Systemic sclerosis SSC Is a multi system autoimmune

Classification of scleroderma Localized scleroderma Morphea Linear scleroderma “En coup de sabre” Systemic sclerosis

Diffuse cutaneous SSC - Prodromal phase –arthrlgia, puffness of the finger raynaud - During

Scleroderma: edematous changes, hands Copyright © 1972 -2004 American College of Rheumatology Slide Collection.

Scleroderma: puffy phase, hand Copyright © 1972 -2004 American College of Rheumatology Slide Collection.

Scleroderma: skin induration, hands Copyright © 1972 -2004 American College of Rheumatology Slide Collection.

Scleroderma: Mauskopf, facial changes Copyright © 1972 -2004 American College of Rheumatology Slide Collection.

Scleroderma: acrosclerosis Copyright © 1972 -2004 American College of Rheumatology Slide Collection. All rights

Scleroderma: hands Copyright © 1972 -2004 American College of Rheumatology Slide Collection. All rights

Scleroderma: digital pitting scars Copyright © 1972 -2004 American College of Rheumatology Slide Collection.

-Raynauds phenomenon -Joints: - Poly arthrlgia and joint stiffness (Small and large joint) -

Scleroderma: acrolysis (radiographs) Copyright © 1972 -2004 American College of Rheumatology Slide Collection. All

Scleroderma: calcinosis and acrolysis (radiograph) Copyright © 1972 -2004 American College of Rheumatology Slide

CREST syndrome: arm (radiograph) Copyright © 1972 -2004 American College of Rheumatology Slide Collection.

Raynaud’s phenomenon Episodic, reversible digital skin color change white to blue to red well-demarcated

Causes of secondary Raynaud’s phenomenon Connective tissue diseases Scleroderma, systemic lupus erythematosus, MCTD, undifferentiated

Scleroderma: Raynaud’s phenomenon, blanching of hands Copyright © 1972 -2004 American College of Rheumatology

Raynaud’s phenomenon: hands Copyright © 1972 -2004 American College of Rheumatology Slide Collection. All

Scleroderma: Raynaud’s phenomenon, cyanosis of the hands Copyright © 1972 -2004 American College of

Esophagus "50%" (abnormal esopheal motility due to loss of smooth muscle function) - Heartburn

Scleroderma: abnormal motility, esophagus (radiograph) Copyright © 1972 -2004 American College of Rheumatology Slide

Diminished lower G I tract motility: - Pseudo-obstruction - Bloating, cramps - Constipation –diarrhea

Scleroderma: wide-mouthed diverticula, colon (radiograph) Copyright © 1972 -2004 American College of Rheumatology Slide

Scleroderma: large-mouth diverticula (radiograph) Copyright © 1972 -2004 American College of Rheumatology Slide Collection.

Lungs Interstitial lung disease - Dyspnea - Pleurisy Chest –x-ray: reticular pattern of linear,

Scleroderma: pulmonary fibrosis (radiograph) Copyright © 1972 -2004 American College of Rheumatology Slide Collection.

Heart Myocardial fibrosis - Arrhythmias - Cardiomyopathy - Pulmonary arterial hypertension - Pericarditis and

Kidney: - Hypertension - Renal crisis - Fibrosis of the thyroid - Biliary cirrhosis

Scleroderma: kidney (arteriograms) Copyright © 1972 -2004 American College of Rheumatology Slide Collection. All

Limited cutaneous SSC - More benign course - In most patient skin sclerosis remain

Localized scleroderma Morphea - One or more area of erythematous. Small or large plaques

Linear scleroderma: en coup de sabre, scalp and forehead Copyright © 1972 -2004 American

Linear scleroderma: thigh and leg Copyright © 1972 -2004 American College of Rheumatology Slide

Morphea: leg Copyright © 1972 -2004 American College of Rheumatology Slide Collection. All rights

Mixed connective tissue disease: proposed classification criteria The diagnosis of MCTD can be made

Scleroderma-like syndromes Toxin- or drug-induced scleroderma Vinyl chloride Organic solvents and epoxy resins Eosinophilic

Eosinophilic fasciitis: cutaneous lesions, arm Copyright © 1972 -2004 American College of Rheumatology Slide

Baseline Assessment - C B C, ESR, CRP, Thyroid function, Kidney function -Over lap

Pulmonary function test (PETs) - Total lunge capacity (TLC) - Forced vital capacity (FVC)

