bilateral peripheral lung lesions - granulomatous necrotising lesions. Can manifest as symptoms of pneumonia (cough, dyspnea, pleuritic chest pain)
- RENAL: pauci-immune rapidly progressive GN (hematuria and rbc casts)
- SKIN (lower limbs): non-blanching papules, Livedo reticularis
- CARDIAC: pericarditis, myocarditis
- CNS: mononueritis multiplex
- CONSTITUTIONAL: fatigue, fever, weight loss, malaise, arthralgia
How do you diagnose GPA
Gold standard is biopsy (lung or renal):
- necrotising granulomatous inflammation
- giant cells
- macrophages
What investigations would you do to investigate GPA
Urine: UA (hematuria), ACR/PCR, casts
Bloods: ANCA, leukocytosis, thrombocytosis, normocytic anemia, ESR/CRP, raised creatinine
Imaging: CXR/CT chest
Biopsy
What is the first lie induction treatment for GPA
Severe disease: glucocorticosteroids + rituximab OR cyclophosphamide
Non-severe disease: MTX + glucocorticosteroids
What is the maintenance treatment for GPA
Any one of these: rituximab or MTX or azathioprine or mycophenolate
What is the rate of relapse for patients with GPA
50-70%
Name 3 complications of GPA vasculitis
Due to disease
o Nasal deformity
o Renal impairment
o Respiratory insufficiency
Due to treatment
o CYC: infections and balder cancer in 16% after 15 years
o Prednisolone related complication
What type of vasculitis is Polyarteritis Nodosa
medium vessel vasculitis (necrotising inflammation)
What are some clinical features of PAN?
Renal: hypertension, renal infarct
Skin: purpuric rash, subcutaneous nodules, vesicular lesions, levido reticularis
GIT: abdo pain, PR bleeding, N/V, melena
CNS: mononueritis multiplex