The Beneficial Effect of 3-Month-Induction Therapy with Corticosteroids and Mycophenolate Mofetil Followed by Maintenance Therapy with Yearly Rituximab Infusions as Sole Maintenance Therapy in Cryptogenic Chronic Hypersensitivity Pneumonitis

Background: The available data on cryptogenic chronic hypersensitivity pneumonitis (ccHP) indicate an inherited predisposition to disease with triggering autoimmune phenomena. Hence, we evaluated prospectively the role of a new autoimmune regimen in treatment of its severe and progressive disease. Patients and Methods: A total of 9 patients were included in the study. They had criteria for ccHP viz. 1) clinical features of cryptogenic progressive restrictive lung disease, 2) high-resolution computed tomographic pulmonary abnormalities, and 3) bronchoalveolar lavage lymphocytosis (>30%). The regimen consisted of an initial induction phase of 3-month Solumedrol 1 g IV daily for 3 days followed by 1 month of Prednisone (P) 60 mg/day to tapered down to discontinuation by 3rd month. They also had received Mycophenolate mofetil (MMF) 1 g twice daily for 3 months. This stage was followed by a maintenance phase of yearly Rituximab infusions (1 g followed by 1 g 2 weeks later). Results: compared to their previous 6 months deterioration;all patients showed significant improvement in their forced vital volume, diffusion capacity for carbon monoxide, 6-minutes-walk after the induction phase (at 3 months) which improved further at 15 months with Rituximab therapy. Conclusion: After 3-month induction therapy with P and MMF;yearly R treatment is a safe, practical and effective long-term therapy for ccHP.

Effect of Rituximab Versus Mycophenolate Mofetil or Cyclophosphamide as Control in Lupus Nephritis:A Meta-Analysis

Objective:To evaluate the effects of rituximab versus mycophenolate mofetil or cyclophosphamide as control in lupus nephritis by meta-analysis.Methods:A systematic search was carried out up to January 2022,obtaining 7 studies involving 645 participants with lupus nephritis at the commencement of the investigation;198 of them were treated with rituximab,while 447 were treated with mycophenolate mofetil or cyclophosphamide.We determined the odds ratio(OR)and mean difference(MD)with 95%confidence index(CI)to compare rituximab’s efficacy to that of mycophenolate mofetil or cyclophosphamide as control in lupus nephritis using random-or fixed-effects model by dichotomous or continuous techniques.Results:The rituximab group showed significantly higher complete renal remission rate(OR=2.52;95%CI 1.30-4.91,P=0.006)and total renal remission rates(OR=2.22;95%CI 1.36-3.63,P=0.001)than the control group.However,there was no significant difference in terms of end Systemic Lupus Erythematosus Disease Activity Index(SLEDAI)score(MD-1.16;95%CI-2.88-0.57,P=0.19),proteinuria(MD-0.31;95%CI-0.70-0.09,P=0.013),and serum creatinine(MD 0.01;95%CI-0.04-0.07,P=0.64)between the rituximab group and the control.Conclusion:Rituximab exhibited significantly greater complete renal remission rate and total renal remission rates,with no significant difference in terms of shorter-end SLEDAI,proteinuria,and serum creatinine,compared with the control in individuals with lupus nephritis.

Effects of Mycophenolate Mofetil on Renal Interstitial Fibrosis after Unilateral Ureteral Obstruction in Rats

To investigate the effects of mycophenolate mofetil (MMF) on the process of renal interstitial fibrosis,unilateral ureteral obstruction (UUO) model was established in rats Twenty Sprague-Dawley rats underwent UUO and received vehicle ( n =10) or MMF (20 mg kg -1 d -1 , by daily gastric gavage, n= 10) during a period of 5 days following surgery, and the additional 10 rats were served as sham-operated group The rats were killed 5 days after surgery Immunohistochemistry was performed on renal tissue for proliferating cell nuclear antigen (PCNA), α-smooth muscle actin (α-SMA) and type Ⅰ and Ⅲ collagen (colⅠ, colⅢ) Histological studies were also done by MASSON staining Five days after surgery, proliferating cells in tubules, interstitium as well as interstitial myofibroblast (MyoF) infiltration and interstitial colⅠ, colⅢ deposition were all significantly reduced by MMF treatment MMF also alleviated the histological changes of UUO rats These results suggested that the reduction of interstitial MyoF infiltration may be an important event by which MMF prevents renal injury caused by UUO and MMF could be used to limit the progression of renal fibrosis

