Systemic Scleroderma

The systemic scleroderma progressive, usually called " sclérodermie" , and in a more current way " sclerose systémique" is a disseminated affection of conjunctive fabric, entering the group of the Connectivites. It is characterized by a cutaneous and vascular fibrosis.

Epidemiology

It is about a relatively rare disease, whose incidence varies between 5 and 20 new cases per annum and per million inhabitants.

The etiology and physiopathology are imperfectly known. The family and autoimmune determinants are not convainquants and the environmental factors are sometimes possible.

Clinical data

The phenomenon of Raynaud

A Syndrome of Raynaud is observed in 90% of the cases and generally it is about the inaugural symptom. That 5 to 10% of the patients presenting a syndrome of complete Raynaud, apparently isolated, will develop a Connectivite, a scleroderma is generally estimated.

The cutaneous attack

The attack so characteristic of the skin is the element-key of the diagnosis.
  • the sclérodactylie generally associated with the syndrome of Raynaud, leads to frayed, hard and stiff fingers, without any elasticity. The attack gradually gains the hand and the wrist. The sclérodactylie with syndrome of Raynaud can join calcifications under cutaneous, of the télangectasies and an attack oesophagienne to form the Syndrome from CREST.
  • the attack of the face starts around the mouth and the face, erasing the wrinkles and giving an aspect of hard-bound skin, even marmoreal.
  • In diffuse scleroderma, the infiltration reaches the root of the members and the trunk. This form is later, the forecast is less good. The skin is luisante, impossible to grip, and the patient can be enclosed in a true armor.

The locomotor system

  • Reached ostéo-articular: Inflammatory polyarthritises, tendinitises, osteolysis of the ends.
  • Muscular: Muscular weakness of the belts, being able to be accompanied by enzymatic and electric anomalies of attack myogene.
  • Neurological: Mononévrites, of the trigeminal nerve or the carpel tunnel, even polyneuropathies.

Visceral attacks

  • Digestive
The attack oesophagienne relates to 75% of the patients, in the more or less long term. A progressive paralysis of the lower part of the esophagus causes a Dysphagie. There exists a backward flow causing a Oesophagite sometimes ulcerated. There can more rarely be of the attacks of the same type on the level of the stomach and the small intestine, being able to involve a syndrome of Malabsorption. One also described digestive télangiectasies, sources of hemorrhages.
  • Pulmonary
Scleroderma can reach the lung with evolution of a interstitial Fibrose, more or less frequently according to the series published and the mode of diagnosis. It can also exist a pulmonary arterial hypertension, isolated or not.
  • Cardiac
Their frequency is very variable according to the type of series published, clinical, echocardiographic or anatomical. There is no endocardic attack. The pericardial demonstrations are traditional, but it is especially the myocardic attack which is frequent and severe. To note the possibility of coronary, true spasms " Raynaud coronaires".
  • Renal
The " renal crisis sclérodermique" carry out a vital urgency with acute Impaired renal function and Hémolyse. Most of the time it is however about a chronic Impaired renal function slowly progressive with arterial Hypertension.

Complementary examinations

  • There often exists in systemic scleroderma a biological inflammatory syndrome seldom intense.
  • the antinuclear antibodies are positive with presence of antibody anticentromères and of anti-DNA topoisomérase I.
  • the capillaroscopy is an important component of the early diagnosis.
  • the cutaneous biopsy necessary to the diagnosis, and is not even disadvised by certain teams because of possible difficulties of cicatrization.

Evolution and forecast

The forecast of systemic scleroderma is variable, function of the complications, in particular pulmonary, renal and digestive.

Forms " frontières"

  • oedemateuse Form
The disease can start with a long time isolated oedemas which make wander the diagnosis. One should not confuse systemic scleroderma with the Syndrome of Shulman, or fasciite with eosinophilic.

Associated forms

Systemic scleroderma can be associated with a great number of diseases. We quote most frequent:

Treatment

  • basic Treatments.
The majority proved to be disappointing. One can quote the corticothérapie, D-pénicillamine, factor XIII, the Interféron.

It would seem that a treatment prolonged by the traditional immunosuppresseurs can prove to be useful to slow down the evolution of the disease.

  • symptomatic Treatments.
They are likely to be adapted to each case, according to the visceral attacks and their complications.

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