• Budesonide intestinal release formulation
• Children
• Glucocorticosteroids
• IgA nephropathy
• Treatment

• Humans
• Glomerulonephritis, IGA/drug therapy
• Glomerulonephritis, IGA/immunology
• Adult
• Child
• Practice Guidelines as Topic
• Methylprednisolone/therapeutic use
• Glucocorticoids/therapeutic use
• Adrenal Cortex Hormones/therapeutic use
• Treatment Outcome
• Budesonide/therapeutic use

• Università degli Studi di Torino

• IgA nephropathy
• Kidney failure
• Lupus nephritis
• Prognosis
• Systemic lupus erythematosus

• combined nephropathy in hiv-hbv coinfection
• diffuse interstitial lymphocytosis (dil)
• hbv-associated nephropathy (hbvan)
• hepatitis b-associated membranoproliferative glomerulonephritis (mpgn
• hiv associated nephropathy (hivan)

• Corticosteroids
• IgA nephropathy
• Leflunomide
• Meta-analysis
• Proteinuria

• Humans
• Leflunomide/adverse effects
• Glomerulonephritis, IGA/drug therapy
• Glomerulonephritis, IGA/chemically induced
• Immunosuppressive Agents/adverse effects
• Adrenal Cortex Hormones/therapeutic use
• Adrenal Cortex Hormones/pharmacology
• Glomerular Filtration Rate

• APRIL
• BAFF
• IgA nephropathy
• clinical trials
• complement
• endothelin

Optimal supportive therapy with BP and proteinuria control is pivotal in treating patients with IgA nephropathy. Suboptimal treatment of hypertension and proteinuria persisted in many patients with IgA nephropathy in the Cure Glomerulonephropathy Network study. Many patients had above-target proteinuria despite optimal BP control and may benefit from novel therapies or clinical trials.

• ace inhibitors
• iga
• iga deposition
• iga nephropathy
• immunosuppression
• renin angiotensin system

• Adult
• Humans
• Glomerulonephritis, IGA/complications
• Glomerulonephritis, IGA/therapy
• Conservative Treatment
• Glomerulonephritis/complications
• Glomerulonephritis/therapy

• U24 DK100845 NIDDK NIH HHS
• U01DK100866 NIDDK NIH HHS
• U01 DK100867 NIDDK NIH HHS
• U01 DK100866 NIDDK NIH HHS
• U01 DK100846 NIDDK NIH HHS
• U01DK100876 NIDDK NIH HHS
• U01 DK100876 NIDDK NIH HHS
• U01DK100846 NIDDK NIH HHS
• U01DK100867 NIDDK NIH HHS

• Corticosteroids
• Efficacy
• IgA nephropathy
• Safety
• TESTING trial

• Young Adult
• Humans
• Child
• Glomerulonephritis, IGA/diagnosis
• Glomerulonephritis, IGA/drug therapy
• Adrenal Cortex Hormones/adverse effects
• Methylprednisolone/adverse effects
• Clinical Protocols
• Multicenter Studies as Topic

• Humans
• Hematuria
• Glomerulonephritis/diagnosis
• Glomerulonephritis/etiology
• Glomerulonephritis/therapy
• Kidney
• Glomerulonephritis, IGA
• Inflammation

• angiotensin converting enzyme inhibitors (acei)
• angiotensin ii receptor blockers (arb)
• ig a nephropathy
• sle and lupus nephritis
• smoker
• systemic lupus erythromatosus
• systemic steroids

• Episcleritis
• IgA nephropathy
• Ocular manifestations
• Scleritis
• Uveitis

• Endoplasmic reticulum stress
• Glomerular endothelial cells
• IgA nephropathy
• Inflammation
• Podocytes

• Animals
• Humans
• Male
• Mice
• Endothelial Cells/metabolism
• Fatty Acid-Binding Proteins
• Glomerulonephritis, IGA/complications
• Proteinuria/complications
• Vascular Endothelial Growth Factor A/metabolism

• IgA nephropathy
• budesonide
• proteinuria
• renal function
• targeted-release formulation

• Humans
• Budesonide/therapeutic use
• Glomerulonephritis, IGA/drug therapy
• Adrenal Cortex Hormones/therapeutic use
• Glucocorticoids/therapeutic use
• Kidney

• APRIL
• BAFF
• IgA nephropathy
• RNA interference
• clinical trials
• complement
• gut
• monoclonal antibodies