Correlation of abnormal histology with endoscopic findings among mycophenolate mofetil treated patients

AIM To describe all abnormal histological findings and their associated endoscopic presentation in patients using mycophenolate mofetil(MMF).METHODS A retrospective review of all individuals prescribed MMF within 6 mo of a colonoscopy or flexible sigmoidoscopy between 07/2009 and 09/2015 was performed within Northwell Health system.Records were analyzed for age,gender,procedure indication,MMF indication,and both gross and microscopic findings.Only reports with abnormal histology were included.RESULTS One hundred and eighty-four procedures from 170 patients were found,of which 39 met inclusion criteria.Fifty-one point three percent were female.MMF was used for solid organ transplant in 71.8%.Diarrhea was the indication for 71.8% of colonoscopies.Fiftynine percent of reports revealed gross and microscopic abnormalities while 41.0% had only microscopic findings.Only 11 patients' reports(28.2%) indicated a specific histopathology of MMF colitis.Among the entire group,only 23.1% of abnormal histology was isolated proximal to the splenic flexure.CONCLUSION Our results demonstrate a high rate of left sided disease and microscopic findings without gross mucosal abnormalities among patients using MMF.Also,a broader definition of MMF-colonopathy may be appropriate,with a majority of our abnormal histology falling outside of the more narrowly defined MMF-colitis category.Given the high frequency of isolated microscopic abnormalities and distal disease,sigmoidoscopy with random biopsies may be an appropriate,less invasive initial endoscopic examination in selected MMF patients.

Protective Effect of Mycophenolate Mofetil(MMF) Against Short-term Acute Rejection of Kidney Transplant

To observe the protective effect of MMF against short term(3 months) acute rejection of renal transplantion. 112 patients undergone renal transplantation were randomly divided into two groups: MMF group (2.0 g/d) and azathioprine group. Patients in both groups received cyclosporine A (CsA) and steroid hormone treatment in the same way. In three months time, 10/60 cases in the MMF treated group showed acute rejection with an acute rejection rate of 16.6%. 22/52 patients of the AZA group had acute rejection with a rejection rate of 41.5%. The difference between the two groups is significant (P<0 01). Side effects manifested in MMF group includereduction of blood white cell count and platelet count (5 cases) and diarrhea (3 cases). They resume recovery after reduction of the dosage of or stoppage of MMF. Both hepatic renal functions are not affected. In AZA group, liver function is damaged in 9 patients. MMF is effective in the prevention or reduction of short term acute rejection of transplants. Its side effects are mild and reversible.

Correlations between serum kidney injury molecule-1,cystatin C and immunosuppressants:A cross-sectional study of renal transplant patients in Bahrain

Renal transplant patients receive several immunosuppressive drug regimens that are potentially nephrotoxic for treatment.Serum creatinine is the standard for monitoring kidney function;however,cystatin C(Cys C)and kidney injury molecule-1(KIM-1)have been found to indicate kidney injury earlier than serum creatinine and provide a better reflection of kidney function.Here,we assessed Cys C and KIM-1 serum levels in renal transplant patients receiving mycophenolate mofetil,tacrolimus,sirolimus,everolimus,or cyclosporine to evaluate kidney function.We used both the Chronic Kidney Disease Epidemiology Collaboration(CKD-EPI)2021 equation,which is based on creatinine and combined creatinine with Cys C,and the CKD-EPI 2012 equation,which is based on Cys C alone,to estimate glomerular filtration rate(GFR).Then,we assessed the association between serum KIM-1 and GFR<90 mL per minute per 1.73 m2.We observed significantly higher serum Cys C levels in patients with the elevated serum creatinine,compared with those with normal serum creatinine.The estimated GFRs based on creatinine were significantly higher than those based on the other equations,while a significant positive correlation was observed among all equations.Serum KIM-1 levels were negatively correlated with the estimated GFRs by the CKD-EPI Cys C and the combined creatinine with Cys C equations.A serum KIM-1 level above 0.71 ng/mL is likely to indicate GFR<90 mL per minute per 1.73 m2.We observed a significant correlation between serum creatinine and Cys C in our renal transplant patients.Therefore,serum KIM-1 may be used to monitor renal function when using potentially nephrotoxic drugs in renal transplants.

