OBJECTIVE: To study the efficacy and safety of mycophenolate mofetil (MMF) versus cyclophosphamide (CTX) in the treatment of children with Henoch-Schönlein purpura nephritis (HSPN) and nephrotic-range proteinuria. METHODS: A prospective clinical trial was conducted in 68 pediatric patients who were admitted to the Department of Nephrology, Children's Hospital Affiliated to Capital Institute of Pediatrics and who were diagnosed with HSPN and nephrotic-range proteinuria from August 2016 to November 2019. The patients were randomly divided into two groups:MMF treatment ( RESULTS: At months 3, 6, and 12 of treatment, there was no significant difference in the complete remission rate and the response rate between the MMF treament and CTX treatment groups ( CONCLUSIONS MMF and CTX have similar efficacy and safety in the treatment of HSPN children with nephrotic-range proteinuria.
Child ; Cyclophosphamide/adverse effects* ; Humans ; Immunosuppressive Agents/adverse effects* ; Mycophenolic Acid/adverse effects* ; Nephritis/drug therapy* ; Prospective Studies ; Proteinuria/etiology* ; Purpura, Schoenlein-Henoch/drug therapy* ; Retrospective Studies

This case report concerns a 22-year-old woman who had been diagnosed with systemic lupus erythematosus (SLE). She had intermittent fever, butterfly erythema, photosensitivity, oral ulcers, and multiple arthralgia in the past seven years, but she did not adhere to regular treatments. The edema of the lower extremities and face aggravated in the recent two weeks, so she was admitted to our Department of Rheumatology and Clinical Immunology. Meanwhile, we found she had severe hypertension, the maximal blood pressure was 170/120 mmHg. The patient had high SLE disease activity (the disease activity index score was as high as 23) with blood involvement, acute renal insufficiency, multiple serous effusion and rash. After one week treatments of intravenous methylprednisolone 80 mg daily and other drugs, her conditions made some extent improvement. However, she suffered sudden epileptic attacks. No positive neuropathological signs were found, and the blood pressure was up to 190/130 mmHg before the onset of the seizures. Her cerebrospinal fluid (CSF) pressure was 330 mmH₂O, the CSF protein level was normal value, and the white blood cell count was 0 cell/mm³, with no signs of infection. Cranial MRI showed vasogenic edema at bilateral parietal, occipito-parietal regions, and centrum ovale. We prescribed drugs of decreasing intracranial pressure, intravenous drugs of decreasing blood pressure and midazolam for sedation, without corticosteroid impulse therapy. She recovered consciousness in the next day, without epilepsy recurrence. We eventually diagnosed it as posterior reversible encephalopathy syndrome (PRES), according to the history, laboratory results, imaging featuresand clinical outcome. PRES is a disorder of reversible subcortical vasogenic brain edema in patients with acute neurological symptoms (eg, seizures, encephalopathy, headache, and visual disturbances). PRES is mainly caused by blood pressure changes or endothelial injury, which lead to breakdown of the blood-brain barrier and subsequent brain edema. Most patients have a favourable prognosis. SLE complicated with PRES is not rare, especially in patients with disease activity, hypertension, lupus nephritis and/or renal insufficiency, and use of cytotoxic drugs, early recognition and appropriate treatment remain important. Brainstem involvement, intracranial hemorrhage, renal insufficiency and high disease activity of lupus are risk factors for poor prognosis.
Female ; Humans ; Lupus Erythematosus, Systemic/complications* ; Lupus Nephritis ; Magnetic Resonance Imaging ; Posterior Leukoencephalopathy Syndrome/etiology* ; Seizures ; Young Adult

Humans ; Nephritis ; etiology ; therapy ; Prognosis ; Purpura, Schoenlein-Henoch ; complications ; therapy ; Sjogren's Syndrome ; etiology ; therapy

Adalimumab ; adverse effects ; therapeutic use ; Antirheumatic Agents ; adverse effects ; therapeutic use ; Arthritis, Rheumatoid ; drug therapy ; immunology ; Asian Continental Ancestry Group ; Autoantibodies ; immunology ; Female ; Humans ; Immunoglobulin A ; immunology ; Middle Aged ; Nephritis ; etiology ; immunology

