Current and future treatment option for Systemic Sclerosis
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This document discusses current and future treatment options for systemic sclerosis (SSc). It covers treatments for Raynaud's phenomenon like nifedipine, iloprost, and bosentan which aim to dilate blood vessels and improve blood flow. It also discusses immunosuppressants like cyclophosphamide and methotrexate used to treat lung disease and skin thickening. Symptoms of gastrointestinal problems, arthritis, lung fibrosis, pulmonary hypertension, and kidney disease are reviewed along with their treatment options. The document concludes by mentioning research into new treatments like rituximab and autologous stem cell transplant and the work of research groups to advance treatment of SSc.
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Current and future treatment option for Systemic Sclerosis
- 1. Current and future treatment options for SSc Dr Victoria Flower Rheumatology Specialty Registrar RNHRD, Bath
- 2. Systemic Sclerosis Autoimmune •Immune system produces cytokines •Inflammation Overproduction of Collagen •Scar tissue forms, thickening of soft tissues Blood vessel damage •Reduces blood flow to tissues causing damage •Encourages calcium deposits
- 4. Raynaud’s Phenomenon Autoimmune •Immune system produces cytokines •Inflammation Overproduction of Collagen •Scar tissue forms, thickening of soft tissues Blood vessel damage •Reduces blood flow to tissues causing damage •Encourages calcium deposits
- 5. Raynaud’s phenomenon • Cold, white/blue red • Painful hands and reduced fine movement during attacks
- 6. Endothelium • Aim to dilate the blood vessels to improve blood flow and warmth to fingers
- 7. Raynaud’s phenomenon • Nifedipine* • Iloprost • Bosentan • Sildenafil • Reduce frequency and severity of attacks • Heal ulcers • Reduce new ulcers occurring
- 8. Raynaud’s Phenomenon Nifedipine • Tablet three times a day • Blood pressure • Dizziness Iloprost • Intravenous • In hospital • Nausea • Diarrhoea • Muscle cramp • Headaches/jaw pain • blood pressure • Flushing • Expensive Bosentan • Dizzy • Flushing • Liver tests • May reduce OCP • Rarely haemoptysis
- 9. Raynaud’s phenomenon • Digital ulcers • Infection • Antibiotics as well as dilating treatments
- 10. Scleroderma Autoimmune •Immune system produces cytokines •Inflammation Overproduction of Collagen •Scar tissue forms, thickening of soft tissues Blood vessel damage •Reduces blood flow to tissues causing damage •Encourages calcium deposits
- 11. Limited cutaneous SSc • If no lung disease then manage symptoms • Treat Raynaud’s / ulcers/ infection • Hand therapy • Monitor • Early sausage swelling of fingers may benefit from low dose steroids & antihistamines
- 12. Progressive Diffuse or lung disease Maintenance Induction Cyclophosphamide Lung, heart, progressive skin Methotrexate Skin, arthritis, muscle inflammation Mycophenolate, Azathoprine Predominantly lung disease
- 13. Cyclophosphamide What is it? • Also used for other conditions as well – lupus, vasculitis • Intravenous injection (4-6 ‘cycles’) • Takes up to 6 weeks to work • Live vaccines ✗ • Annual Pneumovax ✓ Side effects: • Nausea is common • Haemorrhagic cystitis • Monitor for white blood cells dropping and infection • Fertility/contraception
- 14. Methotrexate What is it? • Used for many Rheumatological conditions • Once a week • Anti-folate • Weekly folic acid supplement • Monthly blood test monitoring • Takes up to 12 weeks to work • Live vaccines ✗ • Annual Pneumovax ✓ Side effects: • Mouth ulcers • Nausea • Hair thinning (usually minor) • Blood count • Liver function tests • Rarely lung inflammation
- 15. Calcinosis • Treat infection • Unfortunately no targeted treatments for calcium deposits • Sometimes surgery but avoid if possible
- 16. Arthritis – joint pain and swelling Autoimmune •Immune system produces cytokines •Inflammation Overproduction of Collagen •Scar tissue forms, thickening of soft tissues Blood vessel damage •Reduces blood flow to tissues causing damage •Encourages calcium deposits Hydroxychloroquine, Methotrexate and Mycophenolate mofetil
