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Systemic scleroderma: definition, treatment

Scleroderma is inflammatory diseases that cause sclerotic thickening of the skin. There are two major forms: localized scleroderma, also called “morphea”, which affect the skin and sometimes in deep forms the underlying musculo-aponeurotic and skeletal planes and systemic scleroderma that affect the skin and organs.

Definition of systemic scleroderma

Systemic scleroderma is a rare disease affecting 3 women in a man, most often between the ages of 50 and 60, which causes tissue fibrosis of the skin and certain organs, particularly the gastrointestinal tract, lung, kidney and kidney. Heart. Achieving these last 3 organs often causes serious complications.

Its evolution is spread over many years, marked by outbreaks.

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Raynaud’s syndrome

Raynaud’s syndrome is characterized by a bleaching of some cold fingers. It is almost always the first sign of scleroderma, especially when it is bilateral, appearing before other signs from a few weeks to a few years (the shorter the delay, the worse the prognosis) and it exists in 95% of cases scleroderma.

The doctor performs a nail capillaroscopy (examination with a powerful magnifying glass of the cuticle vessels and the nail skin fold) showing in favor of scleroderma:

  • a rarefaction of the capillary loops,
  • megacapillaries
  • sometimes the existence of pericapillary edema
  • cuticular hyperkeratosis,
  • erythema,
  • Microhemorrhages visible to the naked eye.

Cutaneous sclerosis

Fingers

The fingers are first inflated and puddled with a tendency to disappear fingerprints. Then the skin becomes tense, indurated giving a “sucked” appearance of the digital pulp

Then the fingers gradually taper and retract in bending.

Complication of sclerosis, painful ulcerated sores occur on pulp

Other areas

Sclerosis can spread to the face (the face is smooth and freezes, there is a tape of

Nose and a decrease in the opening of the mouth which is surrounded by radiated folds in “purse gusset”), limbs and trunk giving a smooth and sheathed appearance of the shoulders, trunk and limbs.

Telangiectasia

These are small purplish vessels that gather into purple spots of one to two millimeters, which develop on the face and on the extremities.

Calcinoses

It is hard nodules, white when they are superficial that can when they reach the skin, let out a chalky porridge. They are more common on the hands and legs.

Mucosal involvement

The oral mucosa is often dry as well as the eyes. This is called dry syndrome.

Organ sclerosis

The digestive tract

The involvement of the esophagus is present in 75% of cases, manifested by gastroesophageal reflux, difficulty swallowing, or even esophageal ulcerations.

The small intestine is also affected by fibrosis or even villous atrophy sometimes responsible for malabsorption syndrome, accentuated by a slowing of intestinal peristalsis, causing microbial proliferation and exposing the risk of intestinal pseudo-occlusions.

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The lungs and the heart

Interstitial pulmonary fibrosis occurs in 25% of patients, causing respiratory problems that can lead to chronic respiratory failure, which is the leading cause of death in patients with CF.

The second leading cause of death is pulmonary arterial hypertension, due to pulmonary fibrosis, pulmonary artery involvement or cardiac involvement. The latter is related to myocardial ischemia, the “myocardial Raynaud phenomenon” and fibrosis.

Kidneys

Renal impairment results in malignant hypertension and kidney failure

Locomotor device

There is damage to the joints (arthritis), tendons, bones (demineralization, destruction of distal bones) and muscles (muscle pain and fragility).

Treatment of systemic scleroderma

Fibrosis control

Surveillance is essential and the treatments that can be tried are numerous because their effectiveness varies a lot from one person to another. Among the treatments used, mention may be made of colchicine, D-penicillamine, interferon-γ, cortisone, ciclosporin, etc.

Regular exercise, massage and rehabilitation try to maintain mobility and combat muscle atrophy.

Raynaud’s syndrome

In addition to protection against cold and smoking cessation, vasodilators such as calcium channel blockers are used: dihydropyridines (nifedipine, amlodipine, etc.) or benzothiazines (diltiazem). In case of ineffectiveness of calcium channel blockers, the doctor prescribes other vasodilators: prazosin, converting enzyme inhibitors, sartans, trinitrine, iloprost …

Telangiectasia

They can be attenuated by pulsed dye vascular laser or KTP.

Subcutaneous calcinosis

The doctor prescribes dressings, even colchicine. Surgical excision of calcinosis is sometimes necessary.

 

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Treatment of manifestations of other organs

Digestive tract

It is necessary to respect the hygienic and dietary measures of gastroesophageal reflux: elimination of acidic foods and alcohol, taking meals in a sitting position, use of several pillows to sleep. The doctor prescribes proton pump inhibitors to limit gastric acidity.

In case of malabsorption, linked to a microbial proliferation favored by the slowing down of the intestinal peristalsis, the doctor prescribes antibiotics intermittently and cyclically one to two weeks every month (ampicillin, tetracyclines or trimethoprim-sulfamethoxazole), combined with a supplementation with iron, folic acid and vitamin B12.

Lungs and heart

For pulmonary interstitial fibrosis, cyclophosphamide alone or in combination with cortisone is used. Pulmonary superinfections are treated with antibiotics and the risk of worsening pulmonary fibrosis is limited by vaccination against influenza and pneumococcus.

Against pulmonary arterial hypertension, vasodilators such as nifedipine are used. Iloprost and esoprostenol.

For myocardial irrigation, calcium channel blockers and angiotensin converting enzyme inhibitors are used.

Waist

Inhibitors of the conversion enzyme such as captopril or vasodilators such as sartans limit arterial hypertension and associated renal failure.

Muscular and articular disorders

The doctor prescribed for joint pain of non-steroidal or steroidal anti-inflammatory drugs (cortisone)

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