Systemic sclerosis is a rare but challenging autoimmune disease with complex disease progression and serious impact on the quality of life of patients.
In the early stages, systemic sclerosis is often characterized by Raynaud’s phenomenon, in which the fingers of patients turn white, purple and red when they are cold or nervous, accompanied by pain and numbness. During this period, the skin may begin to appear slightly swollen and tight, especially in the fingers, the back of the hand and other parts, as if the skin is gradually tightened and the movement is slightly limited, but many patients may mistake it for a common blood circulation problem and delay diagnosis.
With the development of the disease, the skin lesions are gradually aggravated. The thickening and hardening of the skin can spread from the fingers to the forearms, face, neck and even trunk. Facial skin is tight, wrinkles are reduced, expressions become stiff and mask-like, which not only changes the appearance of patients, but also brings great psychological pressure to them. Skin hardening can also lead to severe limitation of joint movement, and patients’daily movements, such as clenching fists, stretching arms, bending and so on, become difficult, greatly affecting self-care ability.
In terms of
internal organs, the progress of the disease should not be underestimated. Digestive system is often invaded, esophageal peristalsis function is weakened, patients will have difficulty swallowing, feel food stagnation in the esophagus when eating, and even need a lot of water to wash food down. Stomach emptying is delayed, causing discomfort such as abdominal distension and nausea. Intestinal involvement can lead to malabsorption, constipation or diarrhea.
The lung is also a common site of involvement. With the gradual formation of pulmonary interstitial fibrosis, patients will have progressive aggravation of expiratory dyspnoea, and their activity endurance will decrease significantly, from the initial asthma of climbing stairs to the later flat walking and even expiratory dyspnoea at rest. This not only reduces the quality of life of patients, but also seriously threatens their lives.
Cardiopathy also plays an important role in the progression of systemic sclerosis. Myocardial fibrosis can lead to arrhythmia, heart failure and other serious consequences, patients may appear palpitation, chest distress, edema and other symptoms, further aggravating the condition.
Renal involvement can lead to scleroderma renal crisis, manifested by a sharp rise in blood pressure, proteinuria, hematuria and so on, which is a serious and dangerous complication of systemic sclerosis, if not treated in time, it can rapidly develop into renal failure.
In terms of
treatment, although there are still many challenges, with the deepening of medical research, some new therapeutic targets and drugs are being developed. For example, drugs that target immune abnormalities and fibrotic processes are expected to improve patient outcomes, offering new hope for controlling the progression of systemic sclerosis. At the same time, the comprehensive treatment model of multidisciplinary collaboration has gradually become an important means to improve the quality of life of patients and delay the progress of the disease.
