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University of the EAST Post Graduate School

Scleroderma
Joaquin masoud C. masoud, DMD,MScD Part 1: introduc:on, clinical feature, pathogenesis

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:on this results in small blood vessels damage and :ssue brosis.

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

Epidemiology
It is Rare in children Its Peak is between age 35-65 More women aected then men Family history of other auto immune diseases Ethnic background inuence survival and disease manifesta:on. ( in iran, the prevalence is more between caspian people)

Environmental factors 1) infec:on 2) occupa:onal exposures: silica dust

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 Raynauds phenomenon 4) early appearance of visceral involvement 5) poor prognosis

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

Clinical features
Raynaud s Typical skin changes Esophageal and small bowel dysfunction Interstitial lung disease Pulmonary hypertension Renal crises

What is Raynaud s Phenomenon


Reversible skin color changes:White to blue to red Due to vasospasm Induced by cold of emotion

Pallor phase cyano:c phase

Causes of Raynaud s
Occlusive arterial disease Rheuma:c diseases:Scleroderma,CREST,MCTD,SLE,RA, Myosi:s Repe::ve vascular injury Hyperviscosity :Polycythemia,Cryoglobulinemi a Thoracic outlet syndrome

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

Esophageal dysmo:lity:heart burn,and reux symptoms


Dilated esophagus on Esophagogram

Inters::al Lung disease


Major cause of morbidly

Renal crises and hypertension in scleroderma


Major complica: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

Clinical feature
2.skin involvement: 1) -edematous phase -indurative phase -atrophic phase 2) firm,thickened bound to underlying soft tissue 3) Decrease in range of motion , loss of facial expression, inability to open mouth fully

Typical skin changes


Tight thick skin,peaked nose Pursed mouth

Salt and pepper pigmenta:on

microstomia

Cold sensi:vity

Edematous phase

Skin Indura:on

Acrosclerosis

Skin microscopy

Terminal digit resorp:on

Acrolysis

Digital pidng scars

CREST syndrome: calcinosis cu:s

Calcinosis and acrolysis

Telangiectasia
Face / mucous membrane blanched by pressure

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:on

4) large intes:ne: chronic cons:pa:on and fecal impac:on diver:cula

Abnormal mo:lity

Diver:cula
Barium enema study - mul:ple wide-mouthed diver:cula of colon - broad base and neck - usually asymptoma:c

Diver:cula

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)

Pulmonary brosis

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

Kidney arteriogram

Kidney, In:mal arterial brosis

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

Pathogenesis

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%

Pathogenesis
4. Environmental factors 1) silica dust 2) organic solvents 3) biogenic amines 4) urea formaldehyde 5) polyvinyl chloride 6) rapeseed oil 7) bleomycin 8) L-tryptophan 9) silicone implant

Part 2- to be discussed by Dr. Muzamil wani

Thank you