Vasculitis
Systemic vasculitis: ANCA associated vasculitis (AAV) and
polyarteritis nodosa (PAN)
Systemic vasculitis is the name for a group of autoimmune
conditions caused by inflammation of the blood vessels. It can
affect different parts of the body, depending on which blood
vessels are involved (e.g., skin, lungs, kidneys, nervous system,
ears, sinuses, eyes and guts). People can present with symptoms
including weight loss, fevers, high inflammatory markers and
feeling generally unwell. There can sometime be a delay in
diagnosis as people are often treated as if they have a severe
infection prior to diagnosis as vasculitis can present in a similar
way to infections. Diagnosis should be confirmed by either biopsy
or imaging as quickly as possible so that treatment can be started.
There may be different doctors involved in the diagnosis and
management of vasculitis from different specialities (e.g.
rheumatology, respiratory, renal, dermatology, renal and ENT)
depending on which areas of the body are affected. Care is then
shared between these specialities and your case may be discussed in
a multidisciplinary (MDT) meeting to decide on the optimum
treatment.
There are different types of vasculitis which present in
slightly different ways:
ANCA associated vasculitis (AAV) includes three conditions which
affect the small blood vessels of the body; they are often (but not
always) associated with a positive ANCA blood test.
- Granulomatosis with polyangiitis (GPA, previously known as
Wegeners) can affect the nose, ears, eyes and sinuses, lungs,
kidneys, joints, nerves and skin.
- Microscopic polyangiitis (MPA) often affects the kidneys, but
can also affect the lungs, skin, nerves and nose to a lesser
extent.
- Eosinopilic granulomatosis with polyangiitis (EGPA, previously
known as Churg Strauss Syndrome) can present with asthma or rashes,
nasal polyps, peripheral nerve and kidney involvement.
Polyarteritis nodosa (PAN) is a type of medium vessel
vasculitis. PAN can present with abdominal or testicular pain, skin
rashes, weight loss and fevers, and can involve the medium-sized
blood vessels in the kidney or abdomen. In some cases PAN can be
linked to having Hepatitis B, which require separate treatment.
Treatment of small and medium vessel vasculitis is aimed at
controlling the inflammation of the blood vessels and preventing
damage to the affected organs. Induction therapy will usually
include high dose steroids, and either cyclophosphamide, rituximab
or methotrexate; maintenance therapy will follow on with a reducing
course of steroids and either rituximab, azathioprine or
methotrexate. Further information about these treatments is given
in the links below, and your doctor and specialist nurse will
discuss these in detail with you. You will also be given
medications to prevent infections and protect your stomach and
bones. UHBristol Rheumatology department is an NHS England
specialist centre for Vasculitis and participates in the UKIVAS
registry study and other observational and interventional
trials.
Information for patients
Conditions:
https://www.vasculitis.org.uk/about-vasculitis/gpa-granulomatosis-with-polyangiitis
https://www.vasculitis.org.uk/about-vasculitis/microscopic-polyangiitis
https://www.vasculitis.org.uk/about-vasculitis/churg-strauss-syndrome
https://www.vasculitis.org.uk/about-vasculitis/polyarteritis-nodosa
Treatments
https://www.versusarthritis.org/about-arthritis/treatments/drugs/cyclophosphamide/
https://www.versusarthritis.org/about-arthritis/treatments/drugs/rituximab/
Information for clinicians
https://academic.oup.com/rheumatology/article/53/12/2306/1802843
