Lupus Hepatitis Research Articles

Overlap syndrome: Juvenile dermatomyositis with systemic lupus erythematosus and lupus hepatitis in a child: An uncommon presentation

Autoimmune connective tissue disease in children carries a unique management challenge to the treating physician due to the potential years of disease burden and resultant complications. Early diagnosis and treatment positively impact disease course. Overlap syndrome in children is a rare entity. We report a case of juvenile dermatomyositis with systemic lupus erythematosus who on follow-up developed lupus hepatitis. Lupus hepatitis in children with overlap syndrome is not a well-known entity and there is paucity of literature describing this association.

Systemic lupus erythematosus and autoimmune hepatitis overlap disease in a hospitalized systemic lupus erythematosus cohort

Liver dysfunction in systemic lupus erythematosus (SLE) is caused by disease activity or secondary conditions like coexistent autoimmune liver diseases. In Taiwan, despite sporadically reported cases of SLE-autoimmune hepatitis (AIH) overlap disease, larger-scale monocentric investigations for such overlapping patients are not available. Retrospective analyses were performed in a hospitalized SLE cohort with 805 patients for identifying co-existent AIH from 2014 to 2023, focusing on distinct therapeutic modalities and differential diagnosis between SLE-AIH overlap and lupus hepatitis (LH). There were 5 cases (a 0.6% occurrence), all females aged 25–58 years (44 ± 13). Ages for the SLE diagnosis were 19–51 years (30 ± 13), while ages for the AIH diagnosis were 22–57 years (36 ± 14). Contradictory to interface hepatitis in SLE-AIH overlap, liver biopsy only demonstrated non-specific abnormalities in LH. Liver cirrhosis was identified in SLE-AIH overlap but not in LH. After corticosteroids/azathioprine therapy, there were normalized liver function in all LH. In 2 SLE-AIH overlap cases refractory to such therapy, one received B-cell depletion therapy (annual rituximab infusion, 375 mg/m2 weekly × 4) and another accepted living-donor liver transplantation from sibling due to advanced liver cirrhosis, leading to improved hepatic dysfunction in both.

Refractory lupus hepatitis successfully treated with telitacicept who failed to belimumab: A case report and literature review.

Background: Systemic lupus erythematosus (SLE)-associated hepatitis ("lupus hepatitis") was one of the most frequent causes of liver function abnormalities in patients with SLE. Lupus hepatitis (LH) is commonly treated with conventional treatment, including non-steroidal anti-inflammatory drugs, corticosteroids, and immunomodulators. However, in refractory cases, other treatment options may be required.Methodology: We report the case of a patient with lupus hepatitis refractory to both conventional therapy and belimumab who was successfully treated with telitacicept, a new dual B lymphocyte stimulator (BLyS)/APRIL (a proliferation-inducing ligand) inhibitor.Literature review was performed on PubMed search forum.Result: The specific search term was "telitacicept", 23 papers were searched, among them 10 case reports/series articles reporting telitacicept treatment were elected.Apart from our literature reporting the effectiveness of telitacicept in treating LH, there is no report on it in treating LH.Conclusion: This case suggests that telitacicept should be an effective and safe treatment for LH refractory, even to those who failed to belimumab based on the standard treatment, and can reduce the dosage of glucocorticoids.However, further investigations, particularly prospective randomized controlled trials, are warranted to verify our findings and ensure patient safety.

Safety and efficacy of telitacicept in refractory systemic lupus erythematosus patients who failed treatment with belimumab : Acase series.

