Systemic sclerosis

The connective or connective tissues are those whose primary function is to provide support and systemic integration to the body. These tissues make it possible to differentiate structures and areas within organs, separate them from each other, achieve a cohesion that allows the different components of the organism not to be piled on top of each other without any form and they are the means through which vessels and nerves circulate. .

There are various types of connective tissues, some specialized, such as bone tissue or cartilage, and others less specialized, such as adipose tissue or serous that line the muscles, organs and joints. If a connective tissue disorder so that this is produced in excess and therefore constricts vessels, organs, skin and various tissues, they suffer fibrosis, which alters both their shape and their functionality. When this occurs in specific tissues and due to vascular, immunological and collagen alterations, it is said that the patient suffers from systemic sclerosis.

How is it produced?

Systemic sclerosis is caused by alterations at the level of the vessels, which become clogged and become more fragile, at the level of the fibroblasts, which produce collagen in excess and form plaques that affect the organs and tissues that support them, and at the level immunologicalSince antibodies have been detected that attack body tissues that they do not recognize as their own, such as ANA antibodies, the so-called Scl-70 antibodies or anti-centromere antibodies; It is because of these antibodies that systemic sclerosis is considered a

Within systemic sclerosis there are two different forms:

• Diffuse systemic sclerosis, with a rapidly evolving skin involvement that begins distally and progresses to the most proximal areas. It is associated with gastrointestinal, lung, kidney, heart and joint disorders.
• Limited systemic sclerosis, of slow progression and exclusive involvement of the fingers and the back of the hands and forearms. It is associated with and with the passage of time there is also pulmonary and gastrointestinal involvement. Sometimes it appears in the context of the so-called CREST syndrome.

It should be said that there is a form of sclerosis whose affectation is merely cutaneous, the so-called localized scleroderma. Its mechanism of action is similar but only affects the skin, subcutaneous tissue, muscle and sometimes the periosteum. It usually manifests itself in childhood and very rarely affects other organs.

Symptoms

This disease affects many organs and systems, hence its name systemic sclerosis. By devices, the different manifestations of this entity can be:

Cutaneous

• Distal finger sclerosis
• Edematous fingers
• Raynaud's syndrome
• Loss of skin folds, which limits mobility and reduces facial expression, characteristic of these patients
• Ulcerations on the tips of the fingers, classically called "rat bite" scars
• Pigmentation changes
• Soft tissue telangiectasia and calcifications

Musculoskeletal

• Joint pain and inflammation due to inflammation of the synovium
• muscle and tendon involvement

Digestive

• Impaired mobility of the esophagus and lower sphincter, leading to gastroesophageal reflux
• Slowed gastric and duodenal motility
• due to fibrosis
• Biliary cirrhosis, in less than a fifth of patients is associated with

Pulmonary

• Interstitial involvement, especially in the lower part of the lungs, with progressive respiratory distress that is the main cause of death in these patients.
• Pleural inflammation with possible effusion
• Alveolar fibrosis with consequent pulmonary hypertension

Cardiac

• Pericarditis and fibrosis of the myocardium, making it difficult to fill and empty the heart
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• Arrhythmias and conduction disturbances

Renal

• Kidney failure, one of the main causes of death in these patients
• malignant hypertension

Neurological

• Polyneuropathies
• Trigeminal nerve neuralgia
• Paresthesia

Sometimes systemic sclerosis is associated with other pathologies, such as Hashimoto's thyroiditis or primary biliary cirrhosis.

Diagnosis

The diagnosis of systemic sclerosis will be based eminently on the patient clinic.

A blood test will show an elevated erythrocyte sedimentation rate (ESR). We should specifically look for antibodies that may be elevated in this pathology, such as Scl-70, anticentromeres or ANAs.

The capillaroscopy is a technique that allows assessing the involvement of the capillaries of the nail bed, which in the case of systemic sclerosis show a characteristic pattern of dilated capillary loops with or without loss and destruction of them depending on the type of involvement, diffuse or limited respectively.

With all this, it can be said that the existence of proximal scleroderma in the fingers of the hands is sufficient to diagnose systemic sclerosis. Likewise, there are some minor criteria, which are bilateral inferior pulmonary fibrosis, sclerodactyly or the presence of “rat bite” scars on the fingers; If two of these three criteria concur, the diagnosis of systemic sclerosis can also be given.

Treatment

The treatment of systemic sclerosis will be based mainly on the treatment of the symptoms, on drugs that can inhibit collagen synthesis, in avoiding external factors that can aggravate symptoms, such as cold or tobacco, and in promoting moderate physical exercise.

Drugs like penicillamine, colchicine, or gamma interferon they can inhibit collagen synthesis and slow the progression of the disease. To avoid Raynaud's clinic, exposure to cold should be avoided and it can be treated with prostaglandins or calcium antagonist vasodilators.

The corticosteroids They will be given to treat synovitis, edema, and alveolar inflammation. In case of pulmonary fibrosis, treatment with corticosteroids and cyclophosphamide will be given.

Esophagitis and gastroesophageal reflux will be treated with dietary measures and antacid drugs.

Arterial hypertension, as well as kidney involvement, will be tried to control with ACE inhibitors type antihypertensiveshe treatment of systemic sclerosis will be based above all on the treatment of symptoms, on drugs that can inhibit collagen synthesis, on avoiding external factors that can aggravate symptoms, such as cold or tobacco, and on promoting physical exercise moderate.

Precautionary measures

Systemic sclerosis is a disease of autoimmune origin, so there are no preventive measures to avoid it.

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