Immunoglobulin A glomerulonephropathy:A review

In this editorial,we comment on the article by Meng et al published in the World Journal of Clinical Cases.We comprehensively review immunoglobulin A nephro-pathy(IgAN),including epidemiology,clinical presentation,diagnosis,and management.IgAN,also known as Berger's disease,is the most frequent type of primary glomerulonephritis(GN)globally.It is mostly found among the Asian population.The presentation can be variable,from microscopic hematuria to a rapidly progressive GN.Around 50%of patients present with single or recurring episodes of gross hematuria.An upper respiratory infection and tonsillitis often precede these episodes.Around 30%of patients present microscopic hematuria with or without proteinuria,usually detected on routine examination.The diagnosis relies on having a renal biopsy for pathology and immunofluorescence microscopy.We focus on risk stratification and management of IgAN.We provide a review of all the landmark studies to date.According to the 2021 KDIGO(kidney disease:Improving Global Outcomes)guidelines,patients with non-variant form IgAN are first treated conservatively for three to six months.This approach consists of adequate blood pressure control,reduction of proteinuria with renin-angiotensin system blockade,treatment of dyslipidemia,and lifestyle modifications(weight loss,exercise,smoking cessation,and dietary sodium restrictions).Following three to six months of conservative therapy,patients are further classified as high or low risk for disease progression.High-risk patients have proteinuria≥1 g/d or<1 g/d with significant microscopic hematuria and active inflammation on kidney biopsy.Some experts consider proteinuria≥2 g/d to be very high risk.Patients with high and very high-risk profiles are treated with immunosuppressive therapy.A proteinuria level of<1 g/d and stable/im-proved renal function indicates a good treatment response for patients on immu-nosuppressive therapy.

Reversible Chronic Interstitial Nephritis Induced by Tacrolimus —Tacrolimus Chronic Interstitial Nephritis

Calcineurin inhibitors (CNI) are potent immunosuppressive agents in prophylaxis against graft rejection and autoimmune diseases including primary glomerulopathies. Previous research showed reversible;acute afferent arteriolar vasculopathy and irreversible chronic interstitial fibrosis associated with CNI nephrotoxicity. In this case report we describe a patient, with minimal change disease, that had developed chronic and progressive renal disease while receiving therapeutic dose of Tacrolimus. His serum creatinine had reached 537 umol/L and his nephrotic state worsened. Kidney biopsy showed chronic interstitial nephritis. Tacrolimus was discontinued and he was treated with 1 mg/kg prednisone in addition to Mycophenolate mofetil (MMF) 1 g twice daily. By the 2nd month;serum creatinine returned to normal and by the 3rd month serum albumin too. After 1 month of therapy;the dose of Prednisone was tapered down gradually till 5 mg daily by the end of 3rd month. Moreover, the dose of MMF was reduced to 500 mg X2 by the end of 3rd month. After 2 years of follow up;he remained stable and without relapse of NS or renal failure. In conclusion, reversible renal disease, due to chronic interstitial nephritis can be induced by CNI which is amenable to treatment with Prednisone and MMF.

Prostatic Abscess Due to Idiopathic Granulomatous Disease: A Rare Complication of a Chameleon Disorder —Prostatic Abscess Due to Idiopathic Granulomatous Disease

Background: Prostatic abscesses are usually diagnosed in the setting of bacterial prostatitis. Rarely, they reveal or complicate granulomatous prostatitis (GP). Four cases of idiopathic xanthogranulomatous GP have been described previously and the present case report is the first of typical idiopathic variety. The case: A 60-year-old man presented with urine retention that was associated with pyuria and massively enlarged prostate. Cystoscopy revealed prostatic abscess (PA) that was opened. Urine and prostatic culture were negative for bacteria. Prostatic biopsy revealed multiple non-caseating granulomata surrounded by lymphocytes, plasma cells yet without foamy histiocytes, parasites and vasculitis. Special stains were negative for vasculitis, fungiand acid-fast organisms. The patient was treated with Solumedrol 1 g intravenously daily for 3 days followed by Prednisone 1 mg/kg/day for 1 month followed by gradual tapering till discontinuation by 3rd month. Moreover, he had received Mycophenolate mofetil (MMF) 1 g twice/daily. By the end of 2nd month;he was asymptomatic and without pyuria. Repeat cystourethroscopy and MRI scan of the prostate showed near normal prostate. In Conclusion: Idiopathic GP can present with PA that requires proper drainage and since it is a locally hyperimmune disease with genetic predisposition;MMF therapy will be maintained for a total of 2 years to prevent future disease-relapse.