This study aimed to determine the effect and safety of telitacicept, an antagonist of BLyS/APRIL-mediated Bcell activation, in patients with systemic lupus erythematosus (SLE) who failed treatment with belimumab and in whom telitacicept was administered combined with conventional therapy. Areview of published reports on telitacicept for SLE was also performed. Aretrospective review was performed of the records of patients seen in the Department of Rheumatology at the Wuhan Hospital of Chinese and Western Medicine, Wuhan, China, with refractory SLE who had failed treatment with belimumab. The terms "systemic lupus erythematosus" and "telitacicept" were used to identify patients reported in the English medical literature. Identified were 14refractory SLE patients, 3males (21%) and 11females(79%). The median age was 32.9years. The median disease duration was 8.9years. Patients in this cohort received telitacicept for an average of 34.1weeks (17-62weeks) and the total SLE responder index4 (SRI-4) response rate was 78.9% (n = 11). The mean SLE Disease Activity Index (SLEDAI) score declined from 8.6 at baseline (95% confidence interval [CI] 7.87-9.28) to 4.29 at the endpoint (95% CI 3.4-5.16). All cases (100%) had hypocomplementemia at baseline, and 7cases (50%) reported normal C3 and C4 levels at the follow-up endpoint. At the observation endpoint, the 24‑h urinary protein value of the 13cases with proteinuria (baseline 24‑h urinary protein > 0.5 g/d) displayed areduction, and 3values turned negative. Although some patients had low serum total immunoglobulin (Ig) levels, subnormal IgG levels, and absolute counts of peripheral blood lymphocytes after treatment, no serious infection was reported. One case was refractory lupus hepatitis confirmed by liver pathology, and upon change to change to telitacicept treatment, liver function returned to normal. This is the first case series in SLE patients who accepted telitacicept treatment after failed treatment with belimumab. Our case series and review of the literature show that telitacicept combined with the original standard treatment may significantly improve disease activity while reducing prednisone use. No major safety issues were seen in this group of patients. Telitacicept may be apromising drug for the treatment of refractory lupus hepatitis.

Mortality and Comorbidities of Covid-19 Patients with Immune Thrombocytopenic Purpura - a Review of the National Database 2019-2020

Introduction: Immune thrombocytopenic purpura (ITP) is a diagnosis of exclusion that is characterized by isolated thrombocytopenia with a generalized purpuric rash. If there is a known case for the ITP, it is referred to as secondary ITP. Secondary ITP may often be caused by drugs, systemic immunologic, or infectious diseases. Covid-19 is a disease caused by the SARS-CoV-2 virus, which caused a pandemic in 2020, affecting hundreds of millions of people worldwide. There have been several different hematologic conditions associated with this viral infection, including deep vein thromboses, pulmonary emboli, and many patients with ITP induced by Covid-19. With several case reports and case series available on these patients, the specifics behind the strength of the association, mortality of the patients, and their comorbidities have not been previously explored. Methods: Using the national inpatient sample from the years 2019-2020, we analyzed adult hospitalized patients with ITP and Covid-19 based on their International Classification of Diseases - 10 clinical modification codes. We calculated the odds ratios for patients with Covid-19 being diagnosed with ITP compared to other hospitalized patients. Additionally, we calculated the odds of several complications, treatments, as well as concurrent conditions in patients with Covid-19 and ITP. The complications measured were epistaxis or hemoptysis, intracranial hemorrhage (ICH), non-intracranial hemorrhage (nICH), deep vein thrombosis (DVT), pulmonary embolism (PE), and death. Treatments measured were transfusion with either red blood cells (RBC) or platelets. The concurrent conditions evaluated were systemic lupus erythematosus (SLE), hepatitis C and heparin-induced thrombocytopenia (HIT). Results Out of the 115,150 hospitalized patients with ITP included in the database, 0.02% of them were diagnosed with Covid-19. The odds of a patient with Covid-19 being diagnosed with ITP were lower than those for other hospitalized patients (OR=0.860 [0.787-0.941], p=0.001). The mean age for patients with ITP diagnosed with Covid-19 was 64.1 compared to 60.9 in patients without Covid-19 (p=0.001). Hospitalized patients with ITP who were also diagnosed with Covid-19 were at a higher risk of developing epistaxis (OR: 1.45 [1.01-2.07], p=0.042), pulmonary embolism (OR: 2.22 [1.35-3.66], p=0.002), and death (OR: 4.39 [3.33-5.80], p<0.001) compared to those without Covid-19. They were not at higher odds of developing a DVT (OR: 1.25 [0.798-1.97], p=0.325), ICH (OR: 0.158 [0.0160-1.56], 0.114) or nICH (OR: 1.03 [0.678-1.55], p=0.905). They were not significantly more or less likely to get transfused with RBC (OR: 0.816 [0.585-1.14], p=0.231) or platelets (OR: 1.14 [0.865-1.53], p=0.337). The ITP patients with concurrent Covid-19 were also not more likely to suffer from SLE (OR: 0.737 [0.453-1.20], p= 0.222), Hepatitis C (OR: 0.760 [0.392-1.47], 0.416), or HIT (OR: 1.16 [0.181-7.49], p=0.872). Conclusion Hospitalized patients with Covid-19 were at a lower risk of being diagnosed with concurrent ITP than other hospitalized Covid-19 patients. Those with ITP who also had Covid 19 had significantly higher odds of complication of bleeding with epistaxis or hemoptysis as well as a higher risk of pulmonary embolism and death. However, they were not at higher risk of hemorrhage elsewhere or DVT. They were not treated with transfusions differently and having SLE, hepatitis C or HIT did not place hospitalized patients with ITP at a higher risk of developing Covid-19.

