Dry eye disease in systemic lupus erythematosus: a cross sectional study

AIM:To explore ocular surface manifestations of dry eye disease(DED)and its influencing factors in systemic lupus erythematosus(SLE)patients.METHODS:Ophthalmological examinations were conducted in SLE patients(n=43)and controls(n=41),including Ocular Surface Disease Index(OSDI),objective scatter index(OSI),tear meniscus height(TMH),lipid layer thickness(LLT),non-invasive Keratograph tear breakup time(NIKBUT),corneal fluorescein score(CFS),Schirmer I test.DED was diagnosed according to the Tear Film and Ocular Surface Society Dry Eye Workshop II Criteria.SLE patients were further divided into DED group and non-DED group,the disease activity,clinical manifestations and laboratory investigations were compared between the two groups.The disease activity was evaluated by Systemic Lupus Erythematosus Disease Activity Index 2000(SLEDAI-2K).Receiver operative characteristic(ROC)curve and multiplefactor binary logistic regression were performed.RESULTS:SLE patients showed higher OSDI[9.1(2.8-15.9)vs 6.3(2.2-7.5),P=0.035],higher OSI[1.67(1.09-2.60)vs 0.96(0.87-1.60),P=0.001],higher CFS[1(0-2)vs 0(0-1),P=0.001],lower LLT[65(42-100)vs 100(79.5-100),P=0.010],and lower NIKBUT[8.03(4.02-9.73)vs 9.67(5.26-12.71),P=0.030]than controls.The 32.6%of SLE patients had DED,which was higher than 12.2%of healthy controls.DED group showed higher SLEDAI-2K score[9.7±6.1 vs 5.4±3.4,P=0.025],higher anti-cardiolipin antibody(ACL)[8.7(3.5-13.2)vs 3.6(2.0-6.9),P=0.035],and higher proportion of patients with cutaneous eruption[42.9%vs 6.9%,P=0.015]than non-DED group.According to multiple-factor binary logistic regression analysis,the SLEDAI-2K score(OR=1.194,P=0.041)and cutaneous eruption(OR=7.094,P=0.045)could be consider as risk factors for DED in SLE patients.The ROC curve of the combined factors including age,disease duration,SLEDAI-2K score,ACL,and cutaneous eruption was analyzed,with a sensitivity of 0.786,a specificity of 0.793,and an area under curve of 0.820.CONCLUSION:Ocular surface affection is frequent in SLE patients,and patients with high disease activity and cutaneous eruption show increased risk of DED.

Ovarian function in patients with systemic lupus erythematosus:Pathogenesis,drug application and prospective therapies

Systemic lupus erythematosus(SLE)is a chronic autoimmune disease in which multiple organs are damaged that prevails in fertile women.Currently,glucocorticoids and immunosuppressants are widely used to treat SLE patients.However,ovarian dysfunction occurs following the use of these drugs in women with SLE.Here,we summarize recent progress in terms of understanding ovarian injury,the effects of drug application and strategies to improve ovarian function in women with SLE.This review could be helpful to precisely cure SLE in women desiring to have offspring.

Case Report: Rhupus (The Association of Systemic Lupus Erythematosus and Rheumatoid Arthritis): About 2 Cases

