Research progress of mechanism of IL-37 in rheumatoid arthritis / 上海交通大学学报（医学版）

Rheumatoid arthritis (RA) is a chronic progressive autoimmune disorder, the mechanism of which is not clear yet. Cytokines play a key role in the pathogenesis of RA. Recent studies on cytokine involvement in autoimmune diseases have found that, as a new member of interleukin-1 (IL-1) family, IL-37 can participate in the regulation of signaling pathway and play its anti-inflammatory role by extracellular binding to membrane receptor and intracellular complex formation. Studies have shown that the expression level of IL-37 in healthy people is extremely low and overexpressed in RA patients, and the serum level of IL-37 in RA patients is positively correlated with the level of pro-inflammation cytokines such as tumor necrosis factor-α (TNF-α), IL-6 and IL-1α, the level of C reactive protein and 28-joint disease activity score (DAS28). However, IL-37 treatment can alleviate the inflammation and pathological symptoms of collagen-induced arthritis (CIA) mice, which indicates that IL-37 has anti-inflammatory effect via negative feedback. The specific anti-inflammatory mechanism may be related to mothers against decapentaplegic homolog 3 (SMAD3) pathway and nuclear factor kappa-B (NF-κB) pathway. This article reviews the structure and expression of IL-37, the receptor and its signal pathway and the function and its mechanism in RA, which provides references for the further study of IL-37 and RA.

Review of 113 cases of autoimmune disorders in pregnancy

Background: Autoimmune disorders are chronic multisystem disorders affecting women of their reproductive age. Pregnancy among these women is complicated by the disease itself. Flaring up of disease, uteroplacental insufficiency due to vasculitis and drugs used for treatment are main reason for unfavourable pregnancy outcomes. The objective of this study was to analyze the pregnancy complications and outcome among 113 cases of autoimmune disorders.Methods: This study is a retrospective analysis of case records of pregnant women with autoimmune disorders. This study was conducted at PSG IMSR and Hospital, Coimbatore, and Tamil Nadu from July 2012 to June 2018. The case sheets were retrieved from medical record department and the details such as type of disease, maternal age, parity, status of disease at conception, treatment taken during pregnancy, maternal complications and fetal complications were collected and analyzed.Results: During 6-year period there were 113 pregnant women with autoimmune disorders, and they were studied of their previous and present pregnancy outcome. The disease incidence was 7.01% per 1000 deliveries. 40.7% were less than 25 years age group and 71.6% were multigravidae. We had forty-one patients (36%) who were positive for APLA syndrome which were the maximum number of patients with auto immune disorder in pregnancy and 37 patients (32.7) with SLE. We had significant numbers of maternal and fetal complications for common disorders. Most of the rare auto immune disorders were diagnosed before pregnancy and these pregnancies were managed by multi-disciplinary approach, and continued on immunomodulators throughout pregnancy, hence maternal and fetal complications were less for them.Conclusions: Adequate pre-conceptional counseling, vigilant monitoring during pregnancy and post-partum will avoid pregnancy related complications and have favorable outcomes.

Progress of lymphoid-specific tyrosine phosphatase inhibitors / 药学学报

Lymphoid-specific tyrosine phosphatase (LYP) is a phosphatase that is encoded by protein tyrosine phosphatase non-receptor type 22 and is mainly distributed in lymphoid. In psychological condition, LYP inhibits T-cell receptor (TCR) signaling in association with C-terminal kinase (CSK). While in pathological condition, mutant LYP dissociates with CSK, which augments the inhibition of TCR signaling and leads to autoimmune diseases. Consequently, LYP is now considered as a new target of type I diabetes, rheumatic arthritis and Graves disease and some other autoimmune disorders. This review mainly focuses on the development of LYP inhibitors in their structures and activities.