Effects of mycophenolate mofetil,valsartan and their combined therapy on preventing podocyte loss in early stage of diabetic nephropathy in rats

Background Podocyte has inflammatory role in the development of diabetic nephropathy （DN）. Mycophenolate mofetil （MMF）, an anti-inflammatory agent, can suppress macrophage infiltration and reduce renal injury in streptozotocin-induced diabetic rats. Angiotensin II receptor blocker （ARB）, another renal protecting agent, can decrease podocyte loss in DN. In this study, we detected the expression levels of monocyte chemoattractant protein-1 （MCP-1） and nephrin to evaluate podocyte＇s role in inflammatory reaction in DN, observe and compare the effect of MMF alone and in combination with valsartan, on preventing podocyte loss in streptozotocin （STZ） induced diabetic rats. Methods Diabetic model was constructed in uninephrectomized male Wistar rats by single peritoneal injection of STZ （65 mg/kg）. The successfully induced diabetic rats were randomly divided into four groups： diabetes without treatment group （DM）, valsartan treated group （DMV）, MMF treated group （DMM）, and combined therapy group （DMVM）. Normal rats of the same sibling were chosen as control （NC）. At the end of the 8th week, serum biochemistry, 24-hour urinary protein （LIP） and the ratio of kidney weight/body weight （RWK/B） were measured. The rats were sacrificed for the observation of renal histomorphology through light and electron microscope. Nephrin, desmin and MCP-1 levels were detected by semi-quantitative immunohistochemical assays. Real-time quantitative PCR was used to detect the mRNA levels of nephrin and MCP-1. Results Compared with group NC, serum glucose level, 24-hour LIP and RWK/B in group DM were significantly higher （P〈0.01）, and the nephrin mRNA level in DM group was significantly lower （P〈0.05）. The nephrin mRNA expression levels in group DMV, DMM and DMVM were all higher than that of DM group （P〈0.05） and no significant differences were found among the three treatment groups （P〉0.05）. Treatment with MMF, valsartan or their combination could significantly decrease the 24-hour LIP and RWK/B, and suppress glomerulosclerosis and interstitial fibrotic lesions in diabetic rats. In diabetic rats, the high expressions of desmin and MCP-1 in kidney were suppressed by valsartan, MMF or their combination. Conclusions Podocytes are involved in the inflammatory reaction of diabetic rats. MMF could suppress MCP-1 and desmin expression, enhance nephrin expression, and attenuate proteinuria in diabetic rats. The combined therapy of valsartan and MMF did not show any superiority over monotherapies on renal protection. MMF may have renoprotective effect in early stages of diabetic nephropathy through preventing podocytes loss and anti-inflammatory activity.

Low-dose tacrolimus ameliorates liver inflammation and fibrosis in steroid refractory autoimmune hepatitis

AIM: To determine the eff icacy of tacrolimus on clinical status, histopathological status and biochemical markers in patients with steroid refractory autoimmune hepatitis (AIH). METHODS: Retrospectively, clinical parameters, biochemistry and histology were obtained from patient records. RESULTS: Nine patients [8 females/1 male, median age 32 (range 16-64) years] were identified to have received tacrolimus for a median duration of 18 (12-37) mo. Before initiation of tacrolimus treatment the patients were maintained on a prednisolone dose of 20 mg daily (range 20-80 mg/d), which was tapered to 7.5 (5-12.5) mg/d (P = 0.004). Alanine aminotransferase and immunoglobulin-G concentrations decreased from 154 (100-475) to 47(22-61) U/L (P = 0.007), and from 16 (10-30.2) to 14.5 (8.4-20) g/L (P = 0.032), respectively. All patients showed improvement of the liver inflammatory activity, as determined by the Ishak score (P = 0.016), while the degree of f ibrosis tended to decrease (P = 0.049). CONCLUSION: The use of low dose tacrolimus can lead to biochemical and histologic improvement of inflammation with no progression of the stage of f ibrosis in patients with steroid refractory AIH. Low dose tacrolimus therapy also allows substantial reduction of prednisone dose.

