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What is Raynaud’s phenomenon? What is Scleroderma? What is the link? Dr John Pauling BMedSci MRCP (Rheumatology) PhD Consultant Rheumatologist RNHRD

What is scleroderma? What is Raynaud's?

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Page 1: What is scleroderma? What is Raynaud's?

What is Raynaud’s phenomenon? What is Scleroderma? What is the link?

Dr John Pauling BMedSci MRCP (Rheumatology) PhD

Consultant Rheumatologist

RNHRD

Page 2: What is scleroderma? What is Raynaud's?

•  “What is Raynaud’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

Page 3: What is scleroderma? What is Raynaud's?

Raynaud’s phenomenon

Maurice Raynaud (1834 – 1881)

De l'asphyxie locale et de la gangrène symétrique des

extrémités.

Doctoral thesis, published February 25, 1862.

Page 4: What is scleroderma? What is Raynaud's?

Initial ischaemia

Pallor

Cyanotic phase

Blue

Hyperaemic phase

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

Page 5: What is scleroderma? What is Raynaud's?

'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

Page 6: What is scleroderma? What is Raynaud's?

www.rnhrd.nhs.uk

Systemic sclerosis (SSc) / Scleroderma

Page 7: What is scleroderma? What is Raynaud's?

www.rnhrd.nhs.uk

Another way of approaching systemic sclerosis

Systemic sclerosis

Blood vessel abnormalities

Fibrosis / scar tissue

Inflammation

Fatigue??

Page 8: What is scleroderma? What is Raynaud's?

www.rnhrd.nhs.uk

A Simpler way of approaching scleroderma

Systemic sclerosis

Blood vessel abnormalities

Fibrosis / Scar tissue Inflammation

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??

Page 9: What is scleroderma? What is Raynaud's?

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

Page 10: What is scleroderma? What is Raynaud's?

Antinuclear autoantibodies

Anticentromere Anti-DNA Anti-topoisomerase Anti-nucleolar

Page 11: What is scleroderma? What is Raynaud's?

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

Page 12: What is scleroderma? What is Raynaud's?

www.rnhrd.nhs.uk

Is Raynaud’s phenomenon in systemic sclerosis actually Raynaud’s phenomenon?

A healthy arteriole Systemic sclerosis

Page 13: What is scleroderma? What is Raynaud's?

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

Page 14: What is scleroderma? What is Raynaud's?

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

Page 15: What is scleroderma? What is Raynaud's?

www.rnhrd.nhs.uk

Theories on the link 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

Page 16: What is scleroderma? What is Raynaud's?

www.rnhrd.nhs.uk

Theories on the link 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

Page 17: What is scleroderma? What is Raynaud's?

www.rnhrd.nhs.uk

Theories on the link 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

Page 18: What is scleroderma? What is Raynaud's?

www.rnhrd.nhs.uk

Theories on the link 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

Page 19: What is scleroderma? What is Raynaud's?

•  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?

Page 20: What is scleroderma? What is Raynaud's?

“The captive” 1940

•  Nazi’s 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