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Scleroderma def., pathology, manifestations, diagnosis, treatment (Systemic sclerosis)

This article is to discuss Scleroderma (Systemic sclerosis) regarding its definition, pathology, pathogenesis, clinical picture ( symptoms and signs )  , diagnostic investigations and treatment approaches .
Definition
• Systemic sclerosis is a connective tissue disease characterized by degenerative changes and fibrosis of the integument (skin) associated with vessel oblitrative disease and internal organ Lesions in:
. • Heart. • Lung.
• Kidney. • GIT.
So it. is a multi-system disease to be differentiated from localized scleroderma syndromes e.g. Morphoea that do not involve internal organ.

Pathology and pathogenesis

1- Vascular lesions:-
• Endothelial damage with release of endothelin causing vasoconstriction.
• Also there is release of cytokines (IL 1,4,6,8 ), transforming growth factor (TGF), and platelets derived growth factor (PDGF) with activation of fibrblasts, this leads to damage of blood vessels and wide spread obliterative arterial lesions with subsequent chronic ischaemia
2- Fibrotic features:
• The activated fibroblasts synthesize increased quantities of collagen types I and III and fibronectin producing fibrosis of the dermis of the skin and internal organs.

Clinical picture of Scleroderma (Systemic sclerosis)

• Mainly in female ( 4 : 1 )
• 4th and 5th decades.
scleroderma-face• Rarely visceral scleroderma occurs in absence of the skin changes.
Skin changes:
     • Initially the skin is oedematous (non pitting edema) and then becomes tight.
     The skin tightening begins on the fingers and hands then the proximal parts of the extremities, in addition        to the thorax and abdomen with restriction of movement of the fingers.
     • Later skin becomes shiny with atrophy and ulceration, areas of hyperpigmentation or hypopigmentation      are common, calcinosis may occur.
     • Raynaud's phenomenon: It is vasospasm of peripheral arteries causing color changes (pallor, cyanosis          and erythema). It occurs in most patients with systemic sclerosis.
Musculoskeletal
     • Non erosive arthritis or arthralgia.
     • Myopathy ( = sclerodermatomyostitis )
GIT
      1. Dysphagia (esophageal hypomotility)
      2. Reflux, (due to loss of lower esophageal tone).
      3. Malabsorption syndrome (due to over bacterial growth).
      4. Colonic involvement with constipation, intestinal pseudo obstruction may occur.
      5. Primary biliary cirrhosis.
Heart
     • Cardiomyopathy with heart failure.
     • Pericarditis, pericardial effusion.
     • Coronary heart disease.
Lung
     • Interstitial pulmonary fibrosis.
     • Pulmonary hypertension.
     • Aspiration pneumonia (due to oesophageal dysfunction).
Kidney
     • Scleroderma kidney (thickness of intima of the interlobular arteries)leading to :-
      - Malignant hypertension. • Renal failure.
      - Scleroderma renal crisis i.e abrut onset of accelerated hypertension, oliguria and microangiopathic hemolysis.

Diffuse skin disease, pulmonary hypertension and renal involvement are associated with poor prognosis .

Investigations
1- ANA +ve in 90% of patients.
2- Auto Ab against sclero nuclear protein called anti-topoisomerase
(Anti-Scl 70) in 20-30% of patients (it is specific).
3- Rheumatoid factor +ve in 30 %.
4- Microangiopathic haemolytic anemia.
5- Skin biopsy :  collagen in dermis.
6-Ba swallow for impaired oesophageal motility.
7- X-ray hand showing calcification around fingers, late erosion and resorption of the tuft of the distal phalanges .
scleroderma-clinical-serologic-classification

Treatment of Scleroderma (Systemic sclerosis) 

no specific satisfactory drug therapy
Penicillimine (antifibrotic drug) causing minimal skin softening.
• Raynaud's may be treated by hand warmers, vasodilators e.g calcium channel blockers e.g verapamil, deltiazem and nifedipine as well as nitroglycerin ointment.
Steroids are indicated for treatment of inflammatory myositis, pericarditis or pulmonary fibrosis.
• Oesophageal symptoms can be treated by prokinetic drugs e.g domperidone.
ACE inhibitors for treatment of hypertension and prevention of renal failure. Also they are used in renal crisis ..
• Bacterial over growth due to intestinal stasis respond to broad spectrum antibiotic.
• Steroids and cyclophosphamide may be of value in treating alveolitis and pulmonary fibrosis.
• Endothelin receptor antagonist (bosentan) can be used for severe pulmonary hypertension and digital ulceration.
• Sympathectomy to prevent auto amputation.
• Other novel agents currently under study include mycophenolate and tacrolimus.
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Tamer Mobarak

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