Progressive Systemic Scleroderma

Progressive systemic scleroderma (PSS) is a chronic inflammatory disease of the connective tissue and the vascular system that is caused by a dysregulation of the body’s immune system. Progressive systemic scleroderma affects women more often than men.

Progressive Systemic Scleroderma

What is progressive systemic scleroderma?

Progressive systemic scleroderma (PPS) is a chronic inflammation of the connective tissue and the vascular system of the skin and internal organs, which is due to a disturbed immune system (autoimmune disease).

Due to a dysregulation of the immune system, it attacks the body’s own collagenous connective tissue (collagenosis) in progressive systemic scleroderma and causes inflammatory reactions that lead to a loss of elasticity in the skin, and later in the organs as well, and to hardening (sclerotherapy).┬áSee gradphysics for Hepatitis E in English.

This leads to edema in the fingers and hands as well as sclerodactyly, the mask face characteristic of progressive systemic scleroderma due to loss of facial expressions, swallowing disorders due to a shortening of the frenulum and inflamed joints (arthralgia).

Causes

The symptoms typical of progressive systemic scleroderma are caused by a disturbance in the immune system, whereby the immune system attacks endogenous structures, in particular the collagenous connective tissue and vascular system of the skin and internal organs, and causes inflammatory reactions.

In addition, with progressive systemic scleroderma, there is a dysregulation of certain cells in the connective tissue (fibroblasts), which means that they synthesize more collagen, which accumulates in the skin and causes the skin and, later on, the connective tissue and skin cells that are characteristic of progressive systemic scleroderma vascular changes in the internal organs.

The triggers for this dysregulation are not yet known. In addition to genetic factors, viral and bacterial antigens as well as certain drugs, tumors, UV light and sex hormones are discussed as triggers for progressive systemic scleroderma.

Symptoms, Ailments & Signs

The symptoms of progressive systemic scleroderma can be divided into three stages. The first thing to notice is skin changes and thickening on the hands and feet. The initial stage of progressive systemic scleroderma is also characterized by edema of the hands.

The connective tissue hardens increasingly. It degrades. Raynaud’s syndrome is also typical. This is characterized by whitish-bluish hands with poor circulation. Sometimes at this stage there is necrosis of the stretched skin areas burdened by hardened scar tissue.

The facial expressions are progressively reduced. The progressive skin tension ensures mask-like facial features. Since the lip region can also be affected by increasing tissue hardening, radial wrinkles often form around the mouth opening. The mouth gets smaller and smaller. Medically it is called a microstomy.

Small blood vessels on the face often dilate into telangiectasias. The skin pigmentation can also be noticeably altered. In the second stage, the inflamed and edema-laden joints can no longer be freely and painlessly moved due to the increasing tightening of the skin. Progressive systemic scleroderma causes the fingers or toes to curve.

The third stage of progressive systemic scleroderma affects internal organs. These are also increasingly affected by hardening. This leads to hardened digestive organs, pulmonary fibrosis and difficulty swallowing due to sclerotic changes in the esophagus and mouth.

Diagnosis & History

Progressive systemic scleroderma is diagnosed based on the presenting symptoms, especially the skin changes. The diagnosis is backed up by tissue removal and analysis (biopsy) from the affected skin areas as well as a blood test.

In the presence of progressive systemic scleroderma, the concentration of certain immunological proteins, the so-called antinuclear antigens, and the ESR (blood sedimentation rate) are increased.

Furthermore, the stage-dependent vascular changes characteristic of progressive systemic scleroderma can be detected by capillary microscopy at the nail bed. A pressure measurement in the esophagus (gullet) can be used to diagnose possible dysphagia. In addition, a cardiac sonography (ultrasound) and an analysis of the lung function provide information about organ involvement.

Progressive systemic scleroderma can have two different forms. The so-called acral scleroderma has a locally limited course, in which only smaller areas of skin, especially on the feet and hands (acren), are affected. The prognosis is usually good if there is no lung involvement.

In the diffuse form of progressive systemic scleroderma, the internal organs (kidneys, lungs, heart) are usually involved, which means that the prognosis and course are poor (unfavorable) depending on the extent of involvement.

Complications

Most complications of a diagnosis such as progressive systemic scleroderma are due to internal organ involvement. In this form of scleroderma, not only the skin is affected by hardening. The immune system is also involved in this rare autoimmune disease due to overreactions. It is all the more important that those affected receive permanent treatment in order to avoid complications.

Nicotine consumption can further aggravate the symptoms of progressive systemic scleroderma. In smokers, the already constricted blood vessels narrow even more. The increased tendency to inflammation is increased. In this severe form of scleroderma, the constriction of the vessels must also be reduced with measures against the effects of cold. With inadequate nutrition, hydration and skin care, the skin of those affected dries out. The disease can lead to swallowing disorders, circulatory disorders in the fingers or a masked face.

Inflammatory flare-ups, intestinal problems and immune deficiencies often occur in those affected. Complications from scleroderma can also occur in the musculoskeletal system. Without lymphatic drainage or physiotherapy, the joints become increasingly stiff. Moderate sports such as swimming are also advisable.

