systemic sclerosis (ssc)
TRANSCRIPT
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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
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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
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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
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Scleroderma: edematous changes, hands
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Scleroderma: puffy phase, hand
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Scleroderma: skin induration, hands
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Scleroderma: Mauskopf, facial changes
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Scleroderma: acrosclerosis
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Scleroderma: hands
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Scleroderma: digital pitting scars
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-Raynauds phenomenon
-Joints: - Poly arthrlgia and joint stiffness
(Small and large joint)
- Frank arthritis
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Scleroderma: acrolysis (radiographs)
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Scleroderma: calcinosis and acrolysis (radiograph)
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CREST syndrome: arm (radiograph)
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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
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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
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Scleroderma: Raynaud’s phenomenon, blanching of hands
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Raynaud’s phenomenon: hands
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Scleroderma: Raynaud’s phenomenon, cyanosis of the
hands
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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
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Scleroderma: abnormal motility, esophagus (radiograph)
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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
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Scleroderma: wide-mouthed diverticula, colon (radiograph)
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Scleroderma: large-mouth diverticula (radiograph)
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Lungs
Interstitial lung disease
- Dyspnea
- Pleurisy
Chest –x-ray: reticular pattern of linear, nodular and
line nodular densities "lower two –thirds"
- Subnormal diffusing capacity
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Scleroderma: pulmonary fibrosis (radiograph)
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Heart
Myocardial fibrosis
- Arrhythmias
- Cardiomyopathy
- Pulmonary arterial hypertension
- Pericarditis and pericardial effusion
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Kidney:
- Hypertension
- Renal crisis
- Fibrosis of the thyroid
- Biliary cirrhosis
- Trigeminal neuropathy
- Secondary sjogren
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Scleroderma: kidney (arteriograms)
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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
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Scleroderma: Mauskopf, facial changes
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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
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Linear scleroderma: en coup de sabre, scalp and forehead
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Linear scleroderma: thigh and leg
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Morphea: leg
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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
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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
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Eosinophilic fasciitis: cutaneous lesions, arm
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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
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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
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2D Echo cardiogram
- PAH
- Lt ventrical function (hypertrophy,
dyskynesis diastolic dysfunction)
- Pericardial effusion
-High resolution chest CTscan
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Treatment
skin involvement
Methotrexate may be considered for treatment of
skin manifestation
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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
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2-Pulmonary arterial Hypertension (PAH)
- Bosentan
- Sidenafil
- Intravenous epoprostenol for severe PAH
-Interstitial lung fibrosis
. Cyclophosphamide
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3-Scleraderma renal crisis
- ACE inhibitors
- Avoid high dose of steroid