How is systemic sclerosis treated? 4 pictures to fix! . . . you "drugs" know.

Collect! Systemic sclerosis (SSC) is a systemic autoimmune disease with unknown etiology. Collagen fiber deposition leads to skin thickening and fibrosis, and visceral organs are involved. It is common in women. Most of the onset age is 30-50 years old.the clinical manifestations were Raynaud's phenomenon, skin changes, bone and joint pain, gastrointestinal symptoms, pulmonary hypertension, interstitial pneumonia, cardiac insufficiency, renal crisis, etc.at present, the aim of early treatment of SSc is to prevent new skin and organ involvement, while the purpose of late treatment is to improve the existing symptoms.the treatment measures mainly include anti-inflammatory and immunomodulatory therapy, treatment of vascular diseases and inhibition of fibrosis.the effect of anti-inflammatory and immunomodulators ▏ glucocorticoids is not significant, but it has certain effect on the early stage of skin lesions (edema stage), joint pain, muscle lesions, serositis and inflammatory stage of interstitial lung disease.it is not recommended for the treatment of diffuse skin type SSC, especially for skin sclerosis.the dosage of glucocorticoid was prednisone 30mg / day for several weeks, and gradually reduced to 5-10mg / day for maintenance treatment.high dose is a risk factor of SSC renal crisis, which can promote renal vascular occlusion in patients with advanced azotemia, so the dosage should be cautious.▏ immunosuppressants such as cyclophosphamide, methotrexate, azathioprine and mycophenolate mofetil are not effective.it has been reported that these drugs may be effective for skin, joint or kidney diseases. When combined with glucocorticoid, it can often improve the curative effect and reduce the dosage of glucocorticoid.among them, methotrexate may be effective in improving early skin sclerosis, but not in other organs.therefore, methotrexate is recommended for the treatment of early skin symptoms of diffuse SSc.cyclophosphamide is recommended for the treatment of interstitial lung disease with SSc, and cyclophosphamide pulse therapy is effective in controlling active alveolitis.2 vasoactive drugs ▏ calcium antagonists calcium antagonists can dilate blood vessels, relax vascular smooth muscle, and reduce peripheral vascular resistance. They are usually only used in patients with positive results of acute vasodilator drug test, and should be selected according to heart rate. Dihydropyridines should be selected for patients with slow basic heart rate, and diltiazem should be selected for patients with faster basic heart rate.it should be used from a small dose. When there is no obvious change in systemic blood pressure, the dose should be gradually increased to reach the maximum tolerable dose within a few weeks, and then maintain the application.▏ prostaglandin derivatives such as eprostol, iloprost and traprost are potent pulmonary vasodilators with antiproliferative and antiplatelet effects, and are also the first-line drugs for the treatment of SSC related Raynaud phenomenon.in who-fc grade III patients, eloprost and traprost are class I recommendations, who-fc grade IV patients are class I recommendations, iloprost and traprost are class II B recommendations.▏ endothelin-1 receptor antagonists such as bosentan, amrisentan, etc.its efficacy has been confirmed in the treatment of SSC related Raynaud phenomenon, especially for SSc patients with pulmonary hypertension, finger tip ulcer and poor circulation.these drugs are recommended by who-fc Ⅱ - Ⅲ as class I and who-fc Ⅳ as class Ⅱ B.▏ PDE-5 inhibitors: sildenafil, Tadanafil, vardenafil, etc.it inhibits the degradation of cGMP and enhances the vasodilation effect of nitric oxide.in who-fc Ⅱ - Ⅲ, who-fc Ⅳ is class Ⅱ B recommendation.sildenafil is recommended for the treatment of SSC related pulmonary hypertension in Europe, with an initial dose of 20mg, three times a day.common adverse reactions include headache, facial flush, etc., but they are generally tolerable.▏ angiotensin converting enzyme inhibitor (ACEI) is mainly used in SSc related renal crisis.early application of ACEI can make some patients avoid dialysis treatment, and 50% of dialysis patients can finally get out of dialysis.before the application of ACEI, the one-year and five-year survival rates of renal crisis were 22% and 18%; after ACEI, the one-year and five-year survival rates of renal crisis were increased to 76% and 65%, respectively.3 the antifibrotic agent penicillamine has been used in the treatment of scleroderma for a long time, which helps to reduce the cross connection of skin collagen and inhibit the synthesis of new collagen.penicillamine is taken on an empty stomach from 0.125g per day. Generally, it increases by 0.125g/d in 2-4 weeks. It can be used to 0.75-1g/d according to the condition.after half a year to one year of treatment, most of the skin sclerosis will be improved, and the frequency of renal crisis and progressive lung involvement may be reduced.it should be continued for 1-3 years.other antifibrotic agents include colchicine. 4 biological agents should be considered for patients with contraindications or ineffective or refractory hormone and immunosuppressive agents. TNF - α antagonist can alleviate skin fibrosis and reduce systemic inflammatory reaction, but it is not suitable for single application. anti CD-20 monoclonal antibody can improve skin fibrosis and pulmonary function, but a large number of RCT results show that rituximab can significantly improve the skin pathological characteristics. SSC treatment drugs recommended by the British Society of Rheumatology and the British Association of Rheumatology Health Professionals (BSR and bhpr) guidelines for the treatment of systemic sclerosis in 2016 are shown in Table 1: Table 1 MTX: MTX for the treatment of crisis; MTX: MTX for the treatment of crisis; MTX: MTX for the treatment of crisis; MTX: MTX for the treatment of crisis. symptomatic treatment of SSC can be divided into localized skin type SSC (lcssc) or diffuse skin type SSC (dcSSc) according to the range of skin thickening. the proximal limb and trunk skin were classified as dcSSc, and only the distal limb skin was classified as lcssc. the treatment of SSC should consider its classification, disease duration and visceral involvement. in addition to symptomatic treatment, patients with early active SSC should be given immunosuppressive therapy. BSR and bhpr guidelines for the treatment of systemic sclerosis (2016) summarize the general treatment principles of SSC, as shown in Figure 1; the recommended treatment options for SSC related diseases in China are shown in Table 2; the recommendations for treatment of systemic sclerosis by the European anti rheumatism alliance in 2017 are shown in Table 3: Figure 1: general treatment principles of SSC; table 2: recommended treatment schemes for SSC related diseases in China; table 3 EULAR recommendations on SSC treatment [1] expert consensus on glucocorticoid therapy for immune-related dermatosis (2018). [2] Huang Jin, he Minhua. Research status of pathogenesis and treatment of systemic sclerosis [J]. Electronic Journal of general Stomatology, 2019,6 (21): 147-148. [3] Liu Junlong, Xiang Yang. Research status and treatment progress of systemic sclerosis [J], [4] guidelines for diagnosis and treatment of systemic sclerosis (2011). [5] guidelines for diagnosis and treatment of systemic sclerosis (2019 Edition). [6] Jiang Zhixing, Liang Jianmin, Xue Yu, Zou Hejian. Interpretation of 2016 BSR and bhpr systemic sclerosis treatment guidelines [J]. [7] Yao Haihong, Bai Ma Yangjin. Update of recommendations on systemic sclerosis treatment by European anti rheumatism alliance in 2017 [J]. Chinese Journal of Rheumatology, 2017,21 (8): 575-576. [8] fan Wenjing, Li Yang. Research progress of systemic sclerosis complicated with pulmonary hypertension [J]. Journal of clinical pulmonary science, 2019, 24 (5): 946-949