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connective tissue disease (CTD)-related pulmonary hypertension (PAH) is one of the challenges that rheumatologists must face, the most common CTD caused by PAH in China is SLE, PAH is a common and serious complication of SLE
s1,slea-related PAH The prevalence rate reached 3.8% in the registered study of the Chinese Systemic Lupus Research Collaboration Group (CSTAR), the proportion of SLE-related PAH in CtD-related PAH in China was nearly 50%On May 29, at the 14th Oriental Cardiology Conference (OCC2020), Professor Li Mengtao, from the Department of Rheumatology immunology at Beijing Concord Hospital, shared with experts the themes of "Treatment Options for SLE-PAH Basic Diseases" and "The Role of hydroxychloroquine in immune disease-related pulmonary hypertension and the value of new coronary pneumonia"Professor Li Mengtao,of SLE-PAH Basic Disease Treatment Options, said that an important concept in SLE-PAH treatment is "
standard treatment"Related Consensusrecommend "
double standard" :(1) SLE condition in a clinical lyse state: SLEDAI 4, BILAG system rating c/D/E, PGA 1 points; Distance of 380-440m, PA2 2 /min/kg; serological, BNP 50ng/L or NT-proBNP 300ng/L (normal or near normal); imaging, right ventricular structure and function normal or near normal; RHC check, right ventricular pressure Hg 8 mm, amyometic index (CI ) and the treatment standard can improve long-term prognosis, the strategy of compliance treatment is to select the corresponding targeted therapy according to the risk stratification Patients with underlying inflammatory manifestations (pulsatis) were treated with more proportion of the risk stratification improvement than patients without the performance of underlying inflammation, i.e patients were more likely to undergo intensive treatment to achieve a prognosis for improvement Professor Li Mengtao combined a case (shown below) to explain the treatment of SLE-PAH underlying disease, including the treatment of metastase shock, as well as the selection of cyclophosphamide (CTX) and hydroxychloroquine (HCQ) immunosuppressants, which showed that after 4 weeks of treatment, the patient's pulmonary vascular resistance decreased from 7.6 to 2.5WU in a single-center study at Beijing Concord Hospital, a total of 110 cases of SLE-PAH confirmed by RHC were included, and 71 patients were subjected to joint targeted therapy, including 48 for intensive immunosuppressive therapy (IST) and 23 for non-IST, which showed better prognosis improvement in the enhanced IST group after the basic diseases and targeted treatment the effect of better patients, continued to be low-risk state, after full communication with this class of patients, the reduction of targeted drugs, follow-up for more than six months, two-thirds of patients can still maintain clinical serology and hemodynamic stability a study of SLE-PAH in Japan (pictured below), treatment is also a high dose of hormone therapy, gradually reducing, play the role of anti-inflammatory, immunosuppressive, select CTX for immunosuppression, patients can be controlled in addition to CTX, the choice of immunosuppressants are thiopental, olemcorol (MMF), tamcmo, etc., CTX is the preferred immunosuppressant in the induction phase In addition to targeted drugs, ctX and MMF are recommended by the American Society of Rheumatology (ACR) for first-line treatment However, the latter kinds of drug-related research and clinical evidence is not enough biologics, bellizumab can selectively identify and block free B lymphocyte stimulators in serum, and the treatment of other small molecule drugs is also being explored the role of oxychloroquine in immune disease-related pulmonary hypertension
The history of HCQ begins with "Quinine", the first clinical report of antimalarial drugs for SLE therapy has a history of more than 120 years; The mechanism of HCQ's treatment of SLE is mainly two points: (1) anti-inflammatory: interfering with T-cell phage and antigen profiling function, anti-proliferative effect on T cells, reducing the production of a variety of pro-inflammatory cytokines (including INF-thym, TNF, IL-1, IL-6 and IL-2), etc.; the relative risk of SLE recurrence decreased by 2.5 times compared to placebo, and the risk of new system damage in all patients decreased by 32% (see chart below), and further research was needed to determine whether the risk of PAH could be reduced Another cause of death in SLE patients is cardiovascular disease, HCQ through antioxidant stress, inhibition of T-cell activity, blocking the interaction between TLRs and nucleic acid ligands to reduce the appearance of cardiovascular complications, whether PAH also has a corresponding effect is not yet determined The value of HCQ in the new coronavirus pneumonia the mechanism of the role of HCQ also includes anti-infection, Professor Li Mengtao reviewed the HCQ treatment of new coronavirus pneumonia (COVID-19) related literature, found that many large-scale clinical trials of critically ill patients, think that HCQ on THE condition of COVID-19 has no significant effect A systematic review, published may 27 in Ann Intern Med (Yearbook of Internal Medicine), 6,
Professor Li Mengtao, , speculated that because HCQ's effect on SLE is slow, it usually takes three months, some COVID-19 patients are progressing faster, 7-10 days of application can not produce significant results Professor Li Mengtao pointed out that HCQ can play a preventive role in COVID-19 can be a key research direction , for example, can study for patients in close contact, the application of HCQ can reduce the state of COVID-19 from latent infection to clinical expression, whether it can produce the effect of blocking immunity and inflammatory storms Professor Li Mengtao concluded that SLE's organs are affected, PAH exceeds neuropsychiatric lupus, the proportion is still rising, how to identify early prevention is still a problem faced by rheumatologists, whether hydroxychloroquine may block the appearance of PAH, COVID-19 can play a preventive role also needfurther research Ref: .
Li M, Zhang W, Leng X, et al Chinese SLE Treatment and Research Group (CSTAR ) registry : 1 Major clinical drance of Chinese patients with lupusthem eryatosus Lupus, 2013, 22 ( 11 ) : 1192-1199 Hao Y J, Jiang X, Zhou W, et al Connective tissue disease-associated pulmonary arterial iperon in Chinese patients Eur Respir J,2014,44 (4): 963-972 , National Rheumatoid Data Center, China Systemic Lupus Research Collaboration Group Consensus on the diagnosis and
treatment of pulmonary arteries associated with systemic lupus erythematosus in China McLaughlin VV, Gaine SP, Howard LS, et al S S S Hearts of p J Am Coil Cardiol, 2013, 62 (25 Suppl): D73-81 Hernandez AV, Roman YM, Pasupuleti V, Barboza JJ, White CM Hydroxychloroquine orquine for The Treatment or Prophylax of COVID-19: A Ofa Province Review Ann Intern Med 2020; 10.7326/M20-2496 doi: 10.7326/M20-2496 Author: Violet Source: MedSci Original