- 17. Hydroxychloroquine • “Plaquenil” • Takes up to 12 weeks to work • Usually well tolerated with few side effects Side effects: • Skin rash • Can exacerbate psoriasis • Indigestion • Headaches • Rarely visual problems 1 in 2000
- 18. Upper Gastrointestinal problems Oesophageal dysmotility Difficulty swallowing Painful swallow Indigestion and acid reflux GORD PPIs to reduce acid Prokinetics Thrush Antibiotics - Fluconazole Delayed emptying of stomach Bloating, Early satiety Wind Prokinetics – domperidone, metoclopramide
- 19. Lower Gastrointestinal problems Reduced movement (peristalsis) of small bowel Bloating, wind and constipation Laxatives Diarrhoea Loperamide Bacterial overgrowth Rotated antibiotics Reduced absorption of nutrients Nutritional advice & supplements Rarely TPN
- 20. Systemic Sclerosis lung disease Autoimmune •Immune system produces cytokines •Inflammation Overproduction of Collagen •Scar tissue forms, thickening of soft tissues Blood vessel damage •Reduces blood flow to tissues causing damage •Encourages calcium deposits
- 21. LungsSymptoms: Short of breath Cough Chest pain Fatigue Ankle swelling Fibrosis: Cyclophosphamide Mycophenolate mofetil Azathioprine Steroids Aim to improves symptoms, breathing tests and QoL Pulmonary hypertension: Warfarin, water tablets Bosentan Sildenafil Iloprost (as an inhaler) Epoprostinol (as a continuous infusion) Improves exercise tolerance, day to day symptoms and function, improves heart pressure
- 22. Kidney – Scleroderma renal crisis High Blood pressure Frothy urine Persistent headache Blurred vision Ankle swelling Short of breath ACEI - lower BP Improves survival and reduces need for long term dialysis. Dialysis Steroids in SSc: Monitor BP, urine dipstick and kidney blood test Avoid high dose steroids if at risk of SRC
- 23. The future for Systemic Sclerosis Autoimmune •Immune system produces cytokines •Inflammation Overproduction of Collagen •Scar tissue forms, thickening of soft tissues Blood vessel damage •Reduces blood flow to tissues causing damage •Encourages calcium deposits EULAR SSc Trials and Research Group - EUSTAR Canadian SSc research groupSSc Research Foundation Rituximab, Abatacept, Tocilizumab Autologous Stem cell transplant
- 24. References (1)Thompson AE, Shea B, Welch V, Fenlon D, Pope JE. Calcium-channel blockers for Raynaud’s phenomenon in systemic sclerosis. Arthritis Rheum 2001;44:1841–7. (2) Pope J, Fenlon D, Thompson A, Shea B, Furst D, Wells G, et al. Iloprost and cisaprost for Raynaud’s phenomenon in progressive systemic sclerosis. Cochrane Database Syst Rev 1998;(2):CD000953. doi: 10.1002/14651858.CD000953. (3) Rademaker M, Cooke ED, Almond NE, Beacham JA, Smith RE, Mant TG, et al. Comparison of intravenous infusions of iloprost and oral nifedipine in treatment of Raynaud’s phenomenon in patients with systemic sclerosis: a double blind randomised study. BMJ 1989;298:561–4. (4) Scorza R, Caronni M, Mascagni B, Berruti V, Bazzi S, Micallef E, et al. Effects of long- term cyclic iloprost therapy in systemic sclerosis with Raynaud’s phenomenon. A randomized, controlled study. Clin Exp Rheumatol 2001;19:503–8. (5) Wigley FM, Seibold JR, Wise RA, McCloskey DA, Dole WP. Intravenous iloprost treatment of Raynaud’s phenomenon and ischemic ulcers secondary to systemic sclerosis. J Rheumatol 1992;19:1407–14. (6) Wigley FM, Wise RA, Seibold JR, McCloskey DA, Kujala G, Medsger TA Jr, et al. Intravenous iloprost infusion in patients with Raynaud phenomenon secondary to systemic sclerosis. A multicenter, placebo-controlled, double-blind study. Ann Intern Med 1994;120:199–206. (7) Badesch DB, Tapson VF, McGoon MD, Brundage BH, Rubin LJ, Wigley FM, et al. Continuous intravenous epoprostenol for pulmonary hypertension due to the scleroderma spectrum of disease. A randomized controlled trial. Ann Intern Med 2000;132:425–34. (8) EULAR recommendations for the treatment of systemic sclerosis: a report from the EULAR Scleroderma Trials and Research group (EUSTAR) O Kowal-Bielecka,1 R Landew ́e,2 J Avouac,3 S Chwiesko,1 I Miniati,4 L Czirjak,5 P Clements,6 C Denton,7 D Farge,8 K Fligelstone,9 I F ̈oldvari,10 D E Furst,6 U M ̈uller- Ladner,11 J Seibold,12 R M Silver,13 K Takehara,14 B Garay Toth,15 A Tyndall,16 G Valentini,17 F van den Hoogen,18 F Wigley,19 F Zulian,20 Marco Matucci-Cerinic,4 and the EUSTAR co-authors