Introduction: The association of systemic lupus erythematosus and rheumatoid arthritis (rhupus) is a rare clinical condition. Throughout the world, 287 cases of Rhupus have been described. We report two new observations of two patients who presented predominantly distal erosive polyarthritis with positive anti-Sm antibodies in one case and SmRNP in the other case. Observations: Case 1: 37 years old patient, with a recent diagnosis of pulmonary tuberculosis. She has since 8 months an inflammatory, bilaterally and symmetrical polyarthralgia without deformation or ankylosing synovitis, associated with malar erythema without other abnormalities. Immunological tests showed: positive Rheumatoid factor at 158 IU/ml, positive Anti-CCP at 550 IU/ml, and positivity of antinuclear at 1/1280 nuclear fluorescence with a strong presence of anti-Sm >8 IU/ml. The diagnosis of rhupus was concluded, without serious visceral involvement. Case 2: A 28-year-old patient, married with 3 children, with bilateral, symmetrical, deforming and chronic polyarthritis affecting large and small joints, which had been evolving for over 5 years without cutaneous abnormality associated. Paraclinical investigations showed: a biological inflammatory syndrome. Immunology was positive, with rheumatoid factors at 78 IU/ml, anti-CCP at 561 IU/ml, antinuclear antibodies at 1/1280 with positive anti-SmRNP and anti-SSA/Ro52, and a positive direct Coombs test. Joint ultrasound revealed tenosynovitis of the extensors and common flexors of the fingers, erosions and synovitis of multiple PPIs. The diagnosis of rhupus was based on the presence of 10 ACR criteria for RA and 8 ACR/EULAR 2019 criteria for SLE. Conclusion: Rheumatoid arthritis is a rare autoimmune disease combining features of both systemic lupus erythematosus and rheumatoid arthritis in the same patient, often sequentially. Despite a growing number of case reports and series, a consensus on the classification of SLE arthritis is still lacking, and diagnostic criteria for rhupus do not exist. These cases of rhupus must be recognized, as the vital and/or functional prognosis may be different from SLE alone or isolated RA.

Life course changes provoked by chronic disease: A study on everyday life for patients with systemic lupus erythematosus

Objective:This study examined patients with systemic lupus erythematosus(SLE)as a means to explore the impacts of chronic disease on life courses.Methods:A semi-structured in-depth interviews were conducted with nine patients in China in 2017;participants were included based on their having or having had facial butterfly erythema.Results:The study focused on both the disease's impact on the patients'health and on their daily lives.Four core themes emerged:visible changes in the patient's bodies,social dilemmas,"the encouragement of disease",and a new perspective on the relationship between disease and health.One important finding was that the occurrence of a chronic disease did not have only negative repercussions;some patients felt that there were advantages to being sick.Chronic disease resulted in a reworking of daily life.The patients developed a self-referential model of healing.Conclusion:The distinct interpretations of the same disease offered by different patients served to yield a more complete understanding of the disease.People with SLE adjust their thinking about the disease based on personal feelings as well as experiences and pursue a dialogue on their illness based on the disease pattern unique to them.The meaning that disease had for the patients was not limited to negative connotations.

Suicide risk in systemic lupus erythematosus:A narrative review

Introduction:Despite the medical and scientific advances,the disease’s restrictions and the perception of personal and social losses related to its course reinforce fear and generate intense suffering in lupus patients.Psychiatric comorbidities,especially major depressive episodes,are highly prevalent during systemic lupus erythematosus.Among them,suicide is a behavior that is much more common than we believe.Objective:To perform a narrative review on suicidal behavior associated with systemic erythematosus lupus(SLE).Results:Studies have shown an increased risk of suicide among patients with chronic diseases and psychiatric disorders,especially depression.However,suicide cannot be attributed only to a higher prevalence of depression and other mental illnesses.Therefore,it is necessary to learn more about the suicide risk factors present in patients with lupus to work on secondary prevention and avoid the premature loss of lives and the additional suffering of families and surrounding communities.The coordination between the studies on suicidal behavior and its intricate network of individual and sociocultural factors and the studies on this multisystem autoimmune disease with a broad manifestation spectrum,lupus,creates new and essential field research.Conclusions:Non-psychiatrist office-based physicians,health clinics,or wards dedicated to the treatment of SLE should recognize and handle the suicide risk factors on their patients to reduce the suffering caused by this disease.

Ankle-Brachial Index in Systemic Lupus Erythematosus:A Senegalese Case-Control Study

