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Overview of Scleroderma
- 1. Overview of SclerodermaOverviewof Scleroderma Scleroderma Patient Education Conference Apr 22, 2017 Nadera J Sweiss, MD, FACR Associate Professor of Medicine Director, Bernie Mac STAR Center and Cold Hand Clinic Section of Rheumatology, Section of Pulmonary Medicine
- 2. DisclosureDisclosure •None •I will discussnon FDA approved therapies
- 3. The Mission ofthe Scleroderma FoundationThe Mission of the Scleroderma Foundation Making life easier for patients and making strides toward a cure by focusing on three critical goals: •SUPPORT: To help patients and their families cope with scleroderma through mutual support programs, peer counseling, physician referrals, and educational information. •EDUCATION: To promote public awareness and education through patient and health professional seminars, literature, and publicity campaigns. •RESEARCH: To stimulate and support research to improve treatment and ultimately find the cause of and cure for scleroderma.
- 4.
- 5. SclerodermaScleroderma • Multisystem disorder •Unknown etiology • Thickening of skin caused by accumulation of connective tissue (collagen types I and III) • Involvement of visceral organs
- 6. What is Scleroderma?Whatis Scleroderma? • The word scleroderma comes from Greek, meaning hard skin. • Early symptoms of the disease often include cold hands, tightening of the skin, changes in facial features, joint pain, heartburn, shortness of breath and fatigue. • Patient awareness about scleroderma is important. It gives a better chance for early intervention, treatment and a return to normal routines and activities. Also, primary care physicians awareness of the disease allows them to make more referrals to specialists at earlier stages.
- 7. Classification of SclerodermaClassificationof Scleroderma • Systemic sclerosis, limited and diffuse • Localized sclerdoerma • Mimicks
- 8. Types of SclerodermaTypesof Scleroderma SclerodermaSclerodermaSclerodermaScleroderma LocalizedLocalized SclerodermaScleroderma LocalizedLocalized SclerodermaScleroderma SystemicSystemic SclerodermaScleroderma (Systemic Sclerosis)(Systemic Sclerosis) SystemicSystemic SclerodermaScleroderma (Systemic Sclerosis)(Systemic Sclerosis) MorpheaMorpheaMorpheaMorphea LinearLinear SclerodermaScleroderma LinearLinear SclerodermaScleroderma LimitedLimited SclerodermaScleroderma LimitedLimited SclerodermaScleroderma DiffuseDiffuse SclerodermaScleroderma DiffuseDiffuse SclerodermaScleroderma SineSine SclerodermaScleroderma SineSine SclerodermaScleroderma
- 9. Limited and DiffuseSSc— Skin Involvement Limited Diffuse
- 10. Overview of SclerodermaOverviewof Scleroderma • Peak age range: 35-64 • Younger age in women and with diffuse disease. • Female:Male = 3:1 • 8:1 in child bearing years • Incidence: 20/million per year in US • Prevalence: 240/million in US.
- 11. SclerodermaScleroderma • Limited Scleroderma •Skin thickening is distal to elbows and knees, not involving trunk • Less organ involvement • Isolated pulmonary hypertension can occur • Diffuse Scleroderma • Skin thickening proximal to elbows and knees, involving the trunk • More likely to have organ involvement • Pulmonary fibrosis and Renal Crisis are more common.
- 12. Limited vs diffuseSSc: sameLimited vs diffuse SSc: same or different disease?or different disease? • Raynaud in both; lcSSc more severe, earlier onset; complicated by critical ischemia • More prominent vascular features in lcSSc; • More prominent fibrosis in dcSSc • GERD prominent in both • ILD in both, oqen more severe in dcSSc • lcSSc generally indolent, better outcome
- 13. SYSTEMIC SCLEROSISSYSTEMIC SCLEROSISLIMITEDLIMITED (lcSSc)(lcSSc) Limited cutaneous systemic sclerosis (lcSSc aka CREST) – calcinosis – Raynaud’s – esophageal dysmotility – sclerodactyly – telangiectases
- 14. SYSTEMIC SCLEROSISSYSTEMIC SCLEROSIS DIFFUSECUTANEOUS (dcSSc)DIFFUSE CUTANEOUS (dcSSc) Diffuse cutaneous systemic sclerosis (dcSSc) – PSS (“progressive systemic sclerosis”) – SS (“scleroderma”)
- 15. Scleroderma manifestationScleroderma manifestation •Raynaud’s phenomenon • Skin • Gastrointestinal • Cardiovascular • Pulmonary • Renal • Systemic • Musculoskeletal (myositis, arthritis)
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- 18. Scleroderma AutoantibodiesScleroderma Autoantibodies AntigenANA Pattern Frequency Clinical Associations Organs Involved Scl-70 (topoisomerase 1) Speckled 10-40 dcSSC Lung fibrosis RNA Polymerase III Speck/Nuc 4-25 dcSSC Renal, Pulmonary HTN Centromere Centromere 15-40 lcSSc, CREST Pulmonary HTN Esophageal U1-RNP Speckled 5-35 lcSSC, MCTD Muscle U3 RNP (fibrillarin) Nucleolar 1-5 dcSSC, poor prognosis Muscle Pulmonary HTN PM-SCL Nucleolar 3-6 Overlap, mixed Muscle Th/To Nucleolar 1-7 lcSSc Pulmonary HTN, Lung fibrosis, Small bowel Anti U11/U12 Nucleolar 1-5 lcSSc & dcSSC Lung Fibrosis Anti-Ku 1-3 Overlap Ssc Muscle, Joint, SLE overlap Adapted from: Nihtyanova SI, Denton CP. Nat Rev Rheumatol 2010; 6:112
- 20. 2013 ACR DiagnosticCriteria2013 ACR Diagnostic Criteria
- 21. Scleroderma ChallengesScleroderma Challenges •While there is no cure for scleroderma, with early intervention, it’s often possible to manage the symptoms and offer a lifetime of hope. With early diagnosis of the disease, better treatment can be offered to patients. • Scleroderma can also happen in men, infants and seniors. It happens with women 4 times greater than with men. • Scleroderma continues to be a challenging disease, that requires: finding a cure, and/or at the very least, finding a better treatment for patients so that they can live a better life.
