1. Reynolds phenomenon
When the patient feels cold or nervous, his hands and feet will suddenly get cold, his fingers (toes) will turn pale and then purple. 10 ~ 15 After the external stimulation, the vasospasm recovered, and the finger (toe) color was normal, showing red or variegated. This change is called paroxysmal vasospasm (Raynaud phenomenon). Paleness caused by cold can also appear on the tip of the nose, the tip of the tongue, lips and earlobes.
2. Skin
At the early stage of onset (edema stage), the skin was slightly red and swollen, and some patients developed erythema, itching and edema. Finger edema can last for a long time in the early stage, and the skin changes stop at the distal end of the upper limb, and can also spread to the forearm, chest, abdomen, back and face. In diffuse scleroderma, the skin is widely hardened and the pigment is deepened or decreased, making the skin look like salt and pepper.
With the progress of the disease, the skin is tight and shiny, the normal wrinkles and folds disappear, and the facial skin becomes thin and dull. The lips are thin and firm, the mouth is limited, and melanin appears all over the body, and some cases are even earlier. Patchy telangiectasia and subcutaneous calcification can occur on fingers, face, lips, tongue and forearm, with fingertips being the most common, ranging from small spots to large masses, covering knees, elbows or other most prominent parts, with different sizes. In patients with CREST syndrome, calcinosis and telangiectasia are often more obvious.
Scleroderma progresses to scleroderma stage, with skin thickening and skin dryness causing itching. This stage develops gradually and lasts for 1 ~ 3 years or more. Finally, inflammation and fibrosis stopped, and the skin shrank and became thinner. Fibrous tissue clings to subcutaneous tissue, so it is not easy to pick it up by hand. Bone ulcer can appear in flexion and contracture, such as near the finger (toe) joint. In the later stage of atrophy, some parts of the skin gradually soften, which can restore normal skin, especially the skin near the trunk and limbs.
3. Muscles and bones
Nonspecific muscle and bone symptoms such as joint pain and myalgia are the earliest manifestations of scleroderma. Arthritis with obvious symptoms sometimes occurs, but the pain and stiffness at the joints are always more serious than the objective signs of inflammation. Muscle atrophy in patients is caused by apraxia, which is the result of limited joint activity caused by skin, joints and muscle bonds.
4. lungs
Pulmonary dysfunction is common in scleroderma, but the clinical symptoms are often not obvious. Until the late stage of the disease, lung involvement can be the cause of death. The common clinical symptoms are shortness of breath after fatigue (exercise dyspnea) and dry cough after fatigue, which generally do not cause chest pain. Chest pain in scleroderma patients is often caused by muscle inflammation, reflux esophagitis, pleurisy or pericarditis. Fibrous alveolitis progresses to pulmonary interstitial fibrosis or intimal fibrosis, and pulmonary vascular diseases caused by smooth muscle hyperplasia will damage pulmonary ventilation function.
5. Gastrointestinal tract
Patients may have symptoms such as narrowing of oral fissure, dry mucous membrane, chewing difficulty caused by periodontal disease, tooth loss, malnutrition and so on. Acid regurgitation, heartburn and burning sensation behind sternum are the most common symptoms of scleroderma. Persistent reflux esophagitis can lead to bleeding, ulcer, stenosis and Barrett's esophagus, which is easy to turn into esophageal cancer. The etiology of reflux esophagitis is related to the dysfunction of esophageal peristalsis, the decrease of lower esophageal sphincter pressure and the decrease of gastric emptying ability caused by excessive deposition and fibrosis of collagen fibers in esophageal submucosa and muscularis. Prolonged gastric emptying time can not only aggravate gastroesophageal reflux, but also lead to indigestion symptoms such as upper abdominal distension and belching.
Weakening of small intestinal peristalsis may be asymptomatic, or it may cause severe chronic pseudo-intestinal obstruction, which is characterized by severe abdominal distension, abdominal pain and vomiting. Scleroderma can also involve the large intestine and rectum. Muscle atrophy of large intestine wall often causes asymptomatic wide-mouth diverticulum of transverse colon and descending colon, which is a specific damage of scleroderma. Decreased colonic motility can cause intractable constipation. Fibrosis of rectal sphincter will cause insurmountable fecal incontinence and rectal prolapse.
6. Heart
Only in the late stage of the disease, most patients show signs of left ventricular dysfunction, which can lead to dyspnea, palpitation and occasional chest pain after fatigue. Pathological examination and sensitivity diagnosis test of the heart show that myocardium, myocardial vessels and pericardium can be involved. The manifestations of cardiomyopathy include intractable congestive heart failure and various atrial and ventricular arrhythmias. Any symptom of heart disease is a sign of poor prognosis. Transmural patchy myocardial fibrosis is the characteristic of SSc, which determines the nature and severity of cardiac lesions. 30% ~ 40% patients with SSc can find pericardial effusion by echocardiography, but obvious pericardial effusion is not common. A large amount of pericardial effusion is an indication of poor prognosis, but pericardial tamponade rarely occurs. Electrocardiogram showed common damage of cardiac conduction system and asymptomatic arrhythmia.