DocSpot what patients want to know

Size: px

Start display at page:

Download "DocSpot what patients want to know"

Chastity Davis
5 years ago
Views:

1 DocSpot what patients want to know Professor Chris Denton Royal Free Hospital London, UK Madrid, February 2012

3 DocSpot Live! Chris Denton Royal Free Hospital

4 DocSpot Live! Chris Denton Royal Free Hospital

5 I have been diagnosed with scleroderma and the biggest problem I have is with itching why is this and what can be done?

6 I have been diagnosed with scleroderma and the biggest problem I have is with itching why is this and what can be done? Itching of the skin (pruritis) is common especially in dcssc Tends to occur in early disease Due to mast cell degranulation and inflammatory cells and mediators Almost always gets better when the skin score falls improvement is a sign that disease is stabilising Other medical causes: Skin disease eczema, dermatitis herpetiformis (coeliac disease) Iron deficiency, thyroid disease Liver or kidney problems (PBC)

7 Natural history of itching in scleroderma intensity In diffuse SSc itching is a sign of activity years Duration of disease

8 Management of itching in scleroderma Determine any other medical cause Skin care emolients/moisturisers/counter-irritants Medication antihistamines (drowsiness) corticosteroids low dose oral prednisolone (topical disappointing) mast cell stabilisation ketotifen disappointing results Leukotriene inhibitors montelukast anecdotal benefit Treat the underlying disease immunosuppression MTX, MMF etc... topical MMF topical tacrolimus

9 I have Raynaud s and feel tired all the time and wonder if there is a link between Raynaud s phenomenon and chronic fatigue syndrome?

10 I have Raynaud s and feel tired all the time and wonder if there is a link between Raynaud s phenomenon and chronic fatigue syndrome? There is no direct link However any chronic health problem can affect general wellbeing. Some associated CTD may cause profound fatigue (SLE, SSc, SS) Consider the Raynaud s Definition of the type of RP Try to minimise the impact of Raynaud s on lifestyle Education, drugs, supplements.

11 Treatment of fatigue Medical approach: General medical assessment e.g. thyroid disease (in 30% of SSc) anaemia etc SSc depression Treatment of underlying connective tissue disease if present (e.g. hydroxychloroquine, azathioprine, low dose prednisolone) In SLE graded exercise programmes have shown promise in clinical trials

12 I have hard lumps of chalk under my skin and have CREST what does this mean?

13 CREST Calcinosis Rayaud s oesophageal dysmotility Sclerodactyly Telangiectasia

14 Major clinical subsets of systemic sclerosis Limited cutaneous SSc Diffuse cutaneous SSc

15 Calcinosis

18 Mechanism of calcinosis in scleroderma Calcinosis deposition of calcium containing salts in soft connective tissue - calcium hydroxyapetite NOT just in CREST also late stage dcssc Cause: Trauma Poor blood supply Altered extracellular matrix Genetics Abnormal metabolism - TGFβ1 and BMPs? Research ongoing (Ariane Herrick, Hope Hospital)

(Lithotripsy sound shock waves) Medical therapy none proven optimise Raynaud s

19 Simple approaches: Wax massage avoid interference Treatment of calcinosis Treat secondary infection Surgical excision open surgery dental drill extraction (Lithotripsy sound shock waves) Medical therapy none proven optimise Raynaud s therapy Diltiazem? Warfarin? Bisphosphonates Minocycline Anti-TNFα and methotrexate in severe cases?

20 The opposite of calcinosis acro-osteolysis

21 My sister has rheumatoid arthritis is there any association with me having scleroderma?

Overlap syndromes $ 8902.8. 0748 8 $$. 8,884.,90/ 9. 3.,,3/8074 4., 10,9:70841,349 07.4330.