Systemic lupus erythematosus (SLE) is associated with accelerated atherosclerosis and increasing cardiovascular risk which is recognized as a major cause of morbidity and death. Whether subclinical atherosclerosis has been evaluated by several methods, there are very limited data about Ankle-Brachial Index (ABI) in patients with systemic lupus erythematosus. The aim of the present study was to compare this index, between SLE patients and controls. We conducted a case-control study in the Department of Internal Medicine of our institution during the period from August 1, 2017 to December 31, 2018. We included 100 subjects, including 50 SLE patients and 50 control cases. This included 44 women and 6 men in patients with an identical distribution in controls. The mean age was 33.5 ± 11.3 years for cases and 33.3 ± 11.3 years (p-value: 0.93) for controls subjects. There was higher frequency of cardiovascular risk factors such as dyslipidemia (p-value at 0.009), low level of serum HDL-cholesterol (p-value??0.001), hypertriglyceridemia (p-value at 0.000) and hyperuricemia (p-value at 0.000) in patients with SLE. Overweight/obesity was higher in controls subjects (p-value at 0.028). There was no statistically significant difference in the frequency of diabetes-mellitus, smoking and high blood pressure. The abnormally ABI was recorded in 19 patients with SLE (38%) and 8 controls (16%) with a p-value: 0.01 and odds ratio: 3.22. Eight patients (16%) and four controls (8%) had low ABI without significant difference (p-value: 0.11 but odds-ratio at 2.98). Eleven patients with SLE (22%) and five controls (10%) had high ABI (p-value: 0.05 and odds-ratio: 3.24). In patients with SLE only disease activity (cSLEDAI) at the inclusion of the study was correlated?to abnormal ABI.?Conclusion:?This study showed an increasing prevalence of abnormal ABI in black African patients with SLE compared to controls with a correlation between disease activity and abnormal ABI.

Acquired von Willebrand Syndrome in a Male with Systemic Lupus Erythematosus Presented with Mucocutaneous Bleeding and Treated with rFVIIa

Background: Systemic lupus erythematosis (SLE) is a disorder with multiple organ involvement. Haematological abnormalities have been addressed in it, but acquired von Willebrand syndrome is a rarer phenomenon in current disease. The Case: We report acquired von Willebrand syndrome and SLE in a man with brown rash on face, gingival bleeding, easy bruising and epistaxis and laboratory finding of decreased complement, high level of anti-nuclear antibody and anti-DNA. These findings confirmed the diagnosis of SLE. He underwent kidney biopsy and experienced severe pain at the site of biopsy, but the ultra-sonography evaluation showed small sub capsular haematoma at the site of biopsy. During the next 48 hours, gradually APTT prolongation was continued and haematocrit dropped. In spite of FFP infusion and taking tranexamic acid every eight hours, there wasn’t any improvement in haemostatic condition. He received Methylprednisolone and Cyclophosphamid pulses. The patient underwent surgery to roll out vascular complication, but there wasn’t any vascular problem. On the third day, recombinant activated factor VII was infused every two hours until oozing was stopped.

An atypical presentation of Kikuchi-Fujimoto disease mimicking systemic lupus erythematosus: case report and literature review

Purpose: To report a case of atypical Kikuchi-Fujimoto disease (KFD) that illustrates several overlapping features with systemic lupus erythematosus (SLE). Methods: A case is reported followed by a review of the current literature. Case report: A 16-year-old boy with an unusual manifestation of Kikuchi-Fujimoto disease (KFD) is described. The patient presented with fever, weight loss and severe abdominal pain, due to extensive necrotizing retroperitoneal and mesenteric lymphadenopathy. During the course of his illness, he developed several symptoms suggestive of systemic lupus erythematosus (SLE): a pericardial effusion, cotton wool spots on the retina and antibodies against nuclear antigens (ANA), Smith (Sm) and ribonucleoprotein (RNP) antigens. However, no additional features of SLE were found. The patient subsequently fully recovered within two months, without initiation of immunosuppressive therapy. His autoantibodies became negative five months after initial presentation and he remains well at his 23 month follow up visit. Discussion: We hypothesize that the autoantibodies developed by our patient were secondary to self-antigen induced autoimmunity related to his extensive tissue necrosis. Despite initially having clinical features suggestive of SLE, our patient’s full and spontaneous recovery strongly supports the diagnosis of KFD. This illustrates the need for careful diagnosis, in order to avoid unnecessary and potentially toxic treatment with immunosuppressive agents.

