Everyday Vasculitis (or what questions do we get asked most!) Lucy Smyth Renal Consultant

Transcription

1 Everyday Vasculitis (or what questions do we get asked most!) Lucy Smyth Renal Consultant

2 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?

3 What is it? Vascul = blood vessel itis = inflammation Can affect small, medium and large vessels Varying patterns of disease according to the vessels affected

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

6 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

7 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

8 ANCA (anti neutrophil cytoplasmic antibodies) P-ANCA C-ANCA

9 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

10 How can we treat it? Cyclo Steroids AZA

11 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

12 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

13 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

14 How can we treat it? Plasma Exchange Cyclo Steroids Rituximab AZA

15 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

16 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

17 How can we treat it? Plasma Exchange Cyclo Steroids Rituximab MMF MTX AZA

18 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

19 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

20 And the extras Stomach protection Infection Bone protection Blood pressure omeprazole/lansoprazole ranitidine Septrin (Pneumocystis / staph) Nystatin (fungal) Calcium/vitamin D Bisphosphonate Ramipril Amlodipine

21 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

22 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

23 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

24 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

25 Risk of relapse increased by C-ANCA ANCA positivity Rapid reduction in treatment Lack of steroid

26 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

27 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

28 And join the West Country Vasculitis Support Group! Thank You Many thanks to Charlotte and Angie for arranging the evening