The systemic sclerosis also known as scleroderma, is a chronic autoimmune disease that primarily affects the skin.
Systemic sclerosis or scleroderma, in addition to affecting the skin, can affect the internal organs (lungs, intestines, kidney or heart). Skin changes are caused by the increase and accumulation of collagen fibers and other proteins. The skin is sclerous (it becomes hard and rigid).
At first, the injuries consist of an inflammation, which later turns into a hardening.
Systemic sclerosis also hinders blood supply by occluding the small arteries and capillaries that carry blood to the tissues, explains the best rheumatologist in Delhi.
What are the usual symptoms?
The most visible effects are skin lesions. It usually begins with a slight swelling of the skin of the hands and feet, sometimes also of the face, which is spreading. Subsequently, the skin becomes stiff and hard, becomes difficult to pinch, and sometimes limits joint movements.
However, in those with whole-body involvement, scars and thickening extend to the underlying structures (fat, muscle, and rarely bone). There are usually changes in the color of the hands that, when exposed to the cold, become excessively pale and later purplish, sometimes accompanied by pain or tingling. These symptoms are called Raynaud’s phenomenon. Poor circulation can cause serious damage, such as ulcers on the fingers or gangrene.
There may be dilated blood vessels on the face, hands, nail folds, and other places. Some patients develop calcium deposits (calcinosis) on the skin and other organs such as the lungs, muscles, and kidneys.
Systemic sclerosis can also lead to joint pain, fatigue, digestive problems, such as difficulty swallowing, heartburn, constipation or diarrhea, and cardiorespiratory problems such as respiratory distress, hypertension, or chest pain, states the top rheumatologist in Delhi.
The most common symptoms are:
- Rigid and hard skin.
- Joint pain
- Raynaud’s phenomenon.
How is it diagnosed?
The diagnosis is made through a specialized medical examination by the rheumatologist in Delhi, which will include a report of the symptoms, a skin examination and a general examination.
Complementary examinations are always performed such as analysis and a chest x-ray, and only in some cases other digestive or cardiorespiratory tests.
The purpose of these tests is to better classify the type of disease and assess its severity and extent.
How do we treat it?
No drug has been shown to be effective in 100% of patients with systemic sclerosis, although there are partial responses to immunosuppressants in cases of systemic involvement.
Treatment of the inflammatory stage of the skin lesion usually requires the administration of corticosteroids.
Pulmonary interstitial involvement may improve, initially, with oral cyclophosphamide / mycophenolate mofetil and steroids.
In the case of pulmonary hypertension, calcium blockers, prostacyclin, or the like can be used.
Gastrointestinal involvement improves with anti-reflux postural measures, H2 blockers, prokinetics, and erythromycin. Broad spectrum antibiotics can be used for malabsorption.
Calcium blockers together with antiplatelet agents are effective in Raynaud’s phenomenon.
Regular monitoring of blood pressure is essential in patients with diffuse sclerosis. Treatment should be started when blood pressure is above 140/90 mmHg. Cases of marked calcinosis may require microsurgery.
For the treatment of muscle involvement, the use of corticosteroids is recommended in vigorous guidelines.
The treatment of arthralgias, arthritis or tenosynovitis requires the use of non-steroidal anti-inflammatory drugs (NSAIDs) and rest. The use of local infiltrations can be very useful.