Dual autoimmune diseases:Rheumatoid arthritis with systemic lupus erythematosus and Type 1 diabetes mellitus with multiple sclerosis

Autoantibodies and inflammation are the hallmarks of autoimmune diseases(ADs).Organ-specific and nonorgan-specific ADs are divided according to whether the autoimmune reaction is directed against a specific tissue(e.g.,thyroid in Hashimoto's thyroiditis)or widely expressed antigens(e.g.,cell nuclei in systemic lupus erythematosus[SLE]).SLE is distinguished by the presence of circulating autoantibodies and immune complex deposition,both of which can induce inflammatory damage to many organs.Rheumatoid arthritis(RA),sometimes called inflammatory arthritis,is a systemic AD that affects the joints and causes synovitis.Multiple sclerosis(MS)is a central nervous system inflammatory disease with various neurological and autoimmune symptoms.Links have been reported between RA and SLE as well as between Type 1 diabetes mellitus and MS.Identification of shared genes and biological processes could aid in the discovery of possible treatment targets in these dual ADs.This review article explores the molecular nature and familial inheritance of dual ADs.

Association between autoimmune pancreatitis and systemic autoimmune diseases

AIM: To investigate the association between autoimmune pancreatitis (AIP) and systemic autoimmune diseases (SAIDs) by measurement of serum immunoglobulin G4 (IgG4). METHODS: The serum level of IgG4 was measured in 61 patients with SAIDs of different types who had not yet participated in glucocorticosteroid treatment. Patients with an elevated IgG4 level were examined by abdominal ultrasonography (US) and, in some cases, by computer tomography (CT). RESULTS: Elevated serum IgG4 levels (919 ± 996 mg/L) were detected in 17 (28%) of the 61 SAID patients. 10 patients had Sj gren's syndrome (SS) (IgG4: 590 ± 232 mg/L), 2 of them in association with Hashimoto's thyroiditis, and 7 patients (IgG4: 1388 ± 985.5 mg/L) had systemic lupus erythematosus (SLE). The IgG4 level in the SLE patients and that in patients with SS were not significantly different from that in AIP patients (783 ± 522 mg/L). Abdominal US and CT did not reveal any characteristic features of AIP among the SAID patients with an elevated IgG4 level. CONCLUSION: The serum IgG4 level may be elevated in SAIDs without the presence of AIP. The determination of serum IgG4 does not seem to be suitable for the differentiation between IgG4-related diseases and SAIDs.

Therapeutic efficacy of anti-CD19 CAR-T cells in a mouse model of systemic lupus erythematosus

Dysregulated B-cell activation plays pivotal roles in systemic lupus erythematosus(SLE),which makes B-cell depletion a potential strategy for SLE treatment.The clinical success of anti-CD19 CAR-T cells in treating B-cell malignancies has attracted the attention of researchers.In this study,we aimed to investigate the feasibility of applying anti-CD19 CAR-T cell therapy to SLE treatment in a mouse disease model.We constructed murine anti-CD19 CARs with either CD28 or 4-1BB as the intracellular costimulatory motif and evaluated the therapeutic function of the corresponding CAR-T cells by infusing them into MRL-lpr mice.Furthermore,anti-CD19 CAR-T cells were transferred to MRL-lpr mice before the onset of disease to determine their role in SLE prevention.According to our observations,compared with antibody treatment,the adoptive transfer of our anti-CD19 CAR-T cells showed a more sustained B-cell-depletion effect in MRL-lpr mice.The transfer of syngeneic anti-CD19 CAR-T cells not only prevented disease pathogenesis before the onset of disease symptoms but also displayed therapeutic benefits at a later stage after disease progression.We also tried to optimize the treatment strategy and found that compared with CAR-T cells with the CD28 costimulatory motif,CAR-T cells with the 4-1BB costimulatory motif showed better therapeutic efficiency without cell enrichment.Taken together,these results show that anti-CD19 CAR-T cell therapy was effective in the prevention and treatment of a murine model of SLE,indicating its potential for clinical use in patients.

Difficulties in the Management of Systemic Autoimmune Diseases in Saint-Louis Du Senegal through the Analysis of a Series of 70 Observations

Introduction: Systemic Autoimmune Diseases (SAID) long considered very rare in Africa are increasingly the subject of publications. The objective of this work is to identify the difficulties in the management of these pathologies in an internal medicine department in northern Senegal by analyzing the epidemiological, clinical-biological, therapeutic and evolutionary aspects of SAID. Methods: This was a descriptive cross-sectional study carried out in the internal medicine department of the Saint-Louis University Hospital Center. Included were all the files of patients followed in outpatient and/or hospitalization for autoimmune diseases according to the criteria of the American College of Rheumatology, during the period from January 2017 to December 2020. The data were analyzed using SPSS software version 21.0. As the study was descriptive, no statistical test was performed. Results: Out of 3800 patients, 70 presented SAID, i.e. a hospital prevalence of 1.8%. Polyarthritis was the first reason for consultation in 97% followed by skin manifestations in 8%. The patients had positive anti-nuclear autoantibodies in 88% of cases. Rheumatoid arthritis was the predominant condition (71%) followed by systemic lupus erythematosus (SLE) (15%) and undifferentiated autoimmune diseases in 10%. Eleven percent (11%) of patients had an associated autoimmune disease. Corticosteroids were used in the treatment of these conditions in 97% of cases and methotrexate was the most prescribed immunosuppressant (54%). Thirty-two percent (32%) of patients are lost to follow-up. Conclusion: SAID are diverse and under diagnosed;they are characterized by diagnostic delay above all linked to access to specialists and sometimes to the high cost of paraclinical examinations, in particular immunology. Treatment remains based primarily on corticosteroid therapy and conventional immunosuppressants in the face of the unavailability of biotherapies.

Sequential bilateral central retinal artery occlusion as the primary manifestation of systemic lupus erythematosus

Bilateral central retinal artery occlusion （CRAO） has been rarely reported as the primary manifestation in patients with systemic lupus erythematosus （SLE）. The severe retinal vaso-occlusive diseases usually cause devastating and permanent damage to visual function in spite of vigorous treatment. A 42-year-old Chinese woman presented with abrupt bilateral vision loss. The diagnosis of bilateral CRAO was suggested by the ocular presentation and fluorescein angiography. Laboratory studies showed positive results of antinuclear antibody, anti-Ro/SSA anti-La/SSB; decreased levels of C3, C4 complement and normal levels of antiphospholipides antibodies （APAs）. Her visual acuity deteriorated despite systemic steroid and immunosuppressant treatment. Severe vaso-occlusive retinopathy may be an earlier manifestation of SLE without elevated level of APAs.

Elevated levels of serum antibodies against alpha-1,6-glucan in patients with systemic lupus erythematosus or rheumatoid arthritis

This study was undertaken to investigate whether levels of anti-alpha-1,6-glucan antibodies in human sera correlate with rheumatoid arthritis(RA)and systemic lupus erythematosus(SLE).Serum samples were collected from patients with SLE(n=30),RA(n=30)and healthy adult volunteers.IgG,IgA and IgM levels against alpha-1,6-glucan were measured using enzyme linked immunosorbent assays.Anti-alpha-1,6-glucan IgG prevalence was raised in patients with active SLE(73.3%)and RA(60%)compared with healthy controls(13.3%).Strong correlation between anti-alpha-1,6-glucan-IgG levels and anti-perinuclear factor(r=0.642;p<0.05)in RA patients or anti-nuclear antibodies(r=0.675;p<0.05)in SLE patients was observed.No significant differences in anti-alpha-1,6-glucan-IgA or-IgM levels were noted between different groups.We conclude that anti-alpha-1,6-glucan-IgG levels were significantly elevated in patients with SLE or RA and positively correlated with disease activity.