• 24-h urine protein excretion (24-h UP)
• IgA nephropathy
• albumin-to-creatinine ratio (ACR)
• kidney disease progression
• protein-to-creatinine ratio (PCR)

Pozzi protocol (methylprednisolone intravenous infusion in 1st-3rd-5th months and oral steroid for 6 months) has been thought to be the classic therapy for IgA nephropathy (IgAN) patients with proteinuria> 1.0 g/24 h. There is no consensus on the treatments for IgAN with active pathological changes, especially for IgAN patients with crescents proportion < 50%. This study aimed to evaluate the effectiveness and safety of the treatment protocol of methylprednisolone intravenous infusion at the 1st-2nd-3rd months for IgAN patients with crescents.

In this prospective, randomized, controlled, non-blind study, 68 IgAN patients with crescents proportion < 50% were divided into the 1–2-3 group receiving 0.25 g/d methylprednisolone intravenously for 3 consecutive days in the 1st-2nd-3rd months, and oral prednisone 0.5 mg/kg/d on consecutive days for 6 months and the 1–3-5 group with the same intravenous methylprednisolone treatment in the 1st-3rd-5th months, and the same oral prednisone. The primary outcome measure was remission of proteinuria (complete or partial); while the secondary outcome measures were deterioration of renal function (evidenced by a 50% rise from baseline serum creatinine levels, or a 25% decline from baseline eGFR levels).

There was no significant difference in incidence of crescents (median 14.66% vs. 11.45%, p = 0.143) between the 1–2-3 and 1–3-5 groups. From month 1 to month 6, there was a decreasing trend in the levels of urine protein and serum creatinine and an increasing trend in eGFR in both groups. The mean period of remission in the 1–2-3 group seemed shorter. Overall, there were 55 (80.89%) patients meeting remission. The rates of remission in the 1–2-3 group and 1–3-5 group were 85.3 and 76.47%, respectively (P = 0.644). The 1–2-3 group had lower creatinine and higher eGFR than the 1–3-5 group, but the difference was not significant. The complication rate was 11.11 and 15.79% in the two groups, respectively. There was no significant difference in the complications between groups.

Both the 1st-3rd-5th and 1st-2nd-3rd protocols can effectively alleviate proteinuria and protect renal function in IgAN patients with crescents but the 1st-2nd-3rd protocol seemed to have better effectiveness.

ClinicalTrials.gov, Identifier: NCT02160132, Registered June 10, 2014.

• Immunoglobulin A nephropathy
• Pathogenesis
• Treatment

• IgA nephropathy
• autoimmune pathogenesis
• complement activation
• renal inflammation
• targeted treatment strategies

Immunoglobulin A nephropathy (IgAN) is the most common cause of glomerulonephritis worldwide, and the optimal approach to its treatment remains a significant challenge.

We did a prospective, randomized, open-labeled, multicenter, controlled trial, comprised of 3-month run-in, 12-month treatment, and 12-month follow-up phases. After 3-month run-in phase, patients with biopsy-confirmed IgAN at risk of progression were randomly allocated to LEF plus low-dose prednisone (LEF + prednisone group) or conventionally accepted-dose prednisone [prednisone(alone) group] Our primary outcome was 24-h urine protein excretion (UPE) and secondary outcomes were serum albumin (sALB), serum creatinine (Scr), and eGFR. Safety was evaluated in all patients who received the trial medications.

One hundred and eight patients [59 in LEF + prednisone group, 49 in prednisone (alone) group]were enrolled and finished their treatment and follow-up periods. There is no significant difference in the baseline level between the two groups. Compared with baseline, both groups showed a significant decrease in 24-h UPE (p < 0.01) and increase in sALB (p < 0.01), with stable Scr and eGFR throughout the 12-month treatment period. What’s more, these effects were sustained through the 12-month follow-up period. However, there was no difference in 24-h UPE, sALB, Scr, and eGFR between the two groups (p > 0.05). At 12 months, a difference in overall response rate, relapsing rate, and incidence of adverse events between the two groups was not significant.

The efficacy and safety of LEF plus low-dose prednisone and conventionally accepted-dose prednisone in the treatment of progressive IgAN are comparable.

