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 Joints Rapid deterioration of function Synovitis = High blood pressure inflammation of joint lining General Lungs Eyes Night sweats Pulmonary haemorrage = bleeding Weight loss Inflammation Infiltrates = inflammation Tiredness Nerve damage Granulomas = inflammatory lumps Rash ENT Gut Nerves Deafness Bleeding Weakness Sinusitis Pancreatitis Loss sensation Nosebleeds Confusion Nasal crusting

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 Steroids AZA

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 Steroids Rituximab AZA

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 Steroids Rituximab MMF MTX AZA

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… Stomach • omeprazole/lansoprazole protection • ranitidine • Septrin (Pneumocystis / staph) Infection • Nystatin (fungal) • Calcium/vitamin D Bone protection • Bisphosphonate • Ramipril Blood pressure • Amlodipine

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