What is Scleroderma? What is Raynaud's?

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Transcript of What is Scleroderma? What is Raynaud's?

  • What is Raynauds phenomenon? What is Scleroderma? What is the link? Dr John Pauling BMedSci MRCP (Rheumatology) PhD Consultant Rheumatologist RNHRD
  • Thank you for joining us to celebrate world scleroderma day (29th June) Relaxed morning with opportunities for questions and answers Opportunity to meet the team from RNHRD Opportunity to meet the teams from the major UK national scleroderma charities www.rnhrd.nhs.uk Welcome
  • Thank you for the support of the UK Raynauds and Scleroderma charities in facilitating this event www.raynauds.org.uk www.sclerodermasociety.co.uk
  • Other avenues are available to patients with Raynauds and Scleroderma needing support
  • What is Raynauds phenomenon? What is scleroderma? How do these conditions affect the body? What are antibodies important with this? Friends say they have Raynauds too but my Raynauds 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
  • Raynauds phenomenon Maurice Raynaud (1834 1881) De l'asphyxie locale et de la gangrne symtrique des extrmits. Doctoral thesis, published February 25, 1862.
  • Initial ischaemia Pallor Cyanotic phase Blue Hyperaemic phase Red / purple Clinical features of Raynauds 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
  • 'The head was completely 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
  • www.rnhrd.nhs.uk Systemic sclerosis (SSc) / Scleroderma
  • www.rnhrd.nhs.uk Another way of approaching systemic sclerosis Systemic sclerosis Blood vessel abnormalities Fibrosis / scar tissue Inflammation Fatigue??
  • www.rnhrd.nhs.uk A Simpler way of approaching scleroderma Systemic sclerosis Blood vessel abnormalities Fibrosis / Scar tissueInflammation Raynauds phenomenon Pulmonary arterial hypertension Scleroderma renal crisis Skin thickening Lung fibrosis Gastrointestinal problems Bowel problems Antibody formation Inflammation in muscles Inflammation in joints Fatigue??
  • www.rnhrd.nhs.uk Subsets of systemic sclerosis (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
  • Antinuclear autoantibodies Anticentromere Anti-DNA Anti-topoisomerase Anti-nucleolar
  • Antibody subsets in scleroderma 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
  • www.rnhrd.nhs.uk Is Raynauds phenomenon in systemic sclerosis actually Raynauds phenomenon? A healthy arteriole Systemic sclerosis
  • www.rnhrd.nhs.uk Evaluating Raynauds phenomenon (microvascular imaging) Thermal Imaging Healthy nailfold capillaries (as is found in primary Raynauds phenomenon) Systemic sclerosis
  • www.rnhrd.nhs.uk So how does this all link together? The Vascular Hypothesis
  • www.rnhrd.nhs.uk Theories on the link between inflammation, vascular abnormalities and fibrosis Damage to lining of blood vessels Antibodies Viruses Toxins e.g. solvents
  • www.rnhrd.nhs.uk Theories on the link between inflammation, vascular abnormalities and fibrosis Damage to lining of blood vessels Intermittent Raynauds attacks Destruction of blood vessels Antibodies Viruses Toxins e.g. solvents
  • www.rnhrd.nhs.uk Theories on the link between inflammation, vascular abnormalities and fibrosis Damage to lining of blood vessels Intermittent Raynauds attacks Complications Destruction of blood vessels Antibodies Viruses Toxins e.g. solvents Persistent Raynauds symptoms Digital ulcers Pulmonary arterial hypertension Scleroderma Renal crisis
  • www.rnhrd.nhs.uk Theories on the link between inflammation, vascular abnormalities and fibrosis Damage to lining of blood vessels Intermittent Raynauds attacks Body recognises tissue damage Complications Destruction of blood vessels Reduced oxygen and nutrient supply to tissues Antibodies Viruses Toxins e.g. solvents Persistent Raynauds symptoms Digital ulcers Pulmonary arterial hypertension Scleroderma Renal crisis Body attempts to make new blood vessels
  • www.rnhrd.nhs.uk Theories on the link between inflammation, vascular abnormalities and fibrosis Damage to lining of blood vessels Tissue fibrosis Intermittent Raynauds 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 Raynauds symptoms Digital ulcers Pulmonary arterial hypertension Scleroderma Renal crisis Skin thickening Scarring of lungs Gastrointestinal dysmotility Body attempts to make new blood vessels
  • www.rnhrd.nhs.uk Another way of looking at it - Normal wound repair
  • We spent much 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?
  • The captive 1940 Nazis presented 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