What happens in the body
The disease is based on three processes: damage to small vessels, disruption of the immune system and excessive formation of connective tissue. The vessels narrow and gradually become overgrown, causing the tissues to suffer from a lack of blood supply. At the same time, cells begin to produce too much collagen, and the skin, and then internal organs, thicken and lose elasticity. Based on the prevalence of skin changes, there are a limited form, in which the hands, forearms and face are affected, and a diffuse form, with a more rapid spread and a greater risk of organ damage.
- Limited form - hands, forearms, face
- Diffuse form - rapid spread to the body
- Scleroderma without skin changes is a rare option
- Localized forms of skin without organ involvement
- Cross syndromes with other rheumatic diseases
Symptoms
The first symptom for most is Raynaud's syndrome, sometimes years before other manifestations. Then swelling of the fingers appears, they look sausage-shaped, and over time the skin becomes dense, shiny, and cannot be folded. Radial wrinkles form around the mouth, mouth opening decreases, and the nose becomes sharper. Painful ulcers appear on the fingers and calcium is deposited. From the organs, heartburn and difficulty swallowing, dry cough and shortness of breath, palpitations, joint pain and muscle weakness appear.
- Raynaud's syndrome with white and blue fingers
- Swelling and hardening of the skin of the fingers
- Limitation of hand mobility and mouth opening
- Sores on fingertips
- Heartburn, difficulty swallowing
- Dry cough and shortness of breath on exertion
- Joint pain, muscle weakness
Diagnostics
The key role is played by a combination of symptoms: Raynaud's syndrome, skin thickening and specific antibodies in the blood. Antinuclear antibodies are determined, and then clarifying ones - to the centromere, to topoisomerase and to RNA polymerase, since they are associated with different variants of the course. Capillaroscopy of the nail bed is very informative, showing characteristic changes in the capillaries. Internal organs must be assessed: spirometry with assessment of diffusion capacity and computed tomography of the lungs, ECG and echocardiography, endoscopy of the esophagus, kidney function tests and regular blood pressure monitoring.
- Antinuclear and specific antibodies
- Capillaroscopy of the nail bed
- Spirometry and assessment of lung diffusion capacity
- Computed tomography of the lungs
- ECG and echocardiography
- Endoscopy of the esophagus
- Creatinine, urine test, blood pressure control
Treatment
There is no single pill for the disease; treatment is selected according to the leading manifestations. For vascular disorders, drugs that dilate blood vessels are used; for finger ulcers, special groups of drugs and wound care are used. If the lungs are affected and skin changes quickly spread, basic immunosuppressive drugs are prescribed, and in some cases genetically engineered ones. For reflux, proton pump inhibitors and diet are used; for kidney damage, drugs of a certain group that lower blood pressure are used, which are prescribed immediately. Glucocorticoids are used sparingly and in low doses.
- Vasodilators for Raynaud's syndrome
- Immunosuppressive therapy for lung and skin lesions
- Proton pump inhibitors for reflux
- Immediate treatment of renal crisis
- Care for finger ulcers
- Caution with glucocorticoids
- Physical therapy to maintain hand mobility
Lifestyle and observation
A lot depends on your daily habits. Fingers need to be protected from the cold: warm mittens, not gloves, warming your hands before going out, avoiding sudden temperature changes. Smoking is especially dangerous for this disease, as it further constricts blood vessels and accelerates lung damage. Regular exercises for the hands and face, moisturizing the skin, eating small portions with the head of the bed elevated for heartburn are useful. Observation by a rheumatologist with a planned assessment of the lungs, heart and kidneys is carried out regularly, even if you feel well.
- Protection of hands from cold, warm mittens
- Complete smoking cessation
- Daily gymnastics for hands and facial expressions
- Moisturizing and gentle skin care
- Fractional meals, raised head of bed
- Regular blood pressure monitoring at home
- Routine examination of the lungs and heart
- Vaccination in consultation with a doctor