SYSTEMIC SCLEROSIS

By:- Vaibhav Swarup

 BACKGROUND

• Also known as scleroderma,

• derived from Greek words skleros (Hard) and

derma (skin).
 DEFINITION

• It’s a multisystem disorder is characterized by

• 1) functional and structural abnormalities of blood
vessels
• 2) fibrosis of the skin and internal organs
• 3) immune system activation
• 4) autoimmunity
 EPIDEMIOLOGY

• 1. Prevalencе: 4-12 new cases per million per year

• 2. Susceptibility: host factor
• 1) gender - female
• 2) age - peak occurrence: age 35-65 years
• 3) genetic background
 CLASSIFICATION

• 1. Linear scleroderma
aka. Localized scleroderma
Morphea is present
Presence only of hard skin
 CLASSIFICATION

• 2. Limited scleroderma
1) symmetric restricted fibrosis - affecting the distal
extremities and face / neck
2) prolonged delay in appearance of distinctive
internal manifestation
3) prominence of calcinosis and telangiectasia
4) good prognosis
 CLASSIFICATION

• CREST syndrome –
• C – calcinosis,
• R – Raynaud’s phenomenon,
• E – esophageal dysmotility,
• S – sclerodactyly,
• T – telangiectasia
CALCINOSIS
RAYNAUD’S PHENOMENON
TELANGIECTASIA
 CLASSIFICATION

• 3. Diffuse scleroderma
1) proximal skin thickening - distal and proximal extremity
and often the trunk and face
2) tendency to rapid progression of skin change
3) rapid onset of disease following Raynaud's
phenomenon
4) early appearance of visceral involvement
5) poor prognosis
 PATHOGENESIS

Susceptible host

Exogenous events

Immune system activation

Endothelial cells activation/

Fibroblasts activation
damage

End stage pathology

Obliterative vasculopathy fibrosis
 LABORATORY FINDINGS

• 1.ANA, RF
• 2.anti-Scl-70 (DNA topoisomerase I) antibody
1) 20-40% in difuse scleroderma
2) 10-15% in limited scleroderma
• 3. anticentrome antibody
1) 50-90% in limited scleroderma
2) 5% in diffuse scleroderma
 DIAGNOSIS

• 1. major criteria : proximal scleroderma

• 2. minor criteria:
1) sclerodactyly
2) digital pitting scar or loss of substance from the finger
pads
3) bibasilar pulmonary fibrosis

* one major or 2 оr more minor criteria for diagnosis

 MEDICATION

• There is no medication that can cure or stop the

overproduction of collagen that is characteristic of
scleroderma. But a variety of medications can help
control scleroderma symptoms and prevent
complications.
 MEDICATION

• Immunosuppressive drug – reduce immune response

(Mycophenolate mofetil –not lower than 3g per day,
cyclophosphamide -50mg/kg daily for 4 constitutive
days, azathioprine, cyclosporine, and Thymoglobulin
(anti-thymocyte globulin)).
• Calcium channel blocker – relaxes blood vessels
(nifedipine -10 to 30 mg 3 times a day)
• Steroid – (Prednisone -5mg a day for 6 months)
• Proton pump inhibitors – decrease acid release in
stomach
 RAYNAUD’S PHENOMENON

• Raynaud's phenomenon and ischemia

• 1) avoid cold exposure
layers of warm, loose-fitting clothing
• 2) quit smoking
• 3) vasodilator therapy
calcium channel blocker (nifedipine), prazosin -1 to
3mg per day, ACE Inh.
• 4) finger / toe necrosis
-intravenous prostaglandin (PGE, PGI)
-amputation
 GASTROINTESTINAL

• 1)Reflux esophagitis and dysphagia

-H2 Blocker, proton-pump inhibitor
2) gastroparesis: promotility agent ( metoclopramide)
3) malabsorption syndrome: broad spectrum antibiotics
 PULMONARY

• 1) Interstitial fibrosis
- corticosteroid
- clophosphamide, azathioprine
• 2) pulmonary artery hypertension
-calciumm channel blocker
- prostacyclin
- transplantation
 RENAL

• 1) Renal crisis
- early detection and ACE inhibitor (25 to 150 mg 3
times a day)
1 year survival without captopril 15%
1 year survival with captopril 76%

- dialysis
 PROGNOSIS

• 1. Quite variable and difficult to predict

• 2. cumulative survival
• diffuse limited
5 yr 70% 90%
10 yr 50% 70%
• 3. major cause of death
1) genetic involvement
2) cardiac involvement
3) pulmonary involvement