Infliximab is a chimeric anti-tumor necrosis factor-alpha monoclonal antibody. Infusion related reactions and infection are well known side effects of infliximab; however, renal complications have not been well recognized. We report on a patient with late onset-acute tubulointerstitial nephritis (ATIN) after treatment with infliximab and mesalazine for Crohn's disease. A 25-year-old woman was admitted with a purpuric rash on both lower extremities and arthralgia. She had been diagnosed with Crohn's disease 5.6 years previously and had been treated with mesalazine and infliximab. Serum creatinine level, last measured one year ago, was elevated from 0.6 mg/dL to 1.9 mg/dL. Results of urinalysis, ultrasound, and serologic examinations were normal. With a tentative diagnosis of Henoch-Schonlein purpura, oral prednisolone was given, and serum creatinine decreased to 1.46 mg/dL, but was elevated to 2.6 mg/dL again at two months after discontinuation of prednisolone. Renal biopsy indicated that ATIN was probably induced by drug, considering significant infiltration of eosinophils. Concomitant use of infliximab with mesalazine was supposed to trigger ATIN. Oral prednisolone was administered, and serum creatinine level showed partial recovery. Thus, ATIN should be suspected as a cause of renal impairment in Crohn's disease even after a long period of maintenance treatment with infliximab and mesalazine.
Adalimumab/therapeutic use ; Anti-Inflammatory Agents/therapeutic use ; Creatine/blood ; Crohn Disease/*drug therapy ; Drug Therapy, Combination ; Eosinophils/immunology ; Female ; Humans ; Infliximab/*adverse effects/*therapeutic use ; Kidney/pathology ; Mesalamine/*adverse effects/*therapeutic use ; Nephritis, Interstitial/*diagnosis/drug therapy/*etiology ; Prednisolone/therapeutic use

OBJECTIVETo investigate the levels and functions of CD4(+)CD25(+) regulatory T cells and specific transcription factor Foxp3 and Th17 cells related cytokine in peripheral blood mononuclear cells (PBMC) and renal tissues, and explore their roles in pathogenesis of Henoch-Schonlein purpura nephropathy (HSPN) in children.

METHODFrom March, 2011 to March, 2013, 30 cases of HSPN children underwent renal biopsy and were treated in Guiyang Children's Hospital were enrolled into this study. Ten healthy children who underwent health check up were enrolled as blood sample control group. The normal kidney tissue specimens were taken from 5 children who underwent surgery for urologic disorders were used as renal sample control group. The circulating proportions of CD4(+)CD25(+) regulatory T cells in PBMC of 30 cases of HSPN children and 10 cases of control group were determined by flow cytometry, respectively.Reverse transcription-polymerase chain reaction (RT-PCR) were used to analyze the mRNA expressions of IL-17, IL-1β and Foxp3 in PBMC. The expression of IL-17 and IL-1β in renal tissue of HSPN and control group were measured by immunohistochemistry. CD4(+)CD25(+) regulatory T cells, Foxp3, IL-17, IL-1β expression were analyzed and compared in HSPN group and control groups respectively.

RESULTThirty cases of HSPN pathological classification were as follows: type I was found in 0 case; type II in 9 cases; type III in 16 cases; type IV in 5 cases; type V in 0 case. The circulating proportions of CD4(+)CD25(+)/CD4(+)T cells and the CD4(+)CD25(+)Foxp3(+)Treg/CD4(+)T cells level were (5.84 ± 0.78)%, (1.01 ± 0.46) % in HSPN groups were substantially lower than those in control group. All these two differences had statistical significance (t = 27.200, 33.260, P < 0.05). The mRNA levels of IL-17, IL-1β in HSPN groups (0.86 ± 0.01,0.71 ± 0.01) were higher than those in control group (t = 25.000, 31.840, all P < 0.05). Foxp3 mRNA expression in HSPN groups (0.24 ± 0.02) were significantly lower than those in control group (t = 21.690, P < 0.05). Protein expression of IL-17 and IL-1β in renal tissues of HSPN children (13.31 ± 0.54, 11.56 ± 0.28) were significantly stronger than those in the control group (t = 27.6, 14.0, all P < 0.01). The highest level of protein expression of IL-17 and IL-1β in renal biopsy of HSPN was in type IV (IV>III>II, F = 545.800, 262.500, all P < 0.01).

CONCLUSIONThe disorder of quantity and function of CD4(+)CD25(+) regulatory T cells, and increase in levels of IL-17, IL-1β (cytokine related to Th17 cells) may play important roles in pathogenesis of HSPN in children; increased protein expression of IL-17, IL-1β in renal tissue may contribute to the development of renal pathological damage in HSPN children.