- 22. Cardiac InvolvementCardiac Involvement Adaptedfrom Desai, et al; Curr Opin Rheumatol 2011m 23:545-554 Cardiac Manifestation Prevalence Diagnosis Treatment Myocarditis Rare Cardiac MRI, Biopsy Cytoxan + steroids Pericardial effusion 5-16% Echocardiogram None; NSAIDs if symptomatic Microvascular CAD > 60% MRI/nuclear medicine Calcium channel blockers Macrovascular CAD 25% Coronary Angiogram Stenting/medical tx Bradyarrhythmias Rare EKG/Holter Pacemaker Tachyarrhythmias 15% EKG/Holter Diltiazem, ablation, defibrillator
- 23. Digital Ischemia/ Ulcersin Systemic SclerosisDigital Ischemia/ Ulcers in Systemic Sclerosis Clinical PresentationClinical Presentation
- 24. Clinical PresentationClinical Presentation DigitalIschemia/ Ulcers in Systemic Sclerosis andDigital Ischemia/ Ulcers in Systemic Sclerosis and MimicsMimics
- 25. Digital Ischemia/ Ulcersin Systemic SclerosisDigital Ischemia/ Ulcers in Systemic Sclerosis Therapeutic OptionsTherapeutic Options • AVOID THE COLD • Vasodilator • Target the endothelium • Target circulating cells • Inhibit mediators of vascular disease • Treat consequences of ischemia- reperfusion tissue injury
- 26. 26Fat Transfer inRaynaud’s Treatment Ca Channel Blockers ARB Protective Measures Protective Measures PDE-I Clopidogre l Endothelin Receptor Blockers Alpha Blockers Topical Nitrates ACE-I ASA Prostanoid s
- 27. 27Fat Transfer inRaynaud’s Treatment Ca Channel Blockers ARB Protective Measures Protective Measures PDE-I Clopidogre l Endothelin Receptor Blockers Alpha Blockers Topical Nitrates ACE-I ASA Prostanoid s Invasive Modalities Surgical Sympathec tomy Neuro- modulators Fat Transfer Fat Transfer
- 28. Periarterial Symapthectomy inthePeriarterial Symapthectomy in the Treatment of Digital IschemiaTreatment of Digital Ischemia
- 29. 29Fat Transfer inRaynaud’s Technique
- 30. 30Fat Transfer inRaynaud’s
- 32. Quantitative Flow Measurementof Digital Arteries at 3 TeslaQuantitative Flow Measurement of Digital Arteries at 3 Tesla Guanzhong Liu, Kezhou Wang, Xinjian Du, Yi Sui1, Michael P Flannery,Guanzhong Liu, Kezhou Wang, Xinjian Du, Yi Sui1, Michael P Flannery, Fady Charbel, Nadera Sweiss, and X. Joe ZhouFady Charbel, Nadera Sweiss, and X. Joe Zhou a ) b ) A 3D angiogram showing all proper palmer digital arteries from a representative female volunteer. (b) A zoomed view of PPDA2 showing the automatically determined perpendicular plane for the subsequent 2D PC acquisition
- 33. ConclusionConclusion • Scleroderma ismultisystem heterogeneous disiease; individualized therapy is warranted • Outcome measures need to be defined • Translational team approach will be helpful to identify responders
- 34.
Editor's Notes
- #27 Initial treatment is preventative – with gloves and cold avoidance But many patients progress to require medical treatment with calcium channel blockers and topical nitrates And there is a whole host of other medications aimed at vasodilation and prevention of arterial thrombosis
- #28 Despite all of these options, A portion of these patients develop severe, refractory symptoms And this is where the hand surgeon comes in to play, invoking invasive modalities With injection of neuromodulators such as botulinum toxin – which has shown to be effective, but short lived, and can be an arduous undertaking for the patient and surgeon when done in the clinic setting Another option would be surgical sympathectomy of the hands of affected feet – this is not a trivial undertaking, with inherent procedural risks, and requires somewhat specialized training and my not be long-lived with the regrowth of the stripped adventitia The intervention we propose he today is autologous fat grafting into the affected extremities
- #30 Here you can see the fat being injected while withdrawing the cannula
- #31 An example of fat transfer to the feet