I. Regarding Raynaud’s phenomenon and ulcers at the ends of the fingers and toes 1. Meta-analyses have shown that nifedipine and intravenous iloprost reduce the frequency and severity of Raynaud’s phenomenon episodes. Nifedipine should be used as the first line of treatment for Raynaud’s phenomenon, while iloprost or other prostaglandin-like agents are often used intravenously for severe Raynaud’s phenomenon (grade A recommendation). 2. 2 RCTs have shown that intravenous prostaglandins (especially iloprost) can heal patients’ phalangeal ulcers, so intravenous prostaglandins (especially iloprost) should be used for active phalangeal ulcers. (Grade A recommendation). 3. The efficacy of bosentan in active phalangeal ulcers is uncertain. 2 high-quality RCTs have shown that bosentan can prevent phalangeal ulcers (especially multiple ulcers) in patients with diffuse disease. When calcium antagonists and prostaglandin-like therapy are ineffective, bosentan should be considered for the treatment of multiple phalangeal ulcers in patients with the diffuse form (grade A recommendation). Second, pulmonary arterial hypertension 1, 2 high-quality RCT studies have shown that bosentan improves exercise capacity, functional class, and certain hemodynamic indices in patients, bosentan is strongly recommended for the treatment of pulmonary arterial hypertension. (Grade A or B recommendation). 2. 2 high-quality RCTs showed that sitaxsentan improved exercise capacity, functional class and certain hemodynamic indices in patients with pulmonary hypertension. Currently, sitaxsentan may also be considered for the treatment of pulmonary hypertension. (Grade A or B recommendation). 3, A high-quality RCT study showed that xitasentan improved exercise capacity, functional class, and certain hemodynamic indices in patients with pulmonary arterial hypertension, and xitasentan may be considered for the treatment of pulmonary arterial hypertension (Grade A or B recommendation). 4. A high-quality RCT showed that continuous intravenous epoprostenol improved exercise capacity, functional class, and certain hemodynamic indices in patients with pulmonary arterial hypertension. Abrupt interruption of the drug can be life-threatening. Intravenous epoprostenol may be used in patients with severe pulmonary arterial hypertension (Class A recommendation). C. Skin lesions 1. 2 RCTs have shown that methotrexate improves skin scores in the early diffuse form, but the benefit for other organ lesions has not been established. Methotrexate may be used for early diffuse skin lesions. (Grade A recommendation). Despite its toxicity, cyclophosphamide is recommended for the treatment of pulmonary fibrosis based on 2 high-quality RCTs (grade A recommendation). v. Scleroderma renal crisis 1. Despite the lack of an RCT basis, experts agree that vascular converting enzyme inhibitors can be used in the treatment of scleroderma renal crisis (grade C recommendation). 2, 4 prospective studies show that the application of hormones and the occurrence of scleroderma renal crisis, so the use of hormones, should closely monitor the patient’s blood pressure and renal function (C-level recommendation). Sixth, gastrointestinal pathology 1, despite the lack of RCT basis, experts agree that proton pump inhibitors can be used to prevent gastrointestinal reflux, esophageal ulcers and strictures. (Grade B recommendation) 2, despite the lack of RCT basis, experts agree that prokinetic drugs can be used for motility disorders of the gastrointestinal tract (e.g., dysphagia, gastroesophageal reflux disease, early satiety, flatulence, and pseudo-obstruction (Grade C recommendation). 3. Despite the lack of an RCT basis, experts agree that antibiotic alternation may be beneficial for patients with intestinal malabsorption due to vigorous bacterial growth.