Sleroderma pdf-Dr. Jm Shafiee

7/31/2019 Sleroderma pdf-Dr. Jm Shafiee

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SclerodermaJoaquin masoud C. masoud, DMD,MScD

Part 1: introduc on, clinical feature,pathogenesis

University of the EASTPost Graduate School


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scleroderma, (systemic sclerosis)

Autoimmune rheuma c diseases Its not contagious, it is not infec ous, it is not

cancerous or malignant.

Its a chronic disease Mul system disease of unknown e ology Pathogenesis include immune system ac va on ,

endothelial ac va on , and broblast ac va onthis results in small blood vessels damage andssue brosis.


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Deni&on

1. Systemic sclerosis (scleroderma) - a mul system disorder characterized by

1) func onal and structural abnormali es of blood

vessels 2) brosis of the skin and internal organs 3) immune system ac va on

4) autoimmunity

2. Localized scleroderma - morphea, linear scleroderma


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Epidemiology

It is Rare in children Its Peak is between age 35-65 More women affected then men Family history of other auto immune diseases Ethnic background inuence survival and

disease manifesta on.

( in iran, the prevalence is more betweencaspian people)


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Environmental factors

1) infec on

2) occupa onal exposures: silica dust


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Classica&on of systemic sclerosis

1. Diffuse cutaneous systemic sclerosis

1) proximal skin thickening

- distal and proximal extremity and often the trunk and

face

2) tendency to rapid progression of skin change

3) rapid onset of disease following Raynaudsphenomenon

4) early appearance of visceral involvement

5) poor prognosis


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2. Limited cutaneous systemic sclerosis 1) symmetric restricted fibrosis - affecting the distal extremities and face/neck 2) prolonged delay in appearance of distinctive

internal manifestation 3) prominence of calcinosis and telangiectasia 4) good prognosis

* CREST syndrome - calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia


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Clinical features

Raynaud s Typical skin changes

Esophageal and small bowel dysfunction Interstitial lung disease Pulmonary hypertension

Renal crises


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What is Raynaud s Phenomenon

Reversible skin color changes:White toblue to red

Due to vasospasm Induced by cold of emotion


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Pallor phase cyano c phase


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Causes of Raynaud s

Occlusive arterial disease Rheuma c

diseases:Scleroderma,CREST,MCTD,SLE,RA,Myosi s

Repe ve vascular injury

Hyperviscosity :Polycythemia,Cryoglobulinemia

Thoracic outlet syndrome


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Clinical features

1. Vascular abnormali es 1) Raynaud's phenomenon - cold hands and feet

with reversible skin color change (white to blue to red) - induced by cold temperature or emo onal stress - ini al complaint in 3/4 of pa ents - 90% in pa ents with skin change

(prevalence in the general popula on: 4-15%) 2) digital ischemic injury


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Esophageal dysmo lity:heart burn,andreux symptoms

Dilated esophagus on Esophagogram


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Inters al Lung disease

Major cause of morbidly


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Renal crises and hypertension inscleroderma

Major complic a on Early in disease rst few years Acute onset hypertension

High Renin Renal impairment Microangiopathic hemoly c anemia,Thrombocytopenia

Renal failure can be reversible if BP treated Drug of choice Angiotensin conver ng enzyme inhibitor


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2.skin involvement:

1)

-edematous phase-indurative phase-atrophic phase2) firm,thickened bound tounderlying soft tissue3) Decrease in range of motion ,loss of facial expression, inabilityto open mouth fully

Clinical feature


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Typical skin changes

Tight thick skin,peaked nose Pursed mouth


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Salt and pepper pigmenta on


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microstomia


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Cold sensi vity


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Edematous phase


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Skin Indura on


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Acrosclerosis


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Skin microscopy


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Terminal digit resorp on


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Acrolysis


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Digital pi ng scars


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CREST syndrome: calcinosis cu s


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Calcinosis and acrolysis


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Telangiectasia

Face / mucous

membrane

blanched by pressure


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Clinical features

4. intes nal involvement

1) esophagus: hypomo lity and retrosternal pain,

reux esophagi s, stricture

2) stomach: delayed emptying

3) small intes ne: pseudo-obstruc on, paraly c ileus,

malabsorp on4) large intes ne: chronic cons pa on and fecal

impac on diver cula


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Abnormal mo lity


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Diver cula

Barium enema study

- mul ple wide-mouthed

diver cula of colon

- broad base and neck

- usually asymptoma c


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Diver cula


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5. lungs 1) 2/3 of patients affected - leading cause of mortality and morbidity in later

stage of systemic sclerosis 2) pathology

- interstitial fibrosis - intimal thickening of pulmonary arterioles (pulmonary hypertension)


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Pulmonary brosis


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7.kidney 1) diffuse scleroderma in association with rapid progression of skin involvement

2) pathology - intimal hyperplasia of the interlobular artery - fibrinoid necrosis of afferent arterioles - glomerulosclerosis 3) proteinuria, abnormal sediment, azotemia, microangiopathic hemolytic anemia, renal failure


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Kidney arteriogram


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Kidney, In mal arterial brosis


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Pathogenesis 1. Vasculopathy of small artery and capillary - endothelial cell injury - adhesion and activation of platelet - PDGF, thromboxane A2 release

- vasoconstriction & growth of endothelial cell and fibroblast - narrowing or obliteration, increased permeability 2. Fibrosis - aberrant regulation of fibroblast cell growth - increased production of extracellular matrix (collagen, fibronectin, and glycosaminoglycan) - thickening of the skin & fibrosis of internal organs


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Pathogenesis


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Pathogenesis 3. Immunologic mechanism 1) cell mediated immunity - skin: cellular infiltrates in perivascular region and

dermis (T cell, Langerhans cell, plasma cell,

macrophage)

2) humoral immunity - hypergammaglobulinemia - autoantibody production antinuclear antibody (+) > 95%


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Pathogenesis

4. Environmental factors1) silica dust2) organic solvents3) biogenic amines

4) urea formaldehyde5) polyvinyl chloride6) rapeseed oil7) bleomycin8) L-tryptophan9) silicone implant


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Part 2- to be discussed by Dr. Muzamil wani


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Thank you

Sleroderma pdf-Dr. Jm Shafiee

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