22 Overlap syndromes $ $$. 8,884.,90/ 9. 3.,,3/ , 10,9:70841, ;09 88:0/ 80,80 31,22,947, $ ö /7420 :5:8 myositis SSc arthritis lupus Sjögrens

23 RA-scleroderma overlap syndrome RF positive arthritis - may be erosive scleroderma - ANA positive often SSc-specific serology

24 Power doppler USS hands Extensor digitorum tenosynovitis

25 Frequency of overlap SSc in the Royal Free Hospital scleroderma cohort $$. 2 90/$$. 11:80$$. $$.4;07,58 3/7420 n = Pakozdi et al, J Rheum 2011; 38: ;07,510,9: :, / 897 -:90/ / 11:80,3/ 2 90/8:-809

26 Distribution of patients with SSc overlap syndromes 4;07,5$$. $$ $$. 31,22,947, $$.$ ö /7420 $$. :5:8 % Pakozdi et al, J Rheum 2011; 38:

27 Management of systemic sclerosis SYSTEMIC SCLEROSIS lcssc dcssc Overlap SSc Therapy: Vascular Therapy: Vascular Immunosuppressive Antifibrotic Identification and treatment of organbased complications Pakozdiet al J Rheum 2011 Manage according to severity and activity of overlap features arthritis, myositis, lupus Overlap features 332 of 1700 (20%) SSc cases Myositis 43% Arthritis 32% Sjogren s 17% SLE 8%

28 Why do I have so much pain in my hands?

29 Causes for musculoskeletal pain in systemic sclerosis Contractural arthropathy Contractural tendinopathy Arthritis Myositis Fasciitis Connective tissue atrophy Calcinosis Neuropathic pain Ischaemic digital vasculopathy Sclerotic Inflammatory Dystrophic Neurovascular

30 Common musculoskeletal manifestations of systemic sclerosis Rheumatoid arthritis overlap tendinitis arthritis

31 Other causes for musculoskeletal pain in systemic sclerois Massive calcific periarthritis Gangrene associated with macrovascular occlusion

32 Therapy for upper limb pain in scleroderma Pain killers Physical therapy Disease modifying drugs for arthritis (DMARDs) SSc management Multidisciplinary management Pain Vascular specialists Nurses Psychologist Physician Surgeon Physiotherapist Occupational therapist

33 I have scleroderma and cannot get out of a chair unaided why?

SSc/myositis overlap % 0570;, 03.0419 0/ 11:808:-809 3 $$.2 48 9 84;07,5 870,9 ;0.425,70/9449 07$$.4;07,58 3/74208!

34 SSc/myositis overlap % 0570;, / 11:808: $$ ;07,5 870,9 ;0.425,70/ $$.4;07,58 3/74208!$ , 3,:94, , $$ ;07,58 3/7420 3#!,3/&#!, 84,884.,90/1706:039 14:3/ 3$$ ;07,5

35 Scleroderma hallmark autoantibodies associated with overlap myositis Anti-Fibrillarin Anti-nRNP Anti-PM-Scl

36 T2W image MRI Lumbar spine back pain and weakness

37 Why am I taking drugs for arthritis when I have been diagnosed with scleroderma?

38 Immunosuppressive therapy for dcssc Potential immunomodulatory strategies Methotrexate Cyclophosphamide Azathioprine Mycophenolate mofetil Anti-thymocyte globulin Ciclosporin, Tacrolimus, Early Basiliximab, stage dcsscrituximab Late stage dcssc Rapamycin Stem cell transplant (autologous/allogeneic) Oral tolerance to type I collagen

Skin score change in UK observational study 0f dcssc Skin score over time, by treatment protocol Cyclo then MMF ATG then MMF MMF 50 40 30 Herrick et al, J Rheum (2010); 37: 116-24.

39 Skin score change in UK observational study 0f dcssc Skin score over time, by treatment protocol Cyclo then MMF ATG then MMF MMF Herrick et al, J Rheum (2010); 37: mrss Baseline Year1 Year2 Year3 Baseline Year1 Year2 Year3 Baseline Year1 Year2 Year3 No active therapy Other (MTX etc) 147 patients from 12 centres No difference between protocols Intention to treat analysis Deterioration usually led to intensified therapy 10 0 Baseline Year1 Year2 Year3 Baseline Year1 Year2 Year3 ESOS EULAR-ODP funded MTX, cyclophosphamide, MMF ~400 subjects Herrick et al, J Rheum (2010); 37:

40 Any more questions?

Having a stem cell transplant for scleroderma a patient and doctor perspective

Having a stem cell transplant for scleroderma a patient and doctor perspective Professor Chris Denton, On behalf of an excellent team across UCLPartners Royal Free Hospital and University College London,