Systemic sclerosis (SSC)

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Dorothy Davis
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1 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 years

2 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

3 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

4 Scleroderma: edematous changes, hands

5 Scleroderma: puffy phase, hand

6 Scleroderma: skin induration, hands

7 Scleroderma: Mauskopf, facial changes

8 Scleroderma: acrosclerosis

9 Scleroderma: hands

10 Scleroderma: digital pitting scars

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

12 Scleroderma: acrolysis (radiographs)

13 Scleroderma: calcinosis and acrolysis (radiograph)

14 CREST syndrome: arm (radiograph)

15 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

16 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

17 Scleroderma: Raynaud s phenomenon, blanching of hands

18 Raynaud s phenomenon: hands

19 Scleroderma: Raynaud s phenomenon, cyanosis of the hands

20 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

21 Scleroderma: abnormal motility, esophagus (radiograph)

22 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

23 Scleroderma: wide-mouthed diverticula, colon (radiograph)

24 Scleroderma: large-mouth diverticula (radiograph)

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

26 Scleroderma: pulmonary fibrosis (radiograph)

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

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

29 Scleroderma: kidney (arteriograms)

30 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

31 Scleroderma: Mauskopf, facial changes

32 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

33 Linear scleroderma: en coup de sabre, scalp and forehead

34 Linear scleroderma: thigh and leg

35 Morphea: leg

36 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

37 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

38 Eosinophilic fasciitis: cutaneous lesions, arm

39 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

40 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

41 2D Echo cardiogram - PAH - Lt ventrical function (hypertrophy, dyskynesis diastolic dysfunction) - Pericardial effusion -High resolution chest CTscan

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

43 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

44 2-Pulmonary arterial Hypertension (PAH) - Bosentan - Sidenafil - Intravenous epoprostenol for severe PAH -Interstitial lung fibrosis. Cyclophosphamide

45 3-Scleraderma renal crisis - ACE inhibitors - Avoid high dose of steroid

46 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

SCLERODERMA. Scleroderma update. No disclosures or conflicts. Leslie Kahl, M.D. April 10, 2015

SCLERODERMA. Scleroderma update. No disclosures or conflicts. Leslie Kahl, M.D. April 10, 2015 Scleroderma update Leslie Kahl, M.D. April 10, 2015 No disclosures or conflicts KT is a 45 year old woman who developed puffiness in her fingers 1/2013 and carpal tunnel syndrome and arthralgias 3/2013.