Everyday Vasculitis Everyday Vasculitis (or what questions do we - - PowerPoint PPT Presentation

Everyday Vasculitis Everyday Vasculitis

(or what questions do we get asked most!) (or what questions do we get asked most!)

Lucy Smyth Renal Consultant

 What is it?  Why have I got it?  How can we treat it?  Why do I feel like I do?  What do the blood tests mean?  Will it go away?  What can I do to help myself?

What is it?

 Vascul = blood vessel  itis = inflammation  Can affect small, medium and large vessels  Varying patterns of disease according to the vessels

affected

Kidneys

Glomerulonephritis = inflammation of the filters Blood and protein in urine Rapid deterioration of function High blood pressure

Joints

Synovitis = inflammation of joint lining

Lungs

Pulmonary haemorrage = bleeding Infiltrates = inflammation Granulomas = inflammatory lumps

Eyes

Inflammation Nerve damage

Nerves

Weakness Loss sensation Confusion

General

Night sweats Weight loss Tiredness Rash

ENT

Deafness Sinusitis Nosebleeds Nasal crusting

Gut

Bleeding Pancreatitis

Why have I got it?

 The way your immune system is made up

 Genetic, but not directly inherited

 What your immune system has come across

 Environment

 The way your immune system has reacted to it

 Often a trigger, eg infection

ANCA (anti neutrophil cytoplasmic antibodies)

 Antibodies that bind to certain white blood cells  Cause the white blood cells to release toxic substances  Causes inflammation of the blood vessels

ANCA (anti neutrophil cytoplasmic antibodies)

 P-ANCA  C-ANCA

How can we treat it?

 Hit it hard

 Induction treatment

 Keep it under control

 Maintenance treatment

 Slowly try to wean down/off

 According to disease activity  P-ANCA min 2-3 years, c-ANCA min 5 years

How can we treat it?

Cyclo AZA Steroids

Cyclophosphamide

 Induction

 The oldest, and not yet surpassed  Much shorter courses now: 3 months = minimal toxicity  Knocks out the antibody producing B cells  Careful monitoring: weekly bloods  Oral or IV pulses  Risks

 Infection, low WBC, hair thinning, infertility, malignancy

Azathioprine

 Maintenance

 Oldest and not yet surpassed  Blocks turnover of inflammatory cells  Need to check TPMT level  Oral, once daily  Monthly bloods  Risks

 Infection, nausea, liver inflammation, low WBC, skin malignancy

Steroids

 Induction / maintenance

 Important for early control of inflammation  No one has yet found a way of avoiding them  Trials being proposed to minimise dosing regimes / avoid  Risks

 Infection, bruising, diabetes, osteoporosis, thinned skin

How can we treat it?

Plasma Exchange Cyclo AZA Steroids Rituximab

Plasma Exchange

 At start of induction treatment  Current indications:

 Creatinine >500  Pulmonary haemorrhage  Removes circulating ANCA  Similar to dialysis; daily sessions for a week  Risks

 Infection, bleeding, allergic reaction

Rituximab

 Induction if cyclophosphamide unsuitable  Relapses at induction or maintenance

 Also knocks out B cells  Similar efficacy as cyclophosphamide, no fewer risks  Slower onset  2 infusions at 2 week interval, then every 6-18 months  Risks

 Infection, infusion reaction, low general antibody level, PML

How can we treat it?

Plasma Exchange Cyclo MMF MTX AZA Steroids Rituximab

Mycophenolate Mofetil

 Induction if disease mild  Maintenance if azathioprine not tolerated / effective

 Oral, 2-3 x day  Monthly bloods  Risks

 Infection, nausea, diarrhoea, anaemia, low WBC

Methotrexate

 Induction / maintenance

 Especially good for granulomatous disease

 ENT and lung masses in GPA

 Can’t use for renal disease or if renal dysfunction  Weekly, usually oral  Monthly bloods  Risks

 Infection, nausea, lung or liver inflammation

And the extras…

• omeprazole/lansoprazole
• ranitidine
• Septrin (Pneumocystis / staph)
• Nystatin (fungal)

Stomach protection

• Calcium/vitamin D
• Bisphosphonate

Infection

• Ramipril
• Amlodipine

Bone protection Blood pressure

Why do I feel like I do (tired)?

 Inflammation causes fatigue  Medications can cause fatigue (steroids)  Inflammation, medications and renal dysfunction

cause anaemia, which causes fatigue

 Illness causes deconditioning

anaemia

 Will improve gradually

 As inflammation settles  When you come off cyclophosphamide  If renal function improves

 If left with significant renal impairment you may need

 Iron  Erythropoietin

What do the blood tests mean?

 CRP

 Increases with inflammation or infection: aim <5

 Haemoglobin

 Marker of anaemia: aim >120

 WBC

 Avoid dropping below 4

 Creatinine / eGFR

 Marker of renal function: will find new baseline

 ANCA

 Disease activity may affect MPO or PR3 titre

Will it go away?

 You will always have the tendency to have vasculitis  Relapses reported at 50% in 5 years (less now?)  You may be able to come off treatment  You may stay off treatment

Risk of relapse increased by…

 C-ANCA  ANCA positivity  Rapid reduction in treatment  Lack of steroid

What can I do to help myself?

 Don’t worry if you don’t take it all in at once

 You will get to know your disease

 Take your medication

 We can try and minimise side effects together

 Make sure you have blood tests when needed  Keep up to date with vaccinations

What can I do to help myself?

 Contact GP or consultant if you are unwell  Keep active, build up your fitness again  Eat healthily; stick to renal dietary advice if needed  Stop smoking

 And join the West Country Vasculitis Support Group!

Thank You

 Many thanks to Charlotte and Angie for

arranging the evening