• IgA nephropathy
• disease progression
• glucocorticoids
• leflunomide
• proteinuria

• Conservative therapy
• IgA nephropathy
• Mild proteinuria
• Steroid
• Tonsillectomy

• Adult
• Anti-Inflammatory Agents/administration & dosage
• Anti-Inflammatory Agents/therapeutic use
• Conservative Treatment
• Disease Progression
• Female
• Glomerular Filtration Rate
• Glomerulonephritis, IGA/complications
• Glomerulonephritis, IGA/physiopathology
• Glomerulonephritis, IGA/therapy
• Hematuria/etiology
• Humans
• Male
• Prednisolone/administration & dosage
• Prednisolone/therapeutic use
• Prognosis
• Proteinuria/etiology
• Recoverin
• Remission Induction
• Retrospective Studies
• Time Factors
• Tonsillectomy
• Young Adult

• IgA
• IgA nephropathy
• clinical trials

IgA nephropathy (IgAN) is the most commonly diagnosed primary glomerulonephritis worldwide. It is a slow progressing disease with approximately 30% of cases reaching end-stage kidney disease within 20 years of diagnosis. It is currently only diagnosed by an invasive biopsy and treatment options are limited. However, the current surge in interest in RNA interference is opening up new horizons for the use of this new technology in the field of IgAN management. A greater understanding of the fundamentals of RNA interference offers exciting possibilities both for biomarker discovery and, more importantly, for novel therapeutic approaches to target key pathogenic pathways in IgAN. This review aims to summarise the RNA interference literature in the context of microRNAs and their association with the multifaceted aspects of IgA nephropathy.

• IgA nephropathy
• RNA interference
• microRNA

• IgA nephropathy
• corticosteroids
• mycophenolate mofetil
• renal insufficiency
• retrospective cohort study

• Adrenal Cortex Hormones
• Glomerular Filtration Rate
• Glomerulonephritis, IGA/drug therapy
• Humans
• Immunosuppressive Agents/adverse effects
• Mycophenolic Acid/therapeutic use
• Renal Insufficiency, Chronic/drug therapy
• Retrospective Studies

• efficacy
• high-risk iga nephropathy
• immunosuppressive
• network meta-analysis
• safety

• Adrenal Cortex Hormones/therapeutic use
• Cyclophosphamide/therapeutic use
• Cyclosporine/therapeutic use
• Drug Therapy, Combination
• Glomerular Filtration Rate
• Glomerulonephritis, IGA/drug therapy
• Humans
• Immunosuppressive Agents/administration & dosage
• Immunosuppressive Agents/adverse effects
• Immunosuppressive Agents/therapeutic use
• Mycophenolic Acid/therapeutic use
• Randomized Controlled Trials as Topic

• ZZ13-YQ-031 Fundamental Research Funds for the Central public welfare research institutes

• Autoimmune disease
• Galactose-deficient IgA1
• Glomerulonephritis
• IgA nephropathy
• IgA vasculitis

• Animals
• Glomerulonephritis, IGA/complications
• Glomerulonephritis, IGA/epidemiology
• Glomerulonephritis, IGA/etiology
• Glomerulonephritis, IGA/therapy
• Humans
• Vasculitis/etiology

• R01 DK078244 NIDDK NIH HHS
• R01 DK082753 NIDDK NIH HHS
• R01 GM098539 NIGMS NIH HHS
• R56 DK078244 NIDDK NIH HHS

Background: The efficacy and safety of corticosteroids and immunosuppressive therapy remain controversial for the treatment of immunoglobulin A nephropathy (IgAN). This study aimed to evaluate the effects of corticosteroid and immunosuppressant therapy in Chinese patients with early-stage IgAN whose estimated glomerular filtration rate (eGFR) was ≥45 ml/min/1.73 m² and proteinuria was ≥1 g/24 h at biopsy.

Methods: Patients with biopsy-proven IgAN were retrospectively enrolled from four study centers between 2007 and 2016. Patients were regularly followed up for at least 1 year or until the study end point. Patients were categorized into three treatment groups: supportive care (SC), steroids alone (CS), and steroids plus immunosuppressants (IT). The observed responses to therapy included complete remission (CR), partial remission (PR), no response (NR), and end-stage renal disease (ESRD). The primary end point of the current study was defined as a 50% decline in eGFR and/or ESRD.