Desmoglein Compensation Theory: An explanations for Early Appearance of Oral lesions as Compared to Skin Lesions in Pemphigus Vulgaris

Pemphigus is a life-threatening autoimmune blistering disease targeting skin and mucous membranes. It is clinically characterized by flaccid blisters and erosions, while histologically shows intraepithelial acantholysis.  The disruption of desmoglein-dependent cell adhesion by autoantibodies is the basic pathophysiology in blister formation of pemphigus. The clinical and histological spectrum of pemphigus is complex and differs in various variants of pemphigus. This review offers an answer to why the splits associated with pemphigus foliaceus occur in the superficial layer of the epidermis, while those of pemphigus vulgaris occur deep in the epidermis. With the help of desmoglein compensation theory, it logically explains why oral erosions develop in patients with pemphigus vulgaris, but not in patients with pemphigus foliaceus and why some patients with pemphigus vulgaris have only oral involvement, but others have extensive lesions on both skin and mucous membranes. Learning objective: After completing this article, readers shall be familiar with the clinical presentations, histologic findings, immunopathology  of classical pemphigus and its  variants. It discusses the  desmoglein compensation theory of pathogenesis. along with the management of pemphigus.

Case of Acute Disseminated Encephalomyelitis.

Acute disseminated encephalomyelitis (ADEM) is characterized by Immune mediated inflammation of brain causing and gray matter. ADEM is an which usually follows a viral or bacterial infection, immunizations, and drug or serum administration. One such case is being reported here where a 3 year old girl was brought in status epilepticus as presenting feature of ADEM.

Varying Arrhythmias during Single Hospital Stay in a Case of Systemic Lupus Erythematosus.

Cardiac involvement is present in more than half of the patients with systemic lupus erythematosus (SLE). However, studies on the prevalence of arrhythmias in this disease and laboratorial correlations predictive of their development do not exist. Possible pathophysiological mechanisms include autoimmune process, atherosclerosis and adverse effects of the drugs especially chloroquine used for treatment of this disorder. We present a unusual case of SLE with varying arrhythmias during a single hospital stay.

Management of guillain-barré syndrome-an autoimmune disorder: A review.

Guillain-Barré syndrome is a disorder in which the body's immune system attacks part of the peripheral nervous system. The first symptoms of this disorder include varying degrees of weakness or tingling sensations in the legs. In many instances, the weakness and abnormal sensations spread to the arms and upper body. There is no known cure for Guillain-Barré syndrome, but therapies can lessen the severity of the illness and accelerate the recovery in most patients. There are also a number of ways to treat the complications of the disease. Currently, plasmapheresis and high-dose immunoglobulin therapy are used. Plasmapheresis seems to reduce the severity and duration of the Guillain-Barré episode. In high-dose immunoglobulin therapy, doctors give intravenous injections of the proteins that in small quantities, the immune system uses naturally to attack invading organism. The most critical part of the treatment for this syndrome consists of keeping the patient's body functioning during recovery of the nervous system. This can sometimes require placing the patient on a respirator, a heart monitor, or other machines that assist body function. The aim of present article is to provide in depth knowledge about Guillain-Barré syndrome which is no doubt, a rare autoimmune disorder. In this article the author has explained all the clinical aspects related to Guillain-Barré syndrome. This article presents a brief review of Guillain-Barré syndrome with an emphasis on its possible management and therapies.

A radiographic correlation between systemic disorders and pulp stones.

Aim: The purpose of this study was to determine the correlation between pulp stones and cardiovascular disorders, Type II diabetes mellitus, autoimmune disorders and dental wear defects. This study also aims to evaluate the frequency of pulp stone in population of Dakshina Kannada district (Karnataka, India) and to determine association of pulp stones in different sexes, tooth type, dental arches and sides. Patients and Methods: A total of 1432 teeth of five groups were examined, comprising of patients with C.V.S. disorders; Type II diabetes mellitus, autoimmune disorders, dental wear defects and control group. Teeth were examined under 2Χ magnification on radio visiograph (RVG) and conventional intra-oral periapical radiograph. The presence or absence of pulp stones were recorded. Statistical Analysis Used: Chi-square analysis was used to compare the frequency of occurrence of pulp stones between sexes, tooth type, dental arches and sides. Results: Pulp stones were found in 134 (9.35%) of 1432 teeth detected. Significantly, higher numbers of pulp stones were recorded in patients with cardiovascular disorder (15.86%) than other groups. The occurrence of pulp stones were significantly higher in molars (18.29%) than premolars (6.6%) and in maxillary arch (12.36%) than in mandibular arch (5.95%). No significant difference was found between sexes and sides. Conclusion: Positive correlation was found between systemic disorder and pulp stones. Cardiovascular patients had maximum number of pulp stones followed by dental-wear defects and least number of pulp stones were evident in control group.

Immune responses to Epstein-Barr virus in individuals with systemic and organ specific autoimmune disorders.