Retrospective study of mycophenolate mofetil treatment in IgA nephropathy with proliferative pathological phenotype

Background Mycophenolate mofetil （MMF） and cyclophosphamide （CTX） are widely used in treating various kidney diseases.However,whether they are effective and which one is better for treating IgA nephropathy patients with proliferative pathological phenotype in renal diseases,such as endocapillary proliferation,cellular crescents,and/or capillary loops fibrinoid necrosis is still unknown.We,therefore,initiated a study to compare the effects of MMF and CTX in treating IgA nephropathy with the above pathological lesions.Methods One hundred and nineteen patients with IgA nephropathy who had at least one of the three aforementioned lesions were enrolled.All patients were treated with prednisone; 48 patients received prednisone only （Pred group）,40 received MMF and prednisone （MMF ＋ Pred group）,and 31 were treated with CTX and prednisone （CTX ＋ Pred group）.The median time of follow-up was 30 months （maximum：96 months）.The primary endpoint was defined as renal survival.The incidence of remission of proteinuria was the secondary endpoint.Results Serum creatinine in all groups declined significantly at different follow-up times （P=0.002）,and the differences among the three groups were significant （P＜0.001）.At 24 months of follow-up,the decline rates were 12.35％,32.95％,and 24.14％ in the Pred,MMF ＋ Pred,and CTX ＋ Pred groups respectively.For urine protein excretion,the decline rates were 49.12％ （Pred）,73.67％ （MMF ＋ Pred）,and 63.53％ （CTX ＋ Pred） respectively at 24 months of follow-up.The differences among the three groups were not significant （P=0.714）.Renal survival （the primary endpoint） was significantly different （P=0.027）; however,the sencondary endpoint was similar for all the three groups （P=0.100）.Conclusions For IgA nephropathy patients with endocapillary proliferation,cellular crescents,and/or fibrinoid necrosis of capillary loops,prednisone combined with MMF was more effective in lowering the serum creatinine than with CTX.Combined MMF and orednisone treatment led to a better renal survival compared to that of prednisone with CTX.

Gastrointestinal complications after kidney transplantation

Gastrointestinal complications are common after renal transplantation,and they have a wide clinical spectrum,varying from diarrhoea to post-transplant inflammatory bowel disease(IBD).Chronic immunosuppression may increase the risk of post-transplant infection and medication-related injury and may also be responsible for IBD in kidney transplant re-cipients despite immunosuppression.Differentiating the various forms of post-transplant colitis is challenging,since most have similar clinical and histological features.Drug-related colitis are the most frequently encountered colitis after kidney transplantation,particularly those related to the chronic use of mycophenolate mofetil,while de novo IBDs are quite rare.This review will explore colitis after kidney transplantation,with a particular focus on different clinical and histological features,attempting to clearly identify the right treatment,thereby improving the final outcome of patients.

Mycophenolate mofetil affects monocyte Toll-like receptor 4 signaling during mouse renal ischemia/reperfusion injury

Background Mycophenolate mofetil （MMF） has been used to prevent transplant rejection for many years and has been shown to have protective effects against renal failure. The objective was to investigate the effect of MMF on monocyte Toll-like receptor 4 （TLR4） signaling in the early stages of renal ischemia/reperfusion injury （IRI） of mice. Methods Sixty BALB/C mice were randomly divided into two groups： an IRI group, in which renal IRI was induced by clamping the renal pedicles for 45 minutes, and an MMF group, in which MMF was given （40 mg.kg-l.d-1, intraperitoneally） from 2 days before renal IRI. The plasma creatinine level and renal tissue damage of each group mice were observed 6, 12, 24, and 48 hours after reperfusion. The concentration of plasma high-mobility group box 1 （HMGB-1） （TLR4 ligand）, interleukin 6 （IL-6）, monocyte chemoattractant protein-1 （MCP-1）, and tumor necrosis factor a （TNF-cc） and the expression of TLR-4 on monocytes were determined. Results The plasma creatinine concentration in the MMF group was lower compared to the IRI group （after reperfusion of 6, 12, 24, or 48 hours, P 〈0.05）. Pathological analysis showed that the renal damage was slighter, TLR-4 expression was reduced （after reperfusion of 6, 12, 24, or 48 hours, P 〈0.05）, and the concentration of cytokines in the plasma was lower （P 〈0.05） in the MMF group. No differences in the concentrations of HMGB-1 were observed （P 〉0.05）. Conclusion Monocyte TLR4 signaling is important in the early stage of kidney IRI, but MMF can inhibit it and improve renal function.