Case-Control Studies ; Child ; Child, Preschool ; Female ; Flow Cytometry ; Forkhead Transcription Factors ; genetics ; metabolism ; Humans ; Interleukin-17 ; genetics ; metabolism ; Interleukin-1beta ; genetics ; metabolism ; Kidney ; metabolism ; pathology ; Male ; Nephritis ; etiology ; immunology ; pathology ; Purpura, Schoenlein-Henoch ; complications ; immunology ; pathology ; RNA, Messenger ; genetics ; metabolism ; Reverse Transcriptase Polymerase Chain Reaction ; Severity of Illness Index ; T-Lymphocytes, Regulatory ; immunology ; Th17 Cells ; immunology

Acute Disease ; Child ; Humans ; Male ; Nephritis ; complications ; Posterior Leukoencephalopathy Syndrome ; etiology

OBJECTIVEAnti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a disorder with poor prognosis. This study aimed to improve the diagnosis and treatment of ANCA associated vasculitis of children, to analyze the clinical features, pathological characteristics and the prognosis of children with ANCA-associated vasculitis.

METHODFifteen children with ANCA associated vasculitis who were hospitalized from 2003 to 2012 in our hospital were included. Their data of pre-diagnosis status, clinical manifestations, renal pathology, treatment and prognosis were reviewed retrospectively.

RESULTOf the 15 children, 11 were girls and 4 boys with a mean age of 10.7 years. Fourteen children were categorized as microscopic polyangitis. The time to diagnosis varied from 0.5 month to 40 months. Hematuria and proteinuria were revealed by urine analysis in all of them, only 6 children complained with gross hematuria or edema of oliguria. Decreased glomerular filtration rate was revealed in 13 children, 8 of whom had a creatinine clearance rate of less than 15 ml/(min·1.73 m(2)). Twelve children underwent renal biopsy, crescent formation was found in 11 children. Most of the crescents were cellular fibrous crescents or fibrous crescents. Six children were diagnosed as crescentic nephritis; the process of rapidly progressive nephritis was only observed in 2 children. Segmental glomerulosclerosis or global glomerulosclerosis were found in 10 children, 3 of them were diagnosed as sclerotic glomerulonephritis. Anemia and pulmonary injury were the most common extra renal manifestations. Other extra renal manifestations included rash, pain joint, gastrointestinal symptoms, abnormal findings of cardiac ultrasonography and headache. Eight children were treated with steroid combined with cyclophosphamide, 4 were treated with steroid and mycophenolate mofetil, 2 were treated with steroid, cyclophosphamide and mycophenolate mofetil, 3 children were treated with plasma exchange. Fourteen children were followed up for 0.5 month to 4 years. The renal function did not recover in children with creatinine clearance rate of less than 30 ml/(min·1.73 m(2)), who showed crescentic glomerulonephritis or sclerotic glomerulonephritis. The children who had creatinine clearance rate of more than 30 ml/(min·1.73 m(2))had better prognosis.

CONCLUSIONMore attention should be paid to ANCA-associated vasculitis among school age girls with anemia or pulmonary diseases. The renal damage was serious in children; however, the clinical manifestations were not obvious. Children with a creatinine clearance rate of less than 30 ml/(min·1.73 m(2)) had poor prognosis. Early accurate diagnosis is very important.

Adolescent ; Anemia ; etiology ; pathology ; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis ; complications ; diagnosis ; pathology ; Antibodies, Antineutrophil Cytoplasmic ; blood ; immunology ; Biopsy ; Child ; Child, Preschool ; Creatinine ; blood ; Female ; Glomerulonephritis ; pathology ; Hematuria ; etiology ; pathology ; Humans ; Kidney ; pathology ; physiopathology ; Kidney Function Tests ; Male ; Nephritis ; diagnosis ; etiology ; pathology ; Prognosis ; Proteinuria ; etiology ; pathology ; Retrospective Studies

Adolescent ; Biological Products ; administration & dosage ; therapeutic use ; Child ; China ; Evidence-Based Medicine ; Glucocorticoids ; adverse effects ; therapeutic use ; Humans ; Immunosuppressive Agents ; administration & dosage ; therapeutic use ; Lupus Erythematosus, Systemic ; complications ; pathology ; therapy ; Lupus Nephritis ; etiology ; pathology ; therapy ; Osteoporosis ; etiology ; pathology ; therapy ; Practice Guidelines as Topic ; standards ; Severity of Illness Index ; Societies, Medical

Child ; Female ; Histiocytic Necrotizing Lymphadenitis ; complications ; Humans ; Lupus Erythematosus, Systemic ; etiology ; Lupus Nephritis ; complications