MINISTRY OF EDUCATION AND TRAINING DEPARTMENT OF DEFENSE MILITARY MEDICAL UNIVERSITY HUYNH VAN KHOA CHARACTERISTICS OF IMMUNE DISORDERS AND RESULTS OF PULSE METHYLPREDNISOLONE THERAPY IN SEVERE SYSTEMIC LUPUS ERYTHEMATOSUS Specialization: Internal Medicine Code: 9720107 SUMMARY OF DOCTORAL THESIS HA NOI – 2018 THE THESIS WAS COMPLETED AT: MILITARY MEDICAL UNIVERSITY Full name of supervisor: Associate Pr LE ANH THU Associate Pr LE THU HA Review 1: Associate Pr Nguyen Dang Dung Review 2: Associate Pr Phan Quang Doan Review 3: Associate Pr Dang Hong Hoa The dissertation will be protected before the Board of thesis dissertation at the Military Medical University By the day month year Can study thesis at - National Library of Vietnam - Library of Military Medical University FOREWORDS Systemic lupus erythematosus is a chronic disease characterized by a wide variety of clinical manifestations characterized by the production of autoantibodies that cause disorders of the immune system Systemic lupus erythematosus can occur at any age, including children and older adults, but the incidence rate of women suffering from the disease accounts for 90% of the cases Clinical manifestations of the progression of the disease are usually in the skin, joints, hematology, organ damage (kidney, cardiovascular, respiratory ) Severe internal organ damage is the direct or indirect cause of death The use of high-dose intravenous (pulse therapy) methylprednisolone for cases of flare severe systemic lupus erythematosus life-threatening and many studies have shown to be effective In Vietnam, there are no studies that adequately assess the efficacy and safety of this therapy, especially in cases where lupus is a progressive multiorgan lesion So we conducted this study with the following objectives: Study some characteristics of immune disorders, analysis of association with target organ damage and activity level (SLEDAI score) in patients with severe systemic lupus erythematosus Evaluation of treatment outcome of pulsed methylprednisolone therapy combined with baseline treatment after 12 weeks in patients with severe systemic lupus erythematosus Thesis structure  The thesis has: forewords (3 pages), chapter 1: overview (40 pages), chapter 2: research subjects and methods (19 pages), chapter 3: research results (36 pages), chapters 4: Discussion (30 pages), and conclusions (2 pages), Recommendations (1 page)  In the thesis there are: 48 tables, graphs, diagrams  The thesis has 167 references, including 16 in Vietnamese, 150 in English, French CHAPTER 1- OVERVIEW 1.1 Autoimmune antibodies The autoimmune antibodies and their pathological evidence in patients with lupus are described in the table below: specific antibody Frequency % Main clinical manifestations Anti-dsDNA 70 -80 Kidney, skin Anti –Nucleosome 60- 90 Kidney, skin Anti- Ro 30- 40 Anti – La 15 -20 Skin, kidneys, Fetal cardiovascular problem Fetal cardiovascular problem Anti – Sm 10-30 Kidney disease Anti-NMDA receptor Anti–Phospholipid 33- 50 20- 30 Encephalopathy Circumcision, miscarriage Anti -α Actinin Anti - C1q 20 Kidney disease 40- 50 Kidney disease 1.2 Immune characteristics in patients with SLE Reduction of C3, C4 complement is a common manifestation of lupus erythematosus The disorder of the immunoglobulin in patients with SLE is very diverse and depends on the level of activity of the disease 1.3 Evaluations of progression and severe SLE manifestations 1.3.1 SLEDAI (Systemic Lupus Erythematosus Disease Activity Index) SLEDAI reviews consist of 24 components SLEDAI scores range from to 105 points Descriptor Score Seizure, psychosis, organic brain syndrome, visual disurbance, neuropathy involving a cranial nerve, lupus headache, cerebrovascular accident, vasculitis Arthritis, Myositis, Casts, Hematuria, Proteinuria, Pyuria New malar rash, alopecia, mucous membranes, pleurisy, pericarditis, low complement, increased DNAbinding Fever, thrombocytopenia, leukopenia Evalation process value by SLEDAI SLEDAI score Activity level SLEDAI = Not active SLEDAI – minor active SLEDAI 6-10 Average active SLEDAI 11-19 Severe active SLEDAI  20 Very severe active 1.3.2 Evaluation of severe organ damage  Lupus nephritis had nephrotic syndrome, reduced creatinine clearance> 25% during months of follow-up Proteinuria> 2g / day for months of follow-up, glomerulonephritis progressing rapidly  Severe autoimmune hemolysis (Hb 3g / 24h, decreased urinary protein