Results: A total of 715 patients (male 47% and female 53%) were recruited and followed up for 44.69 ± 24.13 months. The observed CR rate was 81.8% with corticosteroids alone (CS), 62.7% with corticosteroids + immunosuppresants (IT), and 37% with supportive care alone (SC). Renal outcomes were remarkably better in the CS group compared with the SC and IT groups (the percentage of patients reaching the end point in each group was 4.6 vs. 14.4 vs. 11.5%, respectively; p = 0.001). Moreover, 36 and 80-month renal survival were significantly better for the CS group (98.3 and 86.4%) than for the IT (94.2 and 82.4%) and SC (94.0 and 51.6%) groups. Early CKD stage also presented with better kidney survival (p < 0.001). Renal survival of CKD stage 1 patients was relatively good regardless of the specific treatment regimen. CS and IT treatment significantly improved renal survival for CKD stage 2 patients when compared with the SC group (p < 0.001 and 0.007, respectively). However, renal survival of CKD stage 3a patients was not impacted by any of the three treatment regimens. Subgroup analysis also showed that renal survival of patients with proteinuria >3.5 g, M1, E0, S1, T0, and C0 was significantly better in the CS group than in the SC and IT groups. A multivariate model showed that hypertension, serum creatinine, E1 lesion, and T1/T2 lesion remained independent predictors of poor renal survival.

Conclusions: Immunosuppressive therapy does not have further benefit beyond that provided by steroids. Corticosteroids plus optimal supportive care may further be beneficial in treating early-stage IgAN patients in that it could significantly improve the short-term renal outcome.

• Complement system
• IgA nephropathy
• IgA vasculitis
• Lectin pathway
• Rapidly progressive glomerulonephritis

• Abelmoschus manihot
• IgA nephropathy
• NCT 02231125
• Trial registration: ClinicalTrials.gov
• proteinuria
• randomized clinical trial

• Adult
• Biopsy
• Creatinine/blood
• Diagnosis, Differential
• Glomerulonephritis/diagnosis
• Glomerulonephritis, IGA/complications
• Glomerulonephritis, IGA/diagnosis
• Glomerulonephritis, IGA/surgery
• Hematuria/etiology
• Humans
• Hypertension/etiology
• Kidney/diagnostic imaging
• Kidney/pathology
• Kidney Transplantation
• Male
• Renal Insufficiency/etiology
• Ultrasonography

• Galactose-deficient IgA1
• IgA nephropathy
• MEST score
• MEST-C score
• Mesangial deposit
• Oxford classification

• Children
• Histopathology
• IgA nephropathy
• Proteinuria
• Renal biopsy
• Steroid

IgA nephropathy (IgAN) is the most common glomerular disease worldwide. It has a high incidence in Asians and is more likely to progress to end-stage renal disease (ESRD). For high-risk IgAN, which is clinically characterized by massive proteinuria and renal dysfunction, however, there has been no international consensus on treatment options. Compared with other developed countries, IgAN patients in China are often found to have severe kidney function loss at initial diagnosis. Yi-Qi-Qing-Jie formula (YQF; a compound recipe of Chinese medicinal herbs) has shown potential renal protection in our previous clinical studies. To further confirm the efficacy and safety of YQF in the treatment of high-risk IgAN, we have designed a prospective double-blind randomized placebo-controlled trial.

The TCM-WINE study is a single-center, prospective, double-blind randomized placebo-controlled trial. We plan to randomize 60 participants with biopsy-proven IgAN to a YQF combined group (YQF compound combined with prednisolone, and cyclophosphamide if necessary) or an immunosuppression group (placebo-YQF combined with prednisolone, and cyclophosphamide if necessary). The two groups will enter a 48-week in-trial treatment phase and receive post-trial follow-up until study completion (3 years). All patients will receive optimal supportive care. The primary composite outcome is defined as the first occurrence of a 40% decrease in estimated glomerular filtration rate (eGFR) from the baseline lasting for 3 months, initiating continuous renal replacement treatment, or death due to chronic kidney disease (CKD) during the 3-year study phase. The secondary endpoint events are defined as the mean annual eGFR decline rate (eGFR slope, ml/min per 1.73 m² per year), which is calculated by the eGFR regression curve for each eligible patient, and proteinuria remission (prescribed as proteinuria < 0.5 g/day) at weeks 24, 36, and 48 during the in-trial phase. The remission rate of symptoms and inflammation status will be evaluated at week 48. Safety monitoring and assessment will be undertaken during the study.

The TCM-WINE study will evaluate the effects and safety of YQF combined therapy compared with immunosuppression monotherapy on the basis of the optimal supportive treatment in high-risk IgAN. The evidence from this study will provide a novel, effective, and safe Chinese characteristic therapy for high-risk IgAN patients.

