Professor Zhang Xiao: For patients with hard skin, it is recommended to do high-resolution CT in the lungs once a year.

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The treatment of patients with SSc ILD should achieve double goals - not only to control the disease, but also to control the progress of the lung.systemic sclerosis (SSC), also known as scleroderma, is a rare autoimmune disease characterized by fibrous hyperplasia and incurable [1-3].it can cause fibrosis of skin and main organs (such as heart, lung, digestive tract and kidney), and may be life-threatening [2,4].about 25% of SSc patients have significant lung involvement within 3 years after diagnosis [4].SSC can cause interstitial lung disease (ILD) in the lung, known as systemic sclerosis associated interstitial lung disease (SSC ILD) [2,4], which is the key driving factor of death in SSc patients, accounting for about one third of the death toll [2,4].in September this year, a new solution to this intractable disease was finally found. Based on the data from the senscis study, the U.S. drug administration (FDA) approved the extension of indications for idiopathic pulmonary fibrosis (IPF) to SSC ILD, and became the first and only drug to slow down the decline of lung function in patients with SSc ILD.on December 8, at the second ILD innovation and development forum, the "medical community" had the honor to invite Professor Zhang Xiao, vice president of Rheumatology branch of Chinese Medical Doctor Association and director of rheumatology department of Guangdong Provincial People's Hospital, to share their views on the current domestic ssc-ild status, diagnosis and treatment, and the latest released senscis research data.professor Zhang Xiao, 60% of SSc patients died from pulmonary fibrosis and pulmonary hypertension. From the global incidence rate, SSc is still a rare disease category, but in the field of rheumatic immunology, SSc is a relatively important disease.from the pathological point of view, the biggest manifestation is that the patient's skin becomes hard, which is also the pathological change caused by fibrosis.fibrosis in SSc patients can have problems from the surface skin to the internal organs. In the past, the most common cause of death was renal fibrosis leading to renal insufficiency.in recent ten years, the popularization of dialysis technology has gradually controlled the situation of kidney death. "Pulmonary fibrosis and pulmonary hypertension have become the important causes of death of SSc patients, and about 60% of patients die of these two complications."said Professor Zhang Xiao.Figure 1: in patients with scleroderma, the proportion of pulmonary fibrosis in scleroderma related deaths increased from 6% to 33% [5].it should be pointed out here that although the typical SSc patients are skin first manifestation, there are also SSc patients with organ damage as the first manifestation."about 10% - 20% of patients will take ILD as the first manifestation, which is very difficult to diagnose."Professor Zhang Xiao told" the medical community ".on the one hand, if the first symptom of pulmonary fibrosis is pulmonary fibrosis, the probability of misdiagnosis will be greatly increased. because the symptoms are similar to the pathological types and imaging features of IPF, it is difficult for doctors to identify SSC ILD without "hardening" of other organs including skin. on the other hand, it is often too late for doctors to realize the changes in lung interstitium associated with scleroderma when the patient has "scleroderma" symptoms, and the treatment is very limited. how to diagnose and control the progress of SSC ILD patients? SSC ILD is similar to IPF, and its clinical diagnosis is very difficult, so how to distinguish it? Professor Zhang Xiao said that for patients with suspected SSC ILD, high-resolution CT (HRCT) is still the most important clinical means, which is simple, easy to operate and noninvasive. B ultrasound and lung biopsy are also important means to help diagnosis. based on her own experience, she summed up the following three points: SSC ILD belongs to connective tissue disease, and generally has the characteristics of "immune color" (antibody positive or Raynaud phenomenon); patients with SSc ILD may have joint pain / skin changes, but these clinical manifestations are unlikely to occur in IPF patients; there are a large number of lymphocyte infiltration or lymphoid foci formation in pathological biopsy of SSC ILD patients However, IPF patients are less likely to have these phenomena. in case of immunological abnormalities and clinical symptoms, doctors in rheumatology and Immunology Department should fully consider whether the patient is pulmonary fibrosis caused by SSC. It is recommended to receive pulmonary HRCT examination regularly every year, and take corresponding measures as soon as possible to achieve the goal of achieving double goals, i.e. controlling the disease and controlling the progress of the lung. "inflammation is an important initiating factor of SSC ILD. If this factor can be controlled, ILD can be improved to a certain extent by controlling SSC, and even the disease progression can be inhibited and reversed. "said Professor Zhang Xiao. the curative effect of nidanib is definite, and the progression free survival time is significantly improved. Clinically, there is no effective drug for anti fibrosis treatment. The median survival time of SSC ILD patients is only 5-8 years, which has not changed until recent years. nidanib (trade name: vegate) has been approved for listing in China in September 2017, and its approved indication in China is IPF. due to its clear efficacy, it was only 6 months from the application for the market to the approval of the drug. and in September this year, FDA further extended the indication of nidanib to SSC ILD based on the clear clinical data of senscis. senscis is a phase III, double-blind, randomized, placebo-controlled trial involving 576 patients (including Chinese patients) from 32 countries and regions. the primary end point was the annual decrease rate of forced vital capacity (FVC) (ml / year) during 52 weeks. Figure 2: compared with the placebo group, the annual decline rate of FVC was significantly reduced by 43.8%; the difference between the two groups was 40.95 ml / year, P = 0.0350 [6]. the results showed that compared with placebo, nidanib could reduce the decline of lung function in patients with SSc ILD. the annual decline rate of FVC decreased by 43.8% in patients treated with nidanib [6]. in addition, the 52 week, randomized, double-blind phase III inbuild trial data released in September this year showed that the decline rate of lung function of the whole study population was reduced by 57%, and it was equally effective and safe for patients with multiple progressive fibrosis interstitial lung diseases other than IPF [7]. Professor Zhang Xiao said that although the first indication of nidanib was not for rheumatic patients, the news that the new indication of SSC ILD was approved in the United States this year has brought great positive significance. especially under the condition that doctors have limited means to control the treatment of the disease, nidanib is expected to control the progress of pulmonary fibrosis, giving doctors and patients more confidence. we look forward to the early approval of its new indications for rheumatism in China, so as to bring more benefits to SSc patients in China. introduction of experts: Professor Zhang Xiao, doctoral supervisor; director of Department of Rheumatology, Guangdong Provincial People's Hospital; vice president of Rheumatology branch of Chinese Medical Association; executive director of Guangdong Medical Association and chairman of rheumatism immunology branch; vice chairman of Rheumatology society of Guangdong Medical Association; member of China Medical science and Technology Award; and medical accident technical appraisal specialty of national and Guangdong Medical Association Family members. references [1] discler o, et al. Nintedanib for systemic sclerosis associated interactive lung disease. N English J Med. Published, 20 May, 2019 NEJM.org . DOI: 10.1056/NEJMoa1903076[2]Denton CP, Khanna D. Systemic sclerosis. Published 13 April, 2017 Last accessed March 2019.[3]Cottin V, et al. Interstitial lung disease associated with systemic sclerosis (SSc-ILD). Respir. Res. 2019;20(1):13.[4]Solomon JJ, et al. European Respiratory Update: Scleroderma lung disease. Eur. Respir. Rev. 2013; 22: 127, 6–19.[5]Steen VD, et al. Ann Rheum Dis. 2007 Jul;66(7):940-4.[6]1. Denton CP, Khanna D. Systemic sclerosis. Published 13 April, 2017 Last accessed March 2019.[7]Flaherty K, et al. Nintedanib in Progressive Fibrosing Interstitial Lung Diseases N Eng J Med. Published 29 September, 2019. NEJM.org . doi: 10.1056/nejmoa1908681 - end - love me please show me!