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What is scleroderma? What is Raynaud's?
Raynaud's phenomenon and scleroderma are linked vascular conditions. Raynaud's involves reduced blood flow to the extremities while scleroderma causes fibrosis of skin and organs. Damage to blood vessels from Raynaud's attacks may trigger antibodies, inflammation, and attempts to repair tissue through fibrosis. Understanding these pathways could help guide new treatments to prevent complications like lung scarring and kidney or lung failure.
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What is scleroderma? What is Raynaud's?
- 1. What is Raynaud’sphenomenon? What is Scleroderma? What is the link? Dr John Pauling BMedSci MRCP (Rheumatology) PhD Consultant Rheumatologist RNHRD
- 2. • “What isRaynaud’s phenomenon?” • “What is scleroderma?” • “How do these conditions affect the body?” • “What are antibodies important with this?” • “Friends say they have Raynaud’s too but my Raynaud’s seems worse. Are they the same? • “How are all these problems linked?” • “What causes scleroderma?” • “How can understanding the development of scleroderma help guide treatment?” www.rnhrd.nhs.uk What I will try to cover over next 30 minutes or so
- 3. Raynaud’s phenomenon Maurice Raynaud(1834 – 1881) De l'asphyxie locale et de la gangrène symétrique des extrémités. Doctoral thesis, published February 25, 1862.
- 4. Initial ischaemia Pallor Cyanotic phase Blue Hyperaemicphase Red / purple Clinical features of Raynaud’s phenomenon • Usually affects fingers • Can affect toes, thumbs, nipples, nose, earlobes • Episodes precipitated by cold exposure and emotional stress • Episodes accompanied by pain +/- numbness • “Primary” in the majority of cases (excellent prognosis) • “Secondary” forms rare e.g. systemic sclerosis but not to be missed
- 5. 'The head wascompletely withered, of a uniform shade of bronze, the nose was as thin as a knife-blade; the lips had almost disappeared. Two tiny hands of the same bronze colour slowly moved their fingers up and down like little sticks. And the face seemed all the more awesome to me because I could see that a smile was striving to appear on it, to cross its metallic cheeks - and yet could not spread.' With a shock, after the woman had said, “Master, don't you recognize me?”, he realizes that she is Lukeria, who ten years before had been 'the greatest beauty among all the servants in our house, tall, buxom, white- skinned and rosy-cheeked. Turgenev Living Relic 1874 Sketches from a Hunter's Album Scleroderma
- 6. www.rnhrd.nhs.uk Systemic sclerosis (SSc)/ Scleroderma
- 7. www.rnhrd.nhs.uk Another way ofapproaching systemic sclerosis Systemic sclerosis Blood vessel abnormalities Fibrosis / scar tissue Inflammation Fatigue??
- 8. www.rnhrd.nhs.uk A Simpler wayof approaching scleroderma Systemic sclerosis Blood vessel abnormalities Fibrosis / Scar tissueInflammation Raynaud’s phenomenon Pulmonary arterial hypertension Scleroderma renal crisis Skin thickening Lung fibrosis Gastrointestinal problems Bowel problems Antibody formation Inflammation in muscles Inflammation in joints Fatigue??
- 9. www.rnhrd.nhs.uk Subsets of systemicsclerosis (limited versus diffuse cutaneous SSc) Limited cutaneous SSc (lcSSc) • More vascular problems • Relatively mild skin involvement • Digital ulcers • Telangiectasia • Calcinosis • Late pulmonary arterial hypertension in minority of patients Diffuse cutaneous SSc (dcSSc) • More scar tissue problems • More widespread skin involvement • Lower bowel problems • Scar tissue in lungs
- 10. Antinuclear autoantibodies Anticentromere Anti-DNAAnti-topoisomerase Anti-nucleolar
- 11. Antibody subsets inscleroderma RNAP Topo-I Centromere Lung fibrosis Diffuse subtype (fibrosis) Limited subtype (vascular) Overlap features (inflammatory features) Kidney Th RNP RNAP II U3RNP U1RNP Pm-Scl PAH
- 12. www.rnhrd.nhs.uk Is Raynaud’s phenomenonin systemic sclerosis actually Raynaud’s phenomenon? A healthy arteriole Systemic sclerosis
- 13. www.rnhrd.nhs.uk Evaluating Raynaud’s phenomenon(microvascular imaging) Thermal Imaging Healthy nailfold capillaries (as is found in primary Raynaud’s phenomenon) Systemic sclerosis
- 14. www.rnhrd.nhs.uk Theories on thelink between inflammation, vascular abnormalities and fibrosis Damage to lining of blood vessels Antibodies Viruses Toxins e.g. solvents
- 15. www.rnhrd.nhs.uk Theories on thelink between inflammation, vascular abnormalities and fibrosis Damage to lining of blood vessels Intermittent Raynaud’s attacks Destruction of blood vessels Antibodies Viruses Toxins e.g. solvents
- 16. www.rnhrd.nhs.uk Theories on thelink between inflammation, vascular abnormalities and fibrosis Damage to lining of blood vessels Intermittent Raynaud’s attacks Complications Destruction of blood vessels Antibodies Viruses Toxins e.g. solvents Persistent Raynaud’s symptoms Digital ulcers Pulmonary arterial hypertension Scleroderma Renal crisis
- 17. www.rnhrd.nhs.uk Theories on thelink between inflammation, vascular abnormalities and fibrosis Damage to lining of blood vessels Intermittent Raynaud’s attacks Body recognises tissue damage Complications Destruction of blood vessels Reduced oxygen and nutrient supply to tissues Antibodies Viruses Toxins e.g. solvents Persistent Raynaud’s symptoms Digital ulcers Pulmonary arterial hypertension Scleroderma Renal crisis Body attempts to make new blood vessels
- 18. www.rnhrd.nhs.uk Theories on thelink between inflammation, vascular abnormalities and fibrosis Damage to lining of blood vessels Tissue fibrosis Intermittent Raynaud’s attacks Body recognises tissue damage Wound healing cells activated Complications Destruction of blood vessels Reduced oxygen and nutrient supply to tissues Antibodies Viruses Toxins e.g. solvents Persistent Raynaud’s symptoms Digital ulcers Pulmonary arterial hypertension Scleroderma Renal crisis Skin thickening Scarring of lungs Gastrointestinal dysmotility Body attempts to make new blood vessels
- 19. • We spentmuch of the 20th Century identifying the many different features and subtypes of systemic sclerosis • We are now beginning to better understand the triggers of systemic sclerosis and what happens on a cellular/molecular level to cause progression of the disease • Early identification and management of blood vessel abnormalities in scleroderma may help to prevent late complications of scleroderma • Emphasizes the importance of keeping warm, stopping smoking etc. • A lot of work still to do to understand the disease and develop a cure • The horizon looks very promising as this knowledge is being translated into potentially effective treatments to halt the progression and reverse complication of scleroderma www.rnhrd.nhs.uk What does all this mean for patients with systemic sclerosis?
- 20. “The captive” 1940 • Nazi’spresented his work as evidence of “Degenerate Art” • Fled to Switzerland 1933 • Diagnosed with SSc in 1935 • Died of cardiac complications 5 years later Paul Klee (1879-1940) “Death and Fire” 1940 The bars are thought to represent the restrictive effect of being trapped by his cutaneous fibrosis His face is etched with the letters “Tod” meaning death in German