Purpose: Autoimmune diseases usually manifest in genetically predisposed individuals following an environmental trigger. There are several viral infections including Epstein-Barr virus (EBV) implicated in the pathogenesis of autoimmune disorders. The aim of this study was to look at the antibody pattern to EBV proteins in the plasma of both systemic and organ specific autoimmune disorders, estimate pro-inflammatory plasma cytokines (IL-8 and TNF-á) among these autoimmune patients and compare the observations with those in normal healthy controls. Materials and Methods: Samples from 44 rheumatoid arthritis patients, 25 Hashimoto's thyroiditis patients, appropriately age and sex matched healthy controls were tested for EBV IgM antibodies by an immunoblot assay and two cytokines (IL-8 and TNF-á) by commercial assays. Results: Among the rheumatoid arthritis patients, 23 (52%) were positive for EBNA1 antibody, while 13 (52%) of the Hashimoto's thyroiditis patients and 12 (30%) of the healthy controls showed similar bands. The intensity of the bands was high in the autoimmune patients when compared to the bands seen in control samples. The difference in the EBNA1 reactivity between rheumatoid arthritis patients and controls were significant (P = 0.038). There was a significant difference in the IgM reactivity to VCAp19 protein between patients and controls (P = 0.011). Conclusion: Our study showed an increased EBV activation among the autoimmune patient groups compared to the normal healthy controls. Further studies are required to delineate the association between the aetiology of autoimmune disorders and EBV.

A Case of Primary Autoimmune Myelofibrosis / 대한혈액학회지

Myelofibrosis is usually observed in association with hematologic malignancies or metastatic solid tumors, but it has rarely been reported in patients who suffer with autoimmune disorders. Autoimmune myelofibrosis is a distinct clinicopathologic entity and it can occur alone or in association with autoimmune disorders, and the final result is chronic peripheral cytopenia. Primary autoimmune myelofibrosis, in which the autoimmune myelofibrosis is not preceded by a well-defined autoimmune disease, has recently been defined as a distinct clinicopathologic syndrome. We report here on a case of an 18-year-old woman who was diagnosed with primary autoimmune myelofibrosis, and she manifested peripheral pancytopenia, positivity for autoantibodies and Grade III myelofibrosis without having any preceding autoimmune or hematologic disorders.

A Case of Multiple Gastric Carcinoid in a Woman with Systemic Lupus Erythematosus / 대한류마티스학회지

Gastric carcinoid tumor is a rare neoplasm and it accounts for only 0.3% of all the gastric neoplasms. Gastric carcinoid tumor has high incidence in patients who are aged between 50 and 70 years, and it usually develops over a long period of time. Type I gastric carcinoid tumors are associated with autoimmune atrophic gastritis and pernicious anemia, and there are a few cases of gastric carcinoids in patients with other autoimmune diseases like Sjogren's syndrome and autoimmune thyroiditis. Here, we report on a case of recurrent type I gastric carcinoid tumors in a 42-years old woman who suffered with systemic lupus erythematosus.

Efficacy and Mechanism of Action of Saireito Therapy for Autoimmune Recurrent Abortion Indexed by Antinuclear Antibody and Anticardioripine Antibody / 日本東洋医学雑誌

The efficacy of Saireito (a herbal preparation) against autoimmune recurrent abortion was evaluated clinically and immunologically. Saireito therapy was performed with 87 women having experienced 3 or more recurrent abortions in the early stages of pregnancy (less than 12 weeks gestation), and whose antinuclear antibody (ANA) and anti-cardiolipine antibody (ACA) test results were positive. The effects of this therapy on the percentage of women in whom abortion was successfully prevented (the abortion prevention rate), and on the antibody titer were investigated. Among the 49 women who became pregnant during the study period, the abortion prevention rate was 63.3%, the percentage of ANA positive cases was 65.6%(32/49), the percentage of ACA IgG and/or IgM positive cases (as evaluated by SRL methods) was 65.5%(29/49) and the percentage of both ANA and ACA IgG and /or IgM positive cases was 75.0%(12/49). Although the titer of ANA was not significantly reduced by this therapy, the titer of ACA IgM was reduced significantly.These results suggest that Saireito exerts its efficacy by suppressing humoral immunity through its adjustment of the Th1/Th 2 cytokine balance. This therapy is expected to be effective against ACA-positive recurrent abortion by reducing the effects of ACA. Furthermore, in 2 observed child birth cases whose ACA titer were not decreased by Saireito, different effective mechanisms were speculated; for example, suppression of platelet aggregation via saireito's component herbs (ninjin and bukuryou) similar to the effect seen with low-dose aspirin therapy, or its “risui” effect via its components (bukuryou, soujyutu, takusha and chorei).