Systemic lupus erythematosus is associated with lower risk of hepatitis B virus infection: A multivariable Mendelian randomization study in East Asian population.

The relationship between systemic lupus erythematosus (SLE) and hepatitis B virus (HBV) infection is still unclear.We conducted a two-sample Mendelian randomization (MR) analysis using summary statistics from genome-wide association studiesfor SLE and HBV infection in individuals of East Asian ancestry. The inverse-variance weighted (IVW) method, weighted median (WM) method, and MR-Egger method were used to estimate the causal effect of SLE on HBV infection. Additionally, we performed a multivariable MRanalysis adjusting for the effects of body mass indexand rheumatoid arthritis.This MRstudy included a total of 225 106 individuals of East Asian ancestry, comprising 5616 cases and 219 490 controls. The IVW method (OR: 0.79, p = 3.34E-08) and the WM method (OR: 0.79, p = 9.09E-06) revealed a causal relationship between genetically predicted SLE and a low risk of HBV infection. The multivariable MRanalysis still suggested a low risk of HBV infection associated with SLE (OR: 0.83, p = 2.89E-06).Our MRanalysis supports a causal relationship between SLE and a low risk of HBV infection in individuals of East Asian ancestry.

E39 Hepatocellular insufficiency and systemic lupus; what is the relationship?!!

Abstract Introduction Systemic lupus erythematosus (SLE) can be associated with several nonspecific hepatic manifestations, but acute lupus hepatitis remains uncommon and rarely constitutes a revelation mode. Objectives To report a case of systemic lupus presented by hepatocellular insufficiency. Materials and methods The patient was eleven years old when admitted in 2017 for investigation of hepatocellular failure. Physical examination: mucocutaneous jaundice with dark urine without other abnormalities. The biological assessment revealed significant hepatic cytolysis, cholestasis, as well as a biological inflammatory syndrome, polyclonal hypergammaglobulinemia predominant on gamma globulins. Urinary sediment and blood count were normal. Ultrasound and hepatosplenic Doppler showed signs of chronic liver disease: portal hypertension and moderate ascites. Cardiac ultrasound showed pericardial effusion. We carried out an etiological assessment comprising: an exhaustive questioning having eliminated any drug or toxic intake. The serologies for viral hepatitis A, B and C were negative. The search for non-organ-specific anti-tissue antibodies (anti-smooth muscle, anti-mitochondria and anti-LKM1) were negative, as well as a cupric balance. Antinuclear antibody was positive with a titre of 1/1000, anti-DNA was positive (> 866 IU/ml), C3, C4 and CH50 were low, and antiphospholipid antibodies were positive. The diagnosis of systemic lupus erythematosus was made. The patient was on corticosteroid therapy and immunosuppressant. The evolution was marked by the progressive improvement of the hepatic balance sheet. Discussion Although hepatic involvement during SLE is not part of the diagnostic criteria, abnormal liver function tests are frequent, ranging from 23% to 60% depending on the series in the literature. However, SLE-specific liver disease or lupus hepatitis is rare, observed in 1% to3% according to the literature. Our patient was symptomatic with hepatocellular insufficiency. Conclusion Lupus hepatitis is a rare manifestation of SLE and constitutes a rare presenting mode that should not be overlooked. Ethics Associate Professor of pediatrics, department of medicine University Setif-Algeria (pediatric center –Setif-Algeria).

