Nowadays, many people often suffer from scleroderma. This symptom usually affects our body, causing the skin surface to be thick, and swelling, erythema and skin pigmentation often occur. The most obvious thing is that it will cause some blood circulation problems in the patient's body, but you need to be careful to prevent it and do the corresponding work. Symptoms and signs 1. Extrarenal manifestations The typical clinical manifestations of patients are thickening of the skin on the fingers and face, followed by thickening of the skin on the trunk and limbs. Raynaud's phenomenon often occurs first, which is manifested as cold or emotional tension inducing vasospasm, causing whitening of the fingers or cyanosis, usually involving bilateral fingers and sometimes toes. About 80% of patients with Raynaud's phenomenon are due to PSS. Skin changes include swelling, erythema, hypopigmentation or hyperpigmentation of the skin, followed by thinning of the epidermis, the appearance of nodules, and the skin clinging to the subcutaneous tissue. Skin biopsy reveals epidermal atrophy, deep dermal fibrosis, loss of skin appendages, and peripheral vascular infiltration of mononuclear cells and mast cells, especially in the early stages. The esophagus is the most commonly affected visceral organ. Dysphagia secondary to esophageal motility is a common symptom. Esophageal manometry revealed changes in esophageal motility in 87% of patients, with decreased pressure in the lower esophageal sphincter, which can lead to reflux esophagitis and Barrett's esophagus (chronic peptic ulcer and esophagitis syndrome). Extensive small intestinal lesions may lead to malabsorption in some patients. About one-third of patients develop pulmonary fibrosis leading to restrictive respiratory dysfunction. The heart is affected in 30% to 50% of patients, leading to arrhythmias or progressive heart failure. Unlike atherosclerotic heart disease, left-sided cardiac fibrosis is uncommon in these patients. 2. Renal manifestations The frequency of clinical kidney disease is significantly lower than that of morphological kidney lesions, and is estimated to range from 1.5% to 97%, depending on the length of clinical observation. 36% of patients with this disease developed proteinuria, 24% developed hypertension, 19% developed azotemia, and 7% developed malignant hypertension syndrome. In one report, only 10% of 116 patients without clinical manifestations of renal disease died during the 20-year follow-up period, but 60% of 94 patients with proteinuria, hypertension, or renal insufficiency died during the same period. Clinical renal manifestations of PSS are: (1) Proteinuria: Proteinuria is a common symptom, but rarely is it a symptom of nephrotic syndrome. In the early stage, proteinuria is often mild, intermittent or persistent. Generally, the 24-hour urine protein quantity is less than 500 mg, which may be accompanied by mild microscopic hematuria and leukocyturia. Proteinuria can be the only symptom, but most patients also have mild to moderate hypertension and chronic renal damage. The prognosis of patients with simple proteinuria is much better than that of patients with hypertension and azotemia. About 50% of proteinuria patients are associated with the occurrence of hypertension and azotemia. Persistent proteinuria indicates a poor prognosis, with a mortality rate of approximately 64% within 3 years, while only 5% of those without proteinuria die within the same period. (2) Hypertension: Hypertension is considered a clinical sign of renal damage in patients and a common manifestation of renal damage. The incidence rate is 25% to 58%, which has a significant impact on the prognosis of PSS patients. The mortality rate of patients with hypertension is 2.5 times higher than that of those with normal blood pressure, and 2/3 of patients with hypertension die within 20 years. Hypertension in PSS patients can be divided into two types: one is chronic hypertension, which accounts for 75% to 80% of hypertensive patients with this disease. It usually has an insidious onset, a chronic course, and is continuous or intermittent. Acute renal failure rarely occurs. This benign hypertension often occurs in the late stage of the disease and has little effect on the prognosis. Only about 15% of patients with chronic hypertension will die. |
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