Is Tokishakuyakusan Clinically Useful in Treatment of Immunologic Recurrent Abortion? / 日本東洋医学雑誌

Clinical efficacy of tokishakuyakusan against immunologic recurrent abortion was evaluated using methods of diagnostic statistics. The subjects of this study were 38 women with recurrent abortion who had experienced a spontaneous abortion during their first trimester (within 12 weeks of pregnancy) twice, and who took tokishakuyakusan (extract granules) after development of their third pregnancy, but experienced spontaneous abortion again during their first trimester, with the karyotype of the aborted fetus being rated as 46, XX or 46, XY. The control group consisted of 244 women who had experienced 3 or more spontaneous abortions during their first trimester of pregnancy. The detection rates of autoimmune disorders and alloimmune disorders were compared statistically between the two groups. Neither the detection rate of autoimmune recurrent abortion nor the detection rate of alloimmune recurrent abortion differed significantly between the two groups, suggesting that tokishakuyakusan is not clinically effective against immunologic recurrent abortion. Tokishakuyakusan is estimated to prevent abortion caused by compromised luteal insufficiency of the uterus. Thus, saireito seems to provide a more rational means of Kampo therapy for immunologic recurrent abortion.

A Case of Mixed-type Autoimmune Hemolytic Anemia / 대한혈액학회지

We report a case of mixed-type autoimmune hemolytic anemia in a 73-year-old female. At first, she visited at Pusan National University Hospital, complaining of dizziness and dyspnea for 3 months. Her hemoglobin level was 5.5g/dL, so red blood transfusion was requested by her physician. In ABO blood grouping, a discrepancy between cell and serum typing was observed. Also all of donor cells for crossmatching were reactive with patient's serum. High mean corpuscular volume (MCV) value was not normalized after warming. The cold agglutinin titer was 1 : 64 at 4degrees C, and was increased up to 1 : 1,024 at room temperature and 37degrees C, suggesting mixed-type autoimmune hemolytic anemia. The patient's symptoms and signs were improved progressively with corticosteroid therapy, and also ABO discrepancy was disappeared.

Antinuclear Antibody Production in Patients with Malaria Infection / 대한임상병리학회지

BACKGROUND: Infection can activate the immune system and may trigger the production of autoantibodies. It has been reported that malaria infection triggers the production of various autoantibodies. Therefore, we investigated the pattern and significance of antinuclear antibodies (ANA) found in patients with malaria infection. METHODS: Our study group included 36 patients who were diagnosed with malaria infection at Mokdong Hospital from July 1998 to July 2001. We performed antinuclear antibody test using indirect immunofluorescence method (Quantafluor, Sanofi Diagnostics Pasteur Inc., USA), extractable nuclear antigen test (ENA) using double immunodiffusion method (Nova Gel, Inova Diagnostics Inc., USA), anti-double stranded DNA Ab test (anti-ds DNA Ab) using Farr assay (DPC anti-DNA, Diagnostic products Corporation, USA), and anti-single stranded DNA Ab test (anti-ssDNA Ab) using enzyme immunoassay method (QUANTA, Lite ssDNA, Inova Diagnostics Inc., USA). RESULTS: Among the 36 patients, 32 patients (88.9%) showed ANA positivity and 27 patients (75.0%) showed cytoskeleton or speckled pattern of ANA. Anti-ssDNA Ab was found in 3 of 20 patients; however, anti-dsDNA Ab and ENA were not found in all patients. Patients who had ANA showed higher levels of IgG, IgM and IgA, compared with those patients who did not have ANA. Follow up (11-37 month) of the 13 patients with ANA positivity revealed no symptoms associated with autoimmune disorder. CONCLUSIONS: Malaria infection may develop ANA, especially cytoskeleton or speckled pattern. The follow up of patients with ANA positivity showed no symptoms associated with any autoimmune disorder, but further evaluation would be necessary to reveal the relationship between malaria infection and development of autoimmune disorder.