Kidneys and ANCA Vasculitis Flashcards
How do ANCA vasculitic diseases cause problems to blood vessels?
ANCA- Anti Neutrophil Cytoplasm
There is inappropriate degranulation of neutrophils within blood vessels which causes release of pro-inflammatory cytokines and proteins resulting in damage and inflammation. Neutrophils also adhere to the endothelium.
What are the two types of ANCA? What do they target?
C-ANCA = Anti-proteinase 3 (seen in GPA) P-ANCA= Anti-myeloperoxidase (seen in MPA)
What are the three ANCA associated vasculitic disorders?
GPA (c-ANCA) Microscopic Polyangitis ((p-ANCA) Eosinophilic GPA (Churg-Straus Syndrome)- but around 70% are ANCA -Ve
What are some general symptoms of vasculitis?
Fever
Weight loss
Fatige
What are some specific symptoms of vasculitis?
Recurrent nose bleeds Rash Pulmonary haemorrhage and haemoptysis Haematuria Temporal Headache- Giant cell arteritis Jaw Claudication- Giant Cell Arteritis Anterior Ischaemic optic neuropathy- Giant Cell Arteritis
What type of vasculitis is associated with ANCA?
Small vessel vasculitis- GPA, MPA, Eosinophilic GPA
What is the most common small vessel vasculitis?
GPA- Granulomatosis with Polyangitis
What does c-ANCA target?
Proteinase 3
What does p-ANCA target?
Myeloperoxidase
What are some of the symptoms of GPA?
Affects:
Nasopharynx- Recurrent Nose Bleeds, Chronic Sinusitis (swelling seen around eyes) Saddle nose deformity
Lungs- Haemoptysis, Difficulty breathing
Kidneys- Haematuria, increased blood pressure
What is the treatment for GPA?
Steroids
Cyclophosphamide
Other immunosupression
What type of ANCA is associated with microscopic poly-angitis?
p-ANCA- Anti-Myeloperoxidase
Where does myeloperoxidase affect?
Does not affect the naso-pharynx so is not associated with recurrent nose bleeds (unlike GPA)
Affects kidneys, eyes (causes scleritis), lungs
How can you differentiate between MPA and GPA on a biopsy?
GPA shows granulomatous inflammation
MPA does not show granulomatous inflammation
What is Churg-Strauss syndrome also called?
Eosinophilic GPA
What does Churg-Strauss cause to be elevated on an FBC?
Eosinophils are raised- eosinophilia
Churg-Strauss is associated with late onset asthma
What are some areas affected by Churg-Strauss?
Sinuses Lungs- Pulmonary Infiltrates Kidney GI Skin Heart- Myocarditis Nerves- Peripheral Neuropathy
What do Churg-Strauss and GPA have in common?
Both are associated with granuloma formation
Churg-Strauss is also called eosinophilic GPA
In a patient with small cell vasculitis what may be seen on a chest X-ray?
Pulmonary haemorrhage
Granulomas- but these are not due to TB which is another cause of granulomas in the lungs
What kind of rash is seen with a small vessel vasculitis?
Purpuric rash- due to bleeding from small vessels. These rashes are palpable (slightly raised)
If suspecting a vasculitic problem what investigations should be done?
Raised ESR and CRP (ESR particularly raised in small vessel vasculitis)
Immunology tests- ANCA (p or C), FBC (Eosinophilia is associated with Churg-Strauss)
Biopsy of affected areas- GPA and Churg-Strauss involve granuloma formation
What drugs may cause a drug induced vasculitis?
Anti-TNF
Hydralazine
Propythiouracil
Cocaine
What are the two steps to managing a vasculitis?
Induce remission
Maintain remission
What is the treatment to induce remission for the small vessel vasculitis?
Induce remission using:
Steroids e.g. prednisolone
If severe:
Cyclophosphamide
Rituximab (anti-B Cell)
Plasma Exchange to remove ANCA
If mild or moderate:
Methotrexate
Mycophenolate Mofetil
What treatments may be used to maintain remission in small vessel vasculitis?
Generally want to avoid steroid regimes
Cyclophosphamide Rituximab (Anti B Cell) Methotrexate Mycophenolate Mofetil Azathioprine
For safe immunosuppression what should be checked first?
Current infections HIV, HBV, HCV status TB status VZV immunity PJP Prophylaxis (ABx treatment)