Mizoribine versus mycophenolate mofetil or intravenous cyclophosphamide for induction treatment of active lupus nephritis

Background Lupus nephritis （LN） is one of the most serious manifestations of systemic lupus erythematosus.Although there have been substantial improvements in LN treatment over the last decade,the outcome remains unoptimistic in a considerable percentage of patients.The aim of this study was to evaluate the efficacy and safety of mizoribine （MZR）,a novel selective inhibitor of inosine monophosphate dehydrogenase,as induction treatment for active LN in comparison with mycophenolate mofetil （MMF） and intravenous cyclophosphamide （CYC）.Methods Ninety patients with active LN were observed.Thirty patients were given MZR orally at the dose of 300 mg every other day.Thirty patients took MMF at 2 g per day in two divided doses.Thirty patients received CYC intravenously 0.5 g every 2 weeks.Therapeutic effects and adverse events （AEs） were evaluated at the end of 24-week treatment.Oneway analysis of variance （ANOVA） followed by Dunn＇s test was applied to compare the difference among the groups.For comparing categorical data between two groups,χ^2 test was employed.Results Early responses at week 12 were achieved by 73.3%,90.0%,and 96.7% in MZR,MMF,and CYC groups,respectively.There was no significant difference in the complete remission rates （22.7%,24.0%,and 25.0%,respectively） or overall response rates （68.2%,72.0%,and 75.0%,respectively） among the three groups at week 24.The most prominent drop-down of Systemic Lupus Erythematosus Disease Activity Index scores was observed in MMF or CYC group,and the decline of health assessment questionnaire scores in MZR or MMF group was more prominent than that in the CYC group at week 12.Serum complement 3 （C3） or C4 levels were elevated in all groups after the treatments.CYC was more effective in inhibiting anti-double-stranded DNA antibody,while MZR was more effective in inhibiting antinuclear antibody.The incidences of AEs in patients treated with CYC were significantly higher than those in patients treated with MZR or MMF （24.2% for CYC vs.3.3% for MZR,and 2.6% for MMF,P=0.01）.Conclusions MZR is well tolerated and has an effect similar to MMF in the induction therapy of active LN.MZR may serve as an alternative approach for LN patients.

Acute choroidal involvement in lupus nephritis: A case report and review of literature

BACKGROUND Systemic lupus erythematosus(SLE),characterized by the production of autoantibodies and widespread deposition of immune complexes,predominantly affects women of childbearing age.More than one-third of SLE patients present ocular manifestations.Choroidal disease is currently not completely understood,and its precise differentiation from central serous chorioretinopathy is rarely achieved.To date,no more than 60 patients with choroidal involvement have been reported.CASE SUMMARY A 37-year-old Chinese woman experienced decreased visual acuity bilaterally,accompanied by increasing periorbital swelling and severe conjunctival chemosis.Decreased breath sounds in both bases were detected via auscultation,as well as pitting edema in both ankles.SLE and lupus nephritis were diagnosed based on serositis,renal disorder,leukopenia and positive anti-Smith and anti-nuclear antibodies.Lupus choroidopathy was diagnosed based on ocular presentation and imaging. The patient was treated with systemic corticosteroids, spironolactone, hydroxychloroquine(HCQ), mycophenolate mofetil (MMF), and intravenous immunoglobulin. After 4 wkof hospitalization, the patient was discharged. Indocyanine green angiography showed no leakagefrom choroidal vessels, and ocular coherence tomography detected low amounts of subretinalfluid right before discharge. The patient was prescribed oral methylprednisolone, HCQ, and MMF.Two months after the first visit, ophthalmological examination revealed a visual acuity of 20/20bilaterally, and SLE disease activity was well controlled;her symptoms disappeared completely.CONCLUSIONHere we presented a case of lupus choroidopathy, successfully treated with systemic corticosteroids,and discussed previously reported cases, focusing on differential diagnosis with a centralserous chorioretinopathy.