Genotoxicity assessment of root extracts of Paeonia lactiflora Pall

The roots of Paeonia lactiflora Pall., (Paeoniae Radix, PL) are a well-known herbal remedy used to treat fever, rheumatoid arthritis, systemic lupus erythematosus, hepatitis, and gynecological disorders in East Asia. Here we evaluated the genetic toxicity of PL extracts (as a powder [PL-P] and hot-water extract [PL-W]) in accordance with the Organization for Economic Co-operation and Development guidelines. The Ames test revealed that PL-W was not toxic to S. typhimurium strains and E. coli in absence and presence of the S9 metabolic activation system at concentrations up to 5000μg/plate, but PL-P produced a mutagenic response to TA100 in the absence of S9 mix. PL-P was cytotoxic in in vitro chromosomal aberrations (more than a 50% decrease in cell population doubling time), and it increased the frequency of structural and numerical aberrations in absence and presence of S9 mix in a concentration-dependent manner. PL-W was cytotoxic in the in vitro chromosomal aberration tests (more than a 50% decrease in cell population doubling time) only in the absence of S9 mix, and it induced structural aberrations only in the presence of S9 mix. PL-P and PL-W did not produce toxic response during the in vivo micronucleus test after oral administration to ICR mice and did not induce positive results in the in vivo Pig-a gene mutation and comet assays after oral administration to SD rats. Although PL-P showed genotoxic in two in vitro tests, the results from physiologically relevant in vivo Pig-a gene mutation and comet assays illustrated that PL-P and PL-W does not cause genotoxic effects in rodents.

A cross-sectional study of systemic disorders in patients with episcleritis and episcleritis

Episcleritis and scleritis are both inflammatory disorders of the eye and may have underlying systemic disorder precipitating them. Therefore a thorough clinical examination and laboratory investigations are needed to rule them out. Systemic conditions that have been associated include rheumatoid arthritis, Wegener’s granulomatosis, systemic vasculitis, systemic lupus erythematosis, sarcoidosis, spondyloarthropathies, tuberculosis, gout, syphilis, hepatitis B and many others.: To estimate the prevalence of systemic disorders in patients with episcleritis and scleritis visiting ophthalmology OPD. To identify the potential underlying systemic disorders requiring treatment.: This study was conducted in 105 patients who attended ophthalmology outpatient department of a tertiary care hospital over a period of one year and four months. Systemic association of a precipitating disease was noted in 20% of cases and the remaining 80% of cases had no association of an underlying precipitating systemic illness. Tuberculosis was a major precipitating disorder associated with scleritis and episcleritis. Rheumatoid arthritis, systemic lupus erythematosis, Wegener granulomatosis, syphilis, inflammatory bowel disease, sarcoidosis, ankylosing spondylitis and herpes zoster were some of the other systemic associations noted.: Scleritis and episcleritis are common conditions encountered in an ophthalmology out patient department. Their frequent association with an underlying systemic disorder prompts us to assess the systemic status of such patients. Early diagnosis and proper management of such disorders has helped to reduce both systemic and ocular morbidity.

Leveraging cellular immunotherapy at the cutting edge of autoimmune disease R&D

David McCall, Commissioning Editor, BioInsights, talks to Silvio Manfredo-Vieira, Associate Director for Correlative and Translational Studies at the Department of Dermatology, University of PennsylvaniaSilvio Manfredo-Vieira is the Associate Director for Correlative and Translational Studies at the Department of Dermatology of the University of Pennsylvania, where he is responsible for direct translational research necessary to move novel cell therapies developed in the lab into early-phase clinical trials under an academic-industry alliance. He received his PhD at the School of Medicine of Ribeirao Preto, Sao Paulo, Brazil, followed by postdoctoral training at Harvard University and Yale University, where he discovered that gut bacteria translocate to distal tissues and induce autoantibodies typical of lupus and autoimmune hepatitis, indicating a critical role for intestinal microbes in initiating or perpetuating local or systemic autoimmunity.

