Systemic sclerosis-related interstitial pulmonary disease patients, how to turn the risk of "dimensional" yi?

The approval of new indications of Viagra in the treatment of SSC ILD is conducive to the improvement of clinical diagnosis and treatment, which will become a new treatment option for SSC ILD patients.first knowledge of SSC ILD - it is known that SSC ILD is harmful to systemic sclerosis (SSC), also known as scleroderma. It is an inflammatory fibrotic disease characterized by localized or diffuse skin thickening and fibrosis.in addition to skin involvement, it can also involve multiple organs such as heart, lung and digestive tract [1].SSc is a rare disease. The incidence rate and prevalence rate vary from place to place. The majority of the cases are female and the age is 25-55 years old [2].compared with other connective tissue diseases, the proportion of SSC in the lung is higher, and the proportion of ILD in Chinese SSc patients is as high as 85.5% [3,4].the onset of SSc is insidious, clinical asymptomatic, early diagnosis is difficult, some patients directly show end-stage pulmonary fibrosis, lung function damage is serious, is the main cause of death of SSc.ILD often complicates the clinical manifestations of SSC, worsens the disease, reduces the survival rate of patients, and increases the medical burden, which has become the difficulty of breakthrough in medical research in the world.risk identification should not be ignored. Although useful SSC ILD biomarkers have not been found and verified, prognosis and risk information may be provided from several known aspects of the disease as guidance for regular detection and treatment.SSC ILD can occur without dyspnea. In SSc ILD patients, the degree of pulmonary fibrosis on HRCT is a predictor of mortality, and the higher the degree of fibrosis on HRCT, the lower the survival rate [5].early diagnosis of SSC ILD is challenging and requires routine screening.the latest European consensus recommended in 2020 [6]: early HRCT screening, pulmonary function examination and paying attention to symptoms and complaints are of great importance, especially pulmonary function examination.the most common manifestation of pulmonary function examination is the decrease of FVC. In SSc ILD patients, the higher the annual decline rate of FVC, the shorter the survival time [7] (P = 0.003) (Fig. 1).and FVC is an indicator that needs to be paid attention to, regardless of patient screening, disease diagnosis or prognosis evaluation (as shown in Figure 2). The latest European consensus is that the detailed management plan of systemic sclerosis related ILD is urgent. At present, the treatment of SSC ILD is mainly hormone combined with immunosuppressant.however, no drug was approved for this indication in the field of SSC ILD treatment, and there was a high unmet medical demand [8].so is there a targeted anti fibrosis drug with anti-inflammatory effect that can be targeted for the treatment of SSC ILD? The pathogenesis of SSC ILD involves the activation of fibroblasts and the deposition of extracellular matrix [9]. It has been confirmed in the past that vegate can target anti fibrosis and has anti-inflammatory effect, thus slowing down the decline of lung function and delaying the progress of disease [10], which promotes the possibility of exploring the treatment of SSC ILD with nidanib.the positive results of the senscis study of Viagra in the treatment of SSC ILD [11] were published in the New England Journal of medicine in 2019 and presented at the 2019 ATS International Conference, thus bringing new treatment options to patients.the results showed that compared with placebo, nidanib significantly reduced the annual decline rate of FVC in SSc ILD patients and delayed disease progression. at the end of the 52 week trial, the adjusted annual decrease rate of FVC was - 52.4 (ml / year) in the nidanib group and - 93.3 (ml / year) in the placebo group, which was equivalent to slowing down the decline of lung function by 44% (Fig. 3). three international multicenter clinical studies published in the New England Journal of medicine have shown that the effect of reducing lung function decline can last from the beginning to 52 weeks (Fig. 4). in addition, compared with the control group, the proportion of patients with stable and improved FVC% in the nidanib treatment group was more than that in the control group. The predicted FVC% value of 44% of the patients receiving the nidanib treatment did not decrease or even improve, compared with 34% of the patients in the control group [12] (as shown in Figure 5). Figure 3. The annual decline rate of FVC in SSc ILD patients within 52 weeks Figure 4. The annual decrease trend of FVC in patients with SSc ILD reduced by nidanib 5. The change of predicted FVC% from baseline at the 52nd week was recommended by European expert consensus of SSC ILD in 2020: nidanib is an effective choice to start or upgrade treatment. in addition, the 2018 ctd-ild China consensus [13] mentioned that the treatment principle of CTD related ILD (including SSC ILD) is early, standardized and individualized treatment. the choice of treatment plan for CTD related ILD should comprehensively consider the disease activity, ILD severity and progression tendency of CTD, and determine the weight and primary and secondary relationship between immunosuppressive therapy and antifibrotic therapy. no matter CTD activity or remission, no matter CTD progress or reaching the standard, anti fibrosis drugs (such as nidanib) can be timely used to maintain pulmonary function stability to the greatest extent. at present, it is encouraging to note that the indication 14 for SSC ILD is highly recognized by the State Food and drug administration. It is only 12 months from the application for SSC ILD indication to the approval. Viagra is the only drug approved for SSC ILD indications in the world (including China), and the only antifibrotic drug recommended by the new version of SSC ILD expert consensus. SSC ILD is a progressive disease, and its diagnosis and treatment status is not optimistic. the results of this senscis study by vegat are a breakthrough in the clinical treatment and research of SSC ILD. the approval of new indications of nidanib in the treatment of SSC ILD is conducive to the improvement of clinical diagnosis and treatment, which will become a new treatment option for SSC ILD patients. References: [1]. Cai Hourong. Practical interstitial lung disease, 2nd Edition. [2]. Steen VD, et al. Ann Rheum dis 2007; 66:940 – 4. [3]. Walker UA, et al. Ann Rheum dis 2007; 66:754-63. [4]. Hu s, et al. Arthritis res ther. 2018 Oct 22; 20 (1): 235. [5]. Goh ns, et al. Am J respir critical care Med 2008; 177:1248-54.[6].The identification and management of interstitial lung disease in systemic sclerosis: evidence-based European consensus statements. Lancet Rheuma 2020, 2(2): 71-83[7].Guler SA, et al. Ann Am Thorac Soc. 2018 Dec;15(12):1427-1433.[8].Khanna D, et al. Rheumatology (Oxford). 2019 Apr 1;58(4):567-579.[9].Bussone G, et al. Autoimmun Rev. 2011 Mar;10(5):248-55. [10].Fukihara J,et al. Expert Rev Respir Med. 2016 Dec;10(12):1247-1254.[11].Distler O, et al. N Engl J Med. 2019 Jun 27;380(26):2518-2528.[12].Boehringer Ingelheim. Briefing materials for the Meeting of the Arthritis Advisory Committee. 2019 (Accessed July 30, Chinese Journal of internal medicine. 2018; 57 (8): 558-565. [14]