ClinicalTrials.gov, NCT03418779. Registered on 18 June 2018.

• High-risk IgAN
• IgA nephropathy
• Immunosuppressive
• Traditional Chinese medicine
• Yi-Qi-Qing-Jie formula

Observational studies suggest that patients with immunoglobulin A nephropathy (IgAN) showed good responses to corticosteroids (CS) but experienced severe adverse effects. The authors conducted a cohort study to evaluate the effectiveness and safety of half-dose CS plus renin-angiotensin system blockers (RASB) (CS + RASB) versus full-dose CS in IgAN patients.

A total of 162 kidney biopsy-confirmed IgAN patients with protein excretion levels ⩾0.75 g/d and an estimated glomerular filtration rate (eGFR) >30 ml/min/1.73 m² were included. A total of 89 patients received half-dose CS + RASB (half CS + RASB), and 73 patients received full-dose CS (full CS). The primary outcomes were the complete remission rates and incidence of adverse events (AEs). The secondary outcomes included 24 h urinary protein (UP) levels and a combined event.

Over the 18 months follow-up, the complete remission rates were 59% (53/89 patients) and 57% (42/73 patients) in the half CS + RASB and full CS groups (p = 0.88), respectively. A total of five patients suffered from serious AEs (SAEs) in the full CS group during the observation period, and no SAEs were observed in the half CS + RASB group (p = 0.012). The incidences of total AEs (p = 0.003) and infections (p = 0.01) were lower in the half CS + RASB group than in the full CS group.

Although half CS + RASB versus full CS did not differ in terms of reducing proteinuria, therapy with half CS + RASB resulted in fewer AEs in the IgAN patients.

• IgA nephropathy
• cohort study
• corticosteroids
• renin-angiotensin system blockers

• Acute kidney injury
• Anticoagulant-related nephropathy
• Dabigatran
• Direct oral anticoagulants
• IgA nephropathy
• Warfarin-related nephropathy

• Acute Kidney Injury/chemically induced
• Acute Kidney Injury/pathology
• Aged
• Anticoagulants/adverse effects
• Anticoagulants/therapeutic use
• Dabigatran/adverse effects
• Dabigatran/therapeutic use
• Female
• Glomerulonephritis, IGA/complications
• Glomerulonephritis, IGA/pathology
• Hematuria/diagnosis
• Hematuria/etiology
• Humans
• Kidney Glomerulus/pathology
• Kidney Glomerulus/ultrastructure
• Venous Thrombosis/drug therapy
• Withholding Treatment

IgA nephropathy is an inflammatory renal disease characterised by the deposition of IgA in the glomerular mesangium and is the most commonly reported primary glomerulonephritis worldwide. Thirty to forty percent of patients with the disease develop progressive renal function decline, requiring renal replacement therapy within two decades of diagnosis. Despite this, accurate individual risk stratification at diagnosis and predicting treatment response remains a challenge. Furthermore, there are currently no disease specific treatments currently licensed to treat the condition due to long standing challenges in the nature and prevalence of the disease. Despite this, there have been exciting recent advances in the field that may represent paradigm shifts in the way IgA nephropathy is managed in the near future. In this review, we explore the evidence base informing current approaches to management and explore new strategies and future directions in the diagnosis and management of IgA nephropathy.

• Berger’s disease
• IgA nephropathy
• Novel treatments
• Repurposed therapy

• IgA nephropathy
• Trientine hydrochloride
• Wilson’s disease
• Zinc acetate

• Chelating Agents/therapeutic use
• Drug Therapy, Combination
• Glomerulonephritis, IGA/diagnosis
• Glomerulonephritis, IGA/etiology
• Hepatolenticular Degeneration/complications
• Hepatolenticular Degeneration/diagnostic imaging
• Hepatolenticular Degeneration/drug therapy
• Humans
• Male
• Tomography, X-Ray Computed
• Trientine/therapeutic use
• Young Adult
• Zinc Acetate/therapeutic use

Granulomatosis with polyangiitis (GPA) is a systemic ANCA-associated vasculitis characterized by necrotizing granulomatous inflammation and a predilection for the upper and lower respiratory tract. Eosinophilic granulomatosis with polyangiitis (EGPA) is also a systemic ANCA-associated vasculitis, but EGPA is characterized by eosinophilic as well as granulomatous inflammation and is more commonly associated with asthma and eosinophilia.

Polyangiitis overