Scleroderma

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Scleroderma

• By..Manohar Joshi
• Medicine Resident

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Scleroderma

• Also called as systemic sclerosis.
• Occurs due to fibroblast activation.
• There is excessive fibrosis.
• Its cause is unknown , but it occurs due to
  abnormal activation of immune system and
  microvascular injury.
• Along with skin , GIT , lung , heart , kidney get
  involved.

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CLASSIFICATION

• Systemic Sclerosis
• Diffuse scleroderma
• Limited scleroderma(CREST syndrome)
• Sine scleroderma(visceral organ involvement)
• Localized scleroderma
• Morphea(single/multiple plaques of skin
  indurations)
• Linear scleroderma(only face and extremity)
• En coup de sabre
• Others
• Overlap syndrome
• Mixed connective tissue disorder
• Eosinophilic fascitis
• Eosinophilia myalgia syndrome

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Diffuse Limited
Skin involvement Distal proximal extremities, face, trunk Distal to elbow, face
Raynauds phenomenon Onset within 1 year or at time of skin changes May precede skin diseases
Organ Pulmonary (interstitial fibrosis) Renal (hypertensive crises) Gastro and heart GIT involvement, pulmonary hypertension after 10-15 years
Nail folds and capillaries Dilatation and dropout Dilatation without dropout
ANA Anti topoisomerase 1 Anti centromere
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Etiology

• More common in females 3 1
• 3rd to 5th decade.
• More common in monozygotic twins.
• CMV is mediated through direct vascular injury
• Parvo virus B 19.
• Drugs vinyl chloride,
• pentazocine(non-narcotic
  agent)
• bleomycin(anti cancer)

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Pathology

• Two types of response
• Vascular
• Immune

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Vascular response

• It involves raynauds phenomenon.
• Leads to capillary vasospasm of extremites.
• Cold (sudden vasospasm)
• Blue (decreased blood supply)
• Red (reactive vasodilatation)

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Immune response
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Pathology of organs involved

• Skin thin epidermis over compact
  bundle of collagen
• GIT Lower 2/3rd of the oesophagus is
  involved. Thin mucosa and increased collagen in
  lamina propria , submucosa , and serosa.
• Lung diffuse interstitial fibrosis ,
  thickening of alveolar membrane, peribronchial
  and pleural fibrosis.
• Musculoskeletal arthritis, lymphocytic
  infiltration, degeneration of muscle fibers ,
  arterioles thickened

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• Heart Degeneration of myocardial
  fibers, A V conduction defects with arrythmias.
• Kidney intimal hyperplasia of
  interlobular arteries , fibrinoid necrosis ,
  thickening of glomerular basement membrane.
• Other primary biliary cirrosis and
  auto immune thyroiditis.

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Clinical manifestations

• Face mask like facies.
• Pinched up nose
• fish mouth (not able to open
  the mouth) , shiny skin.
• Hands Skin is very taut and cant be pinched up.
• Symmetrical swelling of
  fingers.
• pulp atrophy
• telengactesia
• nail fold thrombi, digital
  infarct and ulcerations

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• GIT Heart burns , reflux oesophagitis
• Dysphagia
• Mal absorption
• Chronic constipation.
• Musculoskeletal Poly arthritis, Leathery
  crepitations , Carpal tunnel syndrome.
• Lung Dyspnea and dry cough , Bilateral basilar
  rales , Interstitial lung disease , PH.
• Heart Pericarditis , heart blocks
• Arrhythmias and LVF.
• Renal Malignant HT and Progressive Renal
  Failure

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• Other Dry mouth or eyes
• Hypothyroidism
• Sjogrens syndrome
• Biliary cirrhosis.

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Laboratory Findings

• Anemia .
• Vitamin B12 deficiency.
• Cryoglobulin present in serum.
• ANA shows anti topoisomerase 1
• Nail fold capillary scopy.
• Sereal PFT
• Oesophageal manomatory

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Treatment

• Raynauds phenomenon
• 1) methyldopa , reserine,
• phenoxybenzamine , prazosin
• 2) calcium channel blocker like
  nifedepine , nifedepine sustain release ,
  diltiazem
• 3) ketaserin 40mg TDS
• 4) fluroxetine 20mg/day
• 5) pentoxyfyline40mg TDS
• improve reperfusion

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• D-penicillamine low dose is required
• 125mg
• Methotrexate show improvement in skin lesion
• Cyclophosphamide has also shown some improvement
  skin lesion and systemic manifestation
• Stem cell transplantation
• Anti platelet
• Dipyridamole 200 to 400mg,it decrease platelet
  adhesion to vessels
• Colchicine , INF-a, INF- ?, recombinant human
  relaxin
• -

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• Proton pump inhibitor - like ranitidine,
  cimitidine can be used in reflux oesophagitis /
  heart burn.
• Pilocarpine - as it increases secretion in
  condition like dry mouth and dry eye.
• Cervical and digital sympathectomy can be helpful
  in vascular injury.