Phenotypic characterization of patients with activated PI3Kδ syndrome 1 presenting with features of systemic lupus erythematosus

Activated phosphoinositide 3-kinase d syndrome 1(APDS1)is a primary immunode-ficiency disease caused by gain-of-function mutations in PIK3CD.Clinical features of autoimmune disease have been reported in patients with APDS1.In this study,we reported three patients with APDS1 presenting with systemic lupus erythematosus(SLE)phenotype.The clinical manifestations included recurrent respiratory tract infection,lymphoproliferation,Coombs-positive hemolytic anemia,decreased complement fractions,positive antinuclear antibodies,renal complications related to SLE associated diseases,which met the clinical spectrum of APDS1 and the classification criteria of SLE.The immunological phenotype included an inversion in the CD4:CD8 ratio,an increase in both non-circulating Tfh CD4^(+)memory T and circulating Tfh populations,a low level of recent thymic emigrant T cells,overexpression of CD57 on T cells,and a decrease in B cells with fewer antibody class switch recombination.These phenotypes detected in patients with APDS1 presenting with SLE were resemble that in patients with APDS1 presenting without SLE.Meanwhile,we described the effect of glucocorticoids and rapamycin therapy on patients with APDS1.The phosphorylation of S6 at Ser235/236 was inhibited in patients with APDS1 who underwent glucocorticoids therapy,including two who presented with SLE phenotype.The phosphorylation of AKT at Ser473 and phosphorylation of S6 at Ser235/236 were inhibited in other patients with APDS1 who underwent rapamycin therapy.Here,we showed the coexistence of immunodeficiency and SLE phenotype in APDS1,and the inhibition of rapamycin in activated Akt-mTOR signaling pathway.

Matrix-assisted laser desorption/ionization time of flight mass spectrometry in the genetic basis of systemic lupus erythematosus and the complicating kidney injury

Background It is necessary to develop some innovative methods to reveal and discover the novel （SLE）-related protein molecules. In the present study, matrix-assisted laser desorption/ionization time of flight mass spectrometry （MALDI- TOF MS） was employed to detect the differential expression of serum polypeptides in the patients with systemic lupus erythematosus （SLE） presenting with a family history or complicating with kidney injury so as to identify the proteins associated with the genetic factors and kidney injury in SLE. Methods The subjects recruited were divided into four groups, that is, a group of SLE patients with both family history and kidney injury, a group of SLE patients with only kidney injury but no family history, a group of SLE patients with neither family history nor kidney injury, and a control group consisting of healthy volunteers. By adopting MALDI-TOF MS analysis, the serum samples obtained from the three groups of SLE patients were examined and compared with those from the control group; the categorized peptide fingerprint profile was established via the biological data collected from the samples. Results The expression of protein with a mlz of 4207 Da increased significantly in SLE patients; the protein with a ml z of 2658 Da was expressed in all SLE patients; three proteins （with mlz of 1465, 5332, and 5900 Da respectively） were expressed in the SLE patients complicated with kidney injury and the protein with a mlz of 1943 Da was expressed in SLE patients with family history. Conclusion A number of differential proteins were successfully detected and identified through MALDI-TOF MS detection and these proteins may be associated with the genetic basis of SLE and the complicating kidney injury.

Sex differences in systemic lupus erythematosus(SLE):an inception cohort of the Chinese SLE Treatment and Research Group(CSTAR)registry XVII

Background:The onset and clinical presentation of systemic lupus erythematosus(SLE)are sex-related.Few studies have investigated the distinctions in clinical characteristics and treatment preferences in male and female SLE patients in the initial cohort.This study aimed to improve the understanding of Chinese SLE patients by characterizing the different sexes of SLE patients in the inception cohort.Methods:Based on the initial patient cohort established by the Chinese SLE Treatment and Research Group,a total of 8713 patients(795 men and 7918 women)with newly diagnosed SLE were enrolled between April 2009 and March 2021.Of these,2900 patients(347 men and 2553 women)were eligible for lupus nephritis(LN).A cross-sectional analysis of the baseline demographic characteristics,clinical manifestations,laboratory parameters,organ damage,initial treatment regimens,and renal pathology classification was performed according to sex.Results:In the SLE group,as compared to female patients,male patients had a later age of onset(male vs.female:37.0±15.8 years vs.35.1±13.7 years,P=0.006)and a higher SLE International Collaborative Clinic/American College of Rheumatology damage index score(male vs.female:0.47±1.13 vs.0.34±0.81,P=0.015),LN(male vs.female:43.6%vs.32.2%,P<0.001),fever(male vs.female:18.0%vs.14.6%,P=0.010),thrombocytopenia(male vs.female:21.4%vs.18.5%,P=0.050),serositis(male vs.female:14.7%vs.11.7%,P=0.013),renal damage(male vs.female:11.1%vs.7.4%,P<0.001),and treatment with cyclophosphamide(CYC)(P<0.001).The frequency of leukopenia(male vs.female:20.5%vs.25.4%,P=0.002)and arthritis(male vs.female:22.0%vs.29.9%,P<0.001)was less in male patients with SLE.In LN,no differences were observed in disease duration,SLE Disease Activity Index score,renal biopsy pathological typing,or 24-h urine protein quantification among the sexes.In comparisons with female patients with LN,male patients had later onset ages(P=0.026),high serum creatinine(P<0.001),higher end-stage renal failure rates(P=0.002),musculoskeletal damage(P=0.023),cardiovascular impairment(P=0.009),and CYC use(P=0.001);while leukopenia(P=0.017),arthritis(P=0.014),and mycophenolate usage(P=0.013)rates were lower.Conclusions:Male SLE patients had more severe organ damage and a higher LN incidence compared with female SLE patients;therefore,they may require more aggressive initial treatment compared to female patients.