A rare presentation of systemic lupus erythematosus with lupus hepatitis

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease characterized by multisystem inflammation and the presence of circulating autoantibodies directed against self-antigens. Prevalence of SLE in children and adolescents is 1-6 per 100,000 population. Liver dysfunction occurs in approximately in 50-60% of the patients with SLE.And the incidence of lupus hepatitis in diagnosed SLE patients is 9.3%. We are reporting a case of 11-year-old female child who presented with fever, skin rashes, oral ulcers, irritability and positive antinuclear antibody (ANA) with deranged liver function test (LFT) in the form of elevated transaminases, bilirubin level and altered coagulation profile. Hepatitis serology was negative, with low C3 levels, diagnosed as SLE with lupus hepatitis with lupus nephritis stage IV with psychosis. Clinical improvement along with improvement in terms of laboratory findings was seen on corticosteroids therapy. It is important to differentiate lupus hepatitis from autoimmune hepatitis (AIH) as AIH presents similar to lupus hepatitis, has poor prognosis. While lupus hepatitis being rare cause for mortality in patients with SLE has good response with early intervention with corticosteroids therapy.

Emergency Management of Patients with Immune Thrombocytopenia and Severe Bleeding

BackgroundSevere bleeding is a rare but potentially life-threatening complication of immune thrombocytopenia (ITP). In this study, we describe the management and clinical outcomes of patients with ITP presenting to the emergency room (ER) with thrombocytopenia and severe bleeding over a 9-year period from 3 Canadian tertiary care centers.MethodsPatients with ITP who had severe thrombocytopenia (platelets <20 x109/L) and severe bleeding, and who presented to any of 3 hospital emergency rooms (ER) affiliated with McMaster University in Hamilton, Canada between 2008 and 2016 were included in this retrospective study. We reviewed ER and hospital records and extracted demographic data, platelet counts, and all treatments received until discharge or day 10 of hospital admission. We described all bleeding and thrombotic events. Bleeding was graded from 0 (none) to 2 (severe) at one or more anatomical sites using the ITP Bleeding Score (Page et al, Br J Haematol 2007). Patients with skin or oral mucous membrane bleeding only were excluded. Data from a sample of 41 charts were extracted in duplicate (by SM and NS) to ensure consistency; discrepancies were resolved by the principal investigator (D.M.A.). Data were summarized descriptively as frequencies (percentage), means (standard deviation [SD]), or medians (interquartile range [IQR]). Ethics approval was obtained from the Hamilton Integrated Research Ethics Board. Funding for this study was provided by a grant from the Platelet Disorder Support Association.ResultsThirty-one patients had platelets <20 x109/L with grade 2 bleeding. Mean (SD) age was 56 years (±24) and 16 patients (52%) were female. Twenty-three patients (74%) had primary ITP; others had secondary ITP due to medications (n=2), systemic lupus erythematosus (n=2), malignancy (n=2), hepatitis (n=1), or other infection (n=1). Sixteen patients (52%) had newly-diagnosed ITP; 15 (48%) had known ITP and had previously received a median (IQR) of 3 (3-4) ITP therapies including splenectomy (n=7). Median (IQR) platelet count at presentation to the ER was 4 x109/L (2-8 x109/L).There were 37 ER visits among 31 patients. Sites of grade 2 bleeding at presentation to the ER were: GI (18/37 visits, 49%), epistaxis (10/37 visits, 27%), urinary (6/37 visits, 16%), gynecological (4/37 visits, 11%) and intracranial (4/37 visits, 11%). Patients were given a median (range) of 2 (0-7) treatment modalities in hospital including corticosteroids (30/37 visits, 81%), intravenous immune globulin (25/37 visits, 68%) and platelet transfusions (17/37 visits, 46%). Other treatments were romiplostim (3/37 visits), tranexamic acid (3/37 visits); and emergency splenectomy, recombinant factor VIIa, Rh immune globulin, danazol, azathioprine and mycophenolate (1/37 visits each).Four patients experienced new or recurrent grade 2 bleeding in-hospital, including two deaths: a 76 year-old female with a platelet count of 11 x109/L on warfarin who developed ICH (received 4 treatment modalities); and a 20-year-old male with a platelet count of 8 x109/L and ICH (received 7 treatment modalities). Median (IQR) length of hospital admission was 4 (3-9) days for 29 admissions.ConclusionsSevere bleeding in patients with ITP is a high risk situation that requires prompt intervention. Treatments and outcomes were variable. A standardized approach to the treatment of bleeding emergencies in ITP is needed. DisclosuresArnold:Amgen: Consultancy, Research Funding; Rigel: Consultancy; Dova: Consultancy; Bristol Myers Squibb: Research Funding; UCB: Consultancy; Novartis: Consultancy, Research Funding.