In the third stage of the disease, pulmonary fibrosis can occur. Other organs can also become inoperable due to increasing tissue hardening. Since life expectancy is reduced in progressive systemic scleroderma, treatment is correspondingly intensive.

When should you go to the doctor?

Changes in skin texture, swelling, discoloration, or growths should be seen by a doctor. If edema, thickening of the skin layers or peculiarities of the limbs develop, a visit to a doctor is advisable.

Increasing complaints or persistent irregularities must be examined and treated. If there are abnormalities in facial expressions, there is cause for concern. If mask-like facial features or a restriction of facial expressions are noticed, a doctor’s visit is necessary. Hardening of the lips is characteristic of progressive systemic scleroderma. The complaints must be clarified so that a treatment plan can be drawn up. In addition, the shape of the mouth and in particular the opening of the mouth in sick people becomes smaller and smaller over time.

If visible blood vessels develop on the face, restrictions on swallowing or visual changes in the oral cavity, a doctor should be consulted. Esophageal abnormalities and a decrease in food intake should also be examined. General functional disorders, hardening inside the body or a feeling of tightness should be presented to a doctor.

The diseases lead to changes in the organs that must be treated in order to avoid complications or life-threatening conditions. Circulatory disorders, a feeling of illness or general malaise should be clarified by a doctor. If performance drops or if immune system disorders are noticed, a doctor should be consulted.

Treatment & Therapy

In the case of progressive systemic scleroderma, the therapeutic measures are aimed at reducing and eliminating the symptomatic complaints and slowing down the progressive course.

Anti- inflammatories such as NSAIDs (nonsteroidal anti-inflammatory drugs such as diclofenac) and glucocorticoids are used, particularly in flare-ups. Immunosuppressants (methotrexate, cyclophosphamide) are also used to minimize and inhibit the dysregulation of the immune system that is present in progressive systemic scleroderma.

Accompanying physiotherapeutic, occupational therapy, heat therapy and physical measures to maintain joint mobility as well as careful skin care with moisturizing ointments and lymphatic drainage are recommended. In the presence of circulatory disorders, vasodilating substances (prostaglandins) and acetylsalicylic acid can also be used, while in the case of progressive systemic scleroderma with kidney involvement, antihypertensive drugs (ACE inhibitors) can also be used.

In some cases of progressive systemic scleroderma, surgery may be needed to repair skin defects or joint stiffness. Active ingredients such as interferon, which inhibits the formation of new connective tissue, and tacrolimus, which suppresses an enzyme involved in the immune system (calcineurin), are currently being tested. Positive experiences have also been made with infused iloprost in progressive systemic scleroderma.

Prevention

Since the triggers for the dysregulation of the immune system in progressive systemic scleroderma are not known, the disease cannot be prevented. However, the symptoms can be alleviated by taking a few simple measures. For example, saliva-stimulating candies (peppermints, chewing gum) and more frequent but smaller meals can alleviate swallowing difficulties and prevent dry mouth. In order to avoid the cold, it is recommended in progressive systemic scleroderma to shift annual vacation to the winter months and to warm regions.

Aftercare

Progressive systemic scleroderma is a subgroup of scleroderma, which is an incurable autoimmune disease. Therefore, direct follow-up care is not possible in this form and a more permanent treatment of the mostly chronically worsening disease is necessary. After a diagnosis and acute treatment, long-term treatment must follow, as well as reducing, delaying or preventing possible late effects over the course of the disease.

These consequences can ultimately lead to the death of the patient in severe forms of the disease. As a rule, the affected person is dependent on a variety of medications for life and regular consultations with specialist physicians are essential. The concrete course and a direct prognosis are not foreseeable, there are very different forms here.

Finally, when it comes to aftercare, palliative care should also be mentioned when it becomes clear that the disease will inevitably lead to death within a certain period of time. The patient’s quality of life can be increased or maintained at a very good level for as long as possible. One should not be afraid to contact trained physicians and nurses in palliative care in good time and to take the wishes of the person concerned into account accordingly.

You can do that yourself

For those affected, it is particularly important that they receive permanent medical care. Discontinuing or changing your medication from prescribed drugs on your own responsibility can lead to complications or an increase in the existing symptoms.

Since the consumption of nicotine leads to a deterioration in health and an increase in existing discrepancies, it should be avoided completely. Likewise, areas where people smoke should be avoided. The patient’s organism reacts even if the nicotine is only absorbed passively.

Although the disease is triggered by a disorder of the immune system, the body’s defenses should be sufficiently strengthened. A healthy lifestyle and a balanced diet contribute to improving well-being. Obesity should be avoided and adequate exercise should take place.

The progressive systemic scleroderma is accompanied by optical changes. In order to avoid mental or emotional problems, self-esteem needs to be strengthened. Otherwise, it can lead to the development of a mental illness. As a balance, attention should be paid to a stable social environment and leisure activities should be aimed at strengthening the zest for life.

Yoga and meditation techniques can be helpful in reducing stressors . These can be carried out independently by the person concerned at any time.