Histone H1/MBP hydrolysing antibodies - novel potential marker in diagnosis of disease severity in systematic lupus erythematosus patients

Recently we have shown the presence of catalytically active IgGs, capable to cleave histone H1 and bovine myelin basic protein (MBP), in blood serum of SLE patients. Here we present data that demonstrate the correlation between a) proteolytic activity towards histone H1 and MBP of IgG-antibodies from blood serum of SLE patients and b) disease severity level in these patients. IgGs were isolated from blood serum by chromatography on protein G-sepharose. Commercial preparations of bovine myelin basic proteins (MBP) and calf thymus histone H1 were used as substrates. Analysis of the proteolytic activity showed that 16 of 38 lgG-preparations (42,1%) obtained from blood serum of SLE patients were capable of cleaving both histone H1 and MBP with different efficiency. It was revealed that the presence in blood serum of lgGs possessing proteolytic activity towards both histone H1 and bMBP closely correlates with manifestation of the disease severity in SLE patients.

Differential Diagnosis and Association of Acquired Immunodeficiency Syndrome and Systemic Erythematous Lupus: A Brief Review

Acquired immunodeficiency syndrome and lupus erythematosus are multisystem diseases that can affect several organs and systems at different stages of disease evolution. Both diseases share common clinical manifestations, which may lead to diagnostic difficulties, especially at the onset of the disease. Another additional challenge is when there is an association of the two pathologies. The objective of this brief review is to describe the clinical manifestations of the diseases and to make considerations regarding the moment of onset of symptoms. Knowledge of these common manifestations and their peculiarities may alert clinicians to possible diagnoses and avoid errors in the evaluation and conduction of these patients.

Targeted treatment strategy for patients with severe pulmonary hypertension secondary to connective tissue diseases

Background:Pulmonary arterial hypertension(PAH)associated with connective tissue diseases(CTD)(CTD-PAH)remains a difficult challenge in clinical practice.We aimed to evaluate the effects of targeted vasodilators in patients with severe CTD-PAH.Methods:The data of 53 patients with severe CTD-PAH hospitalized at the Department of Rheumatology and Immunology,The Affiliated Drum Tower Hospital of Nanjing University Medical School,were retrospectively reviewed.Patients were followed up for an average of 2 years to track their outcomes.The efficacy of treatment and the survival rate of patients with severe CTD-PAH were determined.Results:Among the causes of severe CTD-PAH,systemic lupus erythematosus(SLE)was the most common(39.6%),and the age at onset in patients with SLE-PAH was younger than that of patients with other CTD.Bosentan was more effective than sildenafil in reducing pulmonary artery pressure,improving cardiac function,and increasing survival time.Combination therapy with targeted vasodilators significantly improved the prognosis of patients with severe CTD-PAH compared with monotherapy.Conclusions:Patients with severe CTD-PAH should be treated early with targeted vasodilators.In this study,bosentan was superior to sildenafil.Combined treatment might be an option for severe CTD-PAH.