2 D Echo cardiogram - PAH - Lt ventrical function (hypertrophy, dyskynesis diastolic dysfunction)

Treatment skin involvement Methotrexate may be considered for treatment of skin manifestation Copyright ©

1 -Raynoud – digital ulcer's - Calcium antagonist - Intra venous iloprost for severe

2 -Pulmonary arterial Hypertension (PAH) - Bosentan - Sidenafil - Intravenous epoprostenol for severe

3 -Scleraderma renal crisis - ACE inhibitors - Avoid high dose of steroid Copyright

4 -G. I - PPI------for prevention of SS c -gastro Oesophageal reflex – ulcers

Slides: 46

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Systemic sclerosis (SSC) -Is a multi system autoimmune disease, characterized by fibrosis of the skin and variable pattern of other visceral -SSC: Is a relatively UN common disease -Prevalence in U S A 0, 01 -0, 03% -F: M (3: 1) -Age of onset of 30 -50 years Copyright © 1972 -2004 American College of Rheumatology Slide Collection. All rights reserved.

Classification of scleroderma Localized scleroderma Morphea Linear scleroderma “En coup de sabre” Systemic sclerosis Diffuse cutaneous Limited cutaneous CREST syndrome Systemic sclerosis sine scleroderma Overlap syndromes Scleroderma-like syndromes Copyright © 1972 -2004 American College of Rheumatology Slide Collection. All rights reserved.

Diffuse cutaneous SSC - Prodromal phase –arthrlgia, puffness of the finger raynaud - During the initial phase of disease the skin tends to be inflamed, edematous (no pitting) and erythematous with area of hyper-or hypo pigmentation - Rapid progression of skin changes from the fingers or feet to proximal involvement of the extremities extending above the knee or elbow or to the trunk - Deeper articular of periarticular inflammation or fibrosis (friction rub's) - Flexion contractures of joints (finger, wrists, elbow) - Active inflammatory phase----several weeks or month's - Fibrotic phase - Sclerosis, thickened skin with area of atrophy that can ulcerate at sites of trauma -This active phase is followed by variable degree of improvement - Subcutaneous calcification most commonly in finger tips - Internal organ involvement : Develops during the first 3 years of disease Copyright © 1972 -2004 American College of Rheumatology Slide Collection. All rights reserved.

-Raynauds phenomenon -Joints: - Poly arthrlgia and joint stiffness (Small and large joint) - Frank arthritis Copyright © 1972 -2004 American College of Rheumatology Slide Collection. All rights reserved.

Raynaud’s phenomenon Episodic, reversible digital skin color change white to blue to red well-demarcated Due to vasospasm Usually cold-induced Primary (Raynaud’s disease) and secondary forms Copyright © 1972 -2004 American College of Rheumatology Slide Collection. All rights reserved.

Causes of secondary Raynaud’s phenomenon Connective tissue diseases Scleroderma, systemic lupus erythematosus, MCTD, undifferentiated CTD, Sjogren’s syndrome, dermatomyositis Occlusive arterial disease Atherosclerosis, anti-phospholipid antibody syndrome, Buerger’s disease Vascular injury Frostbite, vibratory trauma Drugs and toxins Beta blockers, vinyl chloride, bleomycin, ergot, amphetamines, cocaine Hyperviscosity/cold-reacting proteins Paraproteinemia, polycythemia, cryoglobulinemia, cryofibrinogenemia, cold agglutinins Copyright © 1972 -2004 American College of Rheumatology Slide Collection. All rights reserved.

Esophagus "50%" (abnormal esopheal motility due to loss of smooth muscle function) - Heartburn or dyspepsia - Esophageal reflux - Peptic esophagitis - Dilatation of the lower one half to two thirds of the esophagus - Hiatus hernia Copyright © 1972 -2004 American College of Rheumatology Slide Collection. All rights reserved.

Diminished lower G I tract motility: - Pseudo-obstruction - Bloating, cramps - Constipation –diarrhea (which can be secondary to bacterial over growth) weight loss and malnutrition - Patchy atrophy of muscularis of large intestine ----wide mouthed diverticula's Copyright © 1972 -2004 American College of Rheumatology Slide Collection. All rights reserved.

Lungs Interstitial lung disease - Dyspnea - Pleurisy Chest –x-ray: reticular pattern of linear, nodular and line nodular densities "lower two –thirds" - Subnormal diffusing capacity Copyright © 1972 -2004 American College of Rheumatology Slide Collection. All rights reserved.

Heart Myocardial fibrosis - Arrhythmias - Cardiomyopathy - Pulmonary arterial hypertension - Pericarditis and pericardial effusion Copyright © 1972 -2004 American College of Rheumatology Slide Collection. All rights reserved.

Kidney: - Hypertension - Renal crisis - Fibrosis of the thyroid - Biliary cirrhosis - Trigeminal neuropathy - Secondary sjogren Copyright © 1972 -2004 American College of Rheumatology Slide Collection. All rights reserved.

Limited cutaneous SSC - More benign course - In most patient skin sclerosis remain limited to the finger - Raynouds phenomenon is present for years before diagnosis - Telangiectasias (over finger , face and lips) - Subcutaneous calcinosis - CREST - Anti Centromere antibody - Isolated PAH Copyright © 1972 -2004 American College of Rheumatology Slide Collection. All rights reserved.

Localized scleroderma Morphea - One or more area of erythematous. Small or large plaques then the skin becomes. Sclerotic and waxy or ivory colored. After several months or years spontaneous softing of the skin - Linear scleroderma. During child hood. Band of sclerosis appears in the upper or lower extremities or front parietal area of the forehead and scalp Copyright © 1972 -2004 American College of Rheumatology Slide Collection. All rights reserved.

Mixed connective tissue disease: proposed classification criteria The diagnosis of MCTD can be made if 3 (one of which must be myositis or synovitis) of 5 clinical criteria and anti-RNP are present Serologic criteria anti-RNP antibodies Clinical criteria swollen hands, synovitis, clinical or biopsy-proven myositis, Raynaud’s phenomenon, acrosclerosis with or without proximal systemic sclerosis Copyright © 1972 -2004 American College of Rheumatology Slide Collection. All rights reserved.

Scleroderma-like syndromes Toxin- or drug-induced scleroderma Vinyl chloride Organic solvents and epoxy resins Eosinophilic myalgia syndrome (L-tryptophan) Toxic oil syndrome Bleomycin Vibration injury Scleromyxedema Scleredema Eosinophilic fasciitis Graft-versus-host disease Copyright © 1972 -2004 American College of Rheumatology Slide Collection. All rights reserved.

Baseline Assessment - C B C, ESR, CRP, Thyroid function, Kidney function -Over lap disease RF, Anti CCP, Muscle enzyme, -ANA. Topo isomerase's (sci-70) - Diffuse. RNA poly merase 111 -Diffuse. Centromere - limited. PM-SCL - overlap. UI-RNP - M. C. T. D Copyright © 1972 -2004 American College of Rheumatology Slide Collection. All rights reserved.

Pulmonary function test (PETs) - Total lunge capacity (TLC) - Forced vital capacity (FVC) - Forced expiratory volume (FEV) - Diffusing capacity of carbon monoxide (DLCO). (decrease) TLC, FVC and FEV -------ILD. DLco progressively decline -----PAH. PETs -----base line and yearly Copyright © 1972 -2004 American College of Rheumatology Slide Collection. All rights reserved.

2 D Echo cardiogram - PAH - Lt ventrical function (hypertrophy, dyskynesis diastolic dysfunction) - Pericardial effusion -High resolution chest CTscan Copyright © 1972 -2004 American College of Rheumatology Slide Collection. All rights reserved.

1 -Raynoud – digital ulcer's - Calcium antagonist - Intra venous iloprost for severe raynoud - Intra venous prostanoids (iloprost) ---active digital ulcers - Bosentan should be considered in multiple ulcers after failure of calcium antagonist and prostanoid therapy Copyright © 1972 -2004 American College of Rheumatology Slide Collection. All rights reserved.

2 -Pulmonary arterial Hypertension (PAH) - Bosentan - Sidenafil - Intravenous epoprostenol for severe PAH -Interstitial lung fibrosis. Cyclophosphamide Copyright © 1972 -2004 American College of Rheumatology Slide Collection. All rights reserved.

4 -G. I - PPI------for prevention of SS c -gastro Oesophageal reflex – ulcers - Prokintec drugs for symptomatic motality Disturbance - Antibiotics for malabsorption caused by Bacteria Copyright © 1972 -2004 American College of Rheumatology Slide Collection. All rights reserved.