Systemic sclerosis (SSC)

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

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

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

Scleroderma: edematous changes, hands

Scleroderma: puffy phase, hand

Scleroderma: skin induration, hands

Scleroderma: Mauskopf, facial changes

Scleroderma: acrosclerosis

Scleroderma: hands

Scleroderma: digital pitting scars

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

Scleroderma: acrolysis (radiographs)

Scleroderma: calcinosis and acrolysis (radiograph)

CREST syndrome: arm (radiograph)

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

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

Scleroderma: Raynaud s phenomenon, blanching of hands

Raynaud s phenomenon: hands

Scleroderma: Raynaud s phenomenon, cyanosis of the hands

Esophagus "50%" (abnormal esopheal motility due to loss of smooth muscle function) esophagus - Heartburn or dyspepsia - Esophageal reflux - Peptic esophagitis - Dilatation of the lower one half to two thirds of the - Hiatus hernia

Scleroderma: abnormal motility, esophagus (radiograph)

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

Scleroderma: wide-mouthed diverticula, colon (radiograph)

Scleroderma: large-mouth diverticula (radiograph)

Lungs Interstitial lung disease - Dyspnea - Pleurisy Chest x-ray: reticular pattern of linear, nodular and line nodular densities "lower two thirds" - Subnormal diffusing capacity

Scleroderma: pulmonary fibrosis (radiograph)

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

Kidney: - Hypertension - Renal crisis - Fibrosis of the thyroid - Biliary cirrhosis - Trigeminal neuropathy - Secondary sjogren

Scleroderma: kidney (arteriograms)

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

Scleroderma: Mauskopf, facial changes

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

Linear scleroderma: en coup de sabre, scalp and forehead

Linear scleroderma: thigh and leg

Morphea: leg

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

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

Eosinophilic fasciitis: cutaneous lesions, arm

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

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

2D Echo cardiogram - PAH - Lt ventrical function (hypertrophy, dyskynesis diastolic dysfunction) - Pericardial effusion -High resolution chest CTscan

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

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

2-Pulmonary arterial Hypertension (PAH) - Bosentan - Sidenafil - Intravenous epoprostenol for severe PAH -Interstitial lung fibrosis. Cyclophosphamide

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

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