Mycophenolate mofetil or tacrolimus compared with azathioprine in long-term maintenance treatment for active lupus nephritis

This study aimed to evaluate the efficacy and safety of mycophenolate mofetil(MMF)or tacrolimus(TAC)compared with azathioprine(AZA)as maintenance therapy for active lupus nephritis(ALN).Patients with ALN who responded to 24 weeks of induction treatment were enrolled.Patients who received MMF or TAC as induction therapy continued MMF or TAC treatment during the maintenance period,whereas those who received intravenous cyclophosphamide were subjected to AZA treatment.The primary endpoint was the incidence of renal relapse.Secondary endpoints included extrarenal flares and composite endpoints(deaths,end-stage renal disease,or doubling of serum creatinine levels).A total of 123 ALN patients(47 in the MMF group,37 in the TAC group,and 39 in the AZA group)were enrolled.The median follow-up time was 60 months.Ten MMF-treated patients,ten TAC-treated patients,and eight AZA-treated patients experienced renal relapses(P=0.844).The cumulative renal relapse rates in the MMF group(P=0.934)and TAC group(P=0.673)were similar to the renal relapse rate in the AZA group.No significant difference in the incidence of severe adverse event was observed among the groups.Long-term maintenance therapies with MMF or TAC might have similarly low rates of renal relapse and similar safety profiles compared with AZA.

Systemic lupus erythematosus with visceral varicella:A case report

BACKGROUND As an autoimmune disease,systemic lupus erythaematosus(SLE)can affect multiple systems of the body and is mainly treated by steroids and immunosuppressive agents.SLE results in a long-term immunocompromised state with the potential of infection complications(e.g.,bacterial,fungal and viral infections).Abdominal pain or acute abdomen are frequently the only manifestations of SLE at disease onset or during the early stage of the disease course.Thus,multidisciplinary collaboration is required to identify these patients because timely diagnosis and treatment are crucial for improving their prognosis.CASE SUMMARY Herein,we reported a case of an SLE patient with visceral varicella that was identified after the onset of abdominal pain.The 16-year-old female patient with SLE was admitted to our hospital due to initial attacks of abdominal pain and intermittent fever.The patient’s condition rapidly became aggravated within a short time after admission,with large areas of vesicular rash,severe pneumonia,respiratory failure,shock,and haematologic system and hepatic function impairment.Based on multidisciplinary collaboration,the patient was diagnosed with visceral disseminated varicella and was administered life support,antiviral(acyclovir),immunomodulatory(intravenous injection of human immunoglobulin),anti-infection(vancomycin)and anti-inflammatory(steroid)therapies.After treatment,her clinical symptoms and laboratory indicators gradually improved,and the patient was discharged.CONCLUSION SLE patients long treated with steroids and immunosuppressive agents are susceptible to various infections.Considering that visceral varicella with abdominal pain as the initial presentation is characterized by rapid progression and often coexists with serious complications,prompt diagnosis and early antiviral therapy are critical to prevent severe life-threatening complications.

Three-Month-Induction Therapy with Prednisone and Mycophenolate Followed by Maintenance Therapy with Mycophenolate Alone for 2 Years: An Effective and Safe Autoimmune Treatment for Triggering Factors Adults with Acute Non-Crescentic Nephritis Associated with Henoch-Schönlein Purpura

Background: Henoch-Schönlein purpura (HSP) is an acute systemic disorder characterized by IgA associated vasculitis. The available data indicate an inherited predisposition to disease with triggering autoimmune phenomena. Hence, we evaluated prospectively the role of a new autoimmune regimen in treatment of its severe nephrotic/nephritic flares associated with non-crescentic nephritis in adult patients. Patients and methods: The regimen consisted of an initial induction phase of 3-month Prednisone and Mycophenolate followed by a maintenance phase of Mycophenolate alone for 2 years. Results: They were satisfactory with complete remission in 5 of 7 patients and partial in 2. Creatinine clearance was normalized in patients with complete remission and remained stable in the partially-responsive ones. Conclusion: Our study has shown the short- and long-term safety and efficacy of such autoimmune regimen directed towards the autoimmune triggering factors in severe forms of non-crescentic HSP.