Liver Abnormalities in Systemic Lupus Erythematosus: A Prospective Observational Study.

ObjectivesIn this study, we aimed to examine and analyze liver abnormalities among patients with systemic lupus erythematosus (SLE), including both newly diagnosed patients and those being followed up, as well as the prevalence of lupus hepatitis.MethodsThis was a prospective observational study. Clinical data, liver function tests (LFTs), and the findings from the ultrasonography of the abdomen among the patients were prospectively recorded and evaluated.ResultsOverall, 28 of the total 135 (20.7%) patients had liver abnormalities, including biochemical and those detected via ultrasonography. Ten patients had transaminitis, defined as aspartate aminotransferase (AST) or alanine aminotransferase (ALT) levels >2 times the upper limit of normal (ULN). Nine patients had elevated alkaline phosphatase (ALP) or gamma-glutamyl transferase (GGT) of >2 times ULN. In three patients, transaminitis was due to anti-tubercular therapy (ATT)-induced hepatitis; in seven (5.2%), no specific cause for transaminitis could be identified, and hence they were classified as cases of lupus hepatitis. On comparing clinical features between patients with (n=7) and without lupus hepatitis (n=128), the condition was more prevalent in newly diagnosed SLE patients compared to those who had been on follow-up [six (85.7%) vs. 30 (23.6%), p=0.002]. All seven patients with lupus hepatitis had complete resolution of the transaminitis on follow-ups. However, one patient who had received ATT (isoniazid, rifampicin, ethambutol, and pyrazinamide) died. Ultrasonography showed fatty liver in seven patients and chronic liver disease in one patient.ConclusionIn this study, transaminitis due to lupus hepatitis was seen in newly diagnosed lupus patients and was not associated with disease activity. Before diagnosing lupus hepatitis, drug-induced liver disease has to be ruled out, and if persistent LFT abnormalities are present, further workup is suggested to rule out overlap with primary biliary cirrhosis and/or autoimmune hepatitis.

Liver involvement in patients with systemic lupus erythematosus

Liver involvement in systemic lupus erythematosus is common and in most cases clinical course is asymptomatic, that makes diagnosis difficult. Determination of the cause of the liver involvement is important to select treatment and to evaluate the prognosis of the disease.The aim of the research was to characterize the clinical features of liver involvement in patients with systemic lupus erythematosus and identify the most significant clinical and laboratory parameters for the differential diagnosis of lupus hepatitis.Materials and methods. The study included 313 patients with systemic lupus erythematosus observed in the E.M. Tareev Clinic of Rheumatology, Internal Medicine and Occupational Diseases of I.M. Sechenov First Moscow State Medical University (Sechenov University) in the period from 2001 to 2019. The verification of diagnosis of systemic lupus erythematosus was based on the criteria of the American College of Rheumatology (1997). Patients examination included complete blood count, biochemical and immunological blood tests and an abdominal ultrasonography. In 13 cases hepatic autoantibodies (ASMA, anti-LKM-1, LC-1, SLA-LP, AMA-M2) were analyzed, in 4 – magnetic resonance cholangiopancreatography and in 6 – liver biopsy were made.Results. Liver involvement were represented by an increase of liver enzymes in 58 (18.5%) cases. Chronic viral hepatitis C was diagnosed in 4 (1.3%) patients. Drug-induced hepatitis was found in 17 (5.4%) patients. Autoimmune liver diseases occured in 2 (0.6%) patients. In 2 (0.6%) patients, liver damage was associated with thrombotic microangiopathy (atypical hemolytic uremic syndrome, hereditary thrombophilia). In 15 (4.8%) cases, the most likely diagnosis was NAFLD. Lupus hepatitis was the most likely cause in 18 (5.7%) patients. Differential diagnosis in cases of liver involvement in patients with systemic lupus erythematosus requires assessment of risk factors for various liver diseases, age of the patients, level of liver enzymes, lupus activity, ultrasound signs of liver steatosis and secondary antiphospholipid syndrome.Determining the cause of the liver involvement for the patients with the systemic lupus erythematosus allows establishing better treatment tactic and improvement of the prognosis.

The possibility of using biomedical cell product in the treatment of chronic glomerulonephritis

The basic principles of rational treatment of glomerulonephritis are considered, based on etiology, clinical manifestations and pathomorphological variants of its different forms. Today it has been established that glomerulonephritis can be primary (the etiology is usually unknown) and secondary, when the disease occurs against the background of concomitant pathology (systemic lupus erythematosus, vasculitis, hepatitis, oncological diseases, chronic viral and bacterial infections). The participation of various pathological, genetic and systemic factors in the development of various forms of glomerulonephritis has been shown. The characteristic clinical picture of manifestations of different forms of the disease and the degree of involvement of different renal structures in them are described. Against the background of the severity of the course and prevalence of various forms of glomerulonephritis, it is especially important to develop therapeutic approaches aimed at the full restoration of kidney function and cure of patients. One of such approaches is the use of biomedical cell products based on allogenic mesenchymal multipotent stromal cells and hematopoietic stromal cells. A number of studies have confirmed that the best results can be achieved with the use of cell products in complex therapies with standard treatment methods (use of cytostatics and steroid anti-inflammatory drugs) and alternative drug approaches (combination of monoclonal antibodies and polyenzyme drugs). At the same time, the use of standard and alternative techniques does not lead to a complete recovery of patients, but only transfers the course of the disease from the exacerbation phase to remission. The relevance of further development of biomedical cell products of allogeneic mesenchymal multipotent bone marrow stromal cells is shown, verification of their safety and efficacy in preclinical and clinical studies.

Hepatitis Lupus in Systemic Lupus Erythematosus in Male Patients

Systemic Lupus Erythematosus (SLE) is a complex autoimmune disease characterized by autoantibodies against the cell nucleus and involves many organ systems in the body with unknown etiologies and various clinical manifestations, disease course and prognosis. SLE can be found at all ages, generally appearing at age 9-58 years with a peak at age 28 years. It is more common in women with a ratio of women to men 15: 1 to 22: 1. The highest incidence and prevalence of SLE was found in North America 23.2 / 100.000 population / year and 241 / 100.0000 population. In Indonesia, there has been an increase in visits to SLE patients from 17.9-27.2% in 2015 to 30.3-58% in 2017. One of the manifestations of SLE is hepatitis lupus, which is inflammation of the liver tissue. Lupus hepatitis can occur in 20-50% of patients with SLE. It was reported that a 20-year-old man presented with complaints of pain in the joints of the right and left hands which increased since 1 week. The patient also complained of reddish patches on the face, hair loss and mouth sores. Physical examination revealed anemic eye conjunctiva, malar rash, oral ulcer. During the joint examination, there was tenderness in bilateral MCP and PIP. The abdominal examination revealed hepatomegaly. Investigations revealed anemia, thrombocytopenia, increased liver function. Abdominal ultrasound revealed hepatomegaly. ANA profile examination was positive for anti RNP, anti-sm, and anti- ribosomal protein antibodies. The patient was diagnosed with Systemic Lupus Erythematosus with lupus hepatitis according to the ACR (American College of Rheumatology) criteria in which the patient had 6 criteria. The patient was given therapy with 2x125 mg of intravenous methyl prednisolone for 3 days and hydroxychloroquine 1x200 mg orally and other symptomatic drugs.

Renal pathological analysis using galactose-deficient IgA1-specific monoclonal antibody is a strong tool for differentiation of primary IgA nephropathy from secondary IgA nephropathy

In several cases with IgA nephropathy (IgAN), differential diagnosis is difficult due to the complication with other systemic diseases which can induce secondary IgAN. Recently, we demonstrated that immunostaining with galactose-deficient IgA1-specific monoclonal antibody (KM55 mAb) specifically showed positive in primary IgAN cases. Here, we report four cases which we could make definitive diagnosis by immunohistological analysis using KM55 mAb. The underlying systemic diseases are rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), hepatitis C (HCV) and Crohn’s disease (CD). Renal pathological findings in the four cases revealed mesangial proliferative glomerulonephritis with IgA and C3 deposits. Immunostaining with KM55 mAb was positive for three cases complicated with RA, SLE and CD, respectively. Thus, these three cases were diagnosed as primary IgAN and treated with tonsillectomy and steroid pulse therapy. These three cases finally achieved clinical remission. On the other hand, the case with HCV showed negative for KM55. Finally, we diagnosed as HCV-related nephropathy and successfully treated by antiviral agents. These cases suggested KM55 mAb is a strong tool to differentiate primary IgAN from secondary IgAN.

Rowell's syndrome with lupus hepatitis: A case report from China

Rowell's syndrome with lupus hepatitis: A case report from China

Clinical manifestations of parvoviral infection in Uzbekistan

Parvoviral infection (PV B19) is an important but underinvestigated problem. Parvovirus B19 is being studied for the first time in Uzbekistan, and its clinical manifestations are not well known, thus requiring differential diagnosis with other viral infections and noncommunicable diseases. Available literature reported the possible relation of PV B19 in children to a number of pathological conditions, such as allergies, severe anemia, arthralgia, periarteritis nodosa, systemic lupus erythematosus, myocarditis, and hepatitis.

Good Response of Lupus Hepatitis to Mycophenolate Mofetil and Belimumab as of Lupus Exanthema to Rituximab: A Case Report

A 66-year-old male patient had been primarily treated for cutaneous lupus erythematosus 15 years ago. He received local corticosteroids and following systemic immunosuppressants: methylprednisolone (MP), chloroquine (removed due to retinal haemorrhage), cyclosporine A and azathioprine (both removed due to non-response). Due to polyarthralgia methotrexate as well as golimumab were started. Despite good response, both drugs had to be stopped due to development of hepatitis confirmed by liver biopsy (virus serological tests were negative). Early after onset of a combination treatment with 1 g mycophenolate mofetil (MMF) per day orally and 10 mg/kg belimumab intravenously at weeks 0, 2, 4, followed by every 4 weeks, a marked decrease in liver enzymes could be detected. Due to insufficient response of lupus exanthema, hydroxychloroquine (HCQ) was added. After 9 months of belimumab administration, the biologic therapy was switched to rituximab (1000 mg intravenously at weeks 0 and 2) in order to achieve a better control of cutaneous manifestation. Surprisingly, after the very first infusion a rapid and marked improvement of lupus exanthema was noticed. Such an improvement had not been experienced since 2003. We could confirm an excellent response of systemic lupus erythematosus (SLE)-associated hepatitis as well as arthralgias to MMF and belimumab. Additionally, there was an excellent and prompt response of SLE-associated skin lesions to rituximab and MMF, despite the fact, that these drugs are still not approved for treatment of SLE. Verbal informed consent was obtained from the patient for the presentation of his disease course and images.