Systemic sclerosis (SSC)

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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


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)

- Heartburn or dyspepsia

- Esophageal reflux

- Peptic esophagitis

- Dilatation of the lower one half to two thirds of the

esophagus

- 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

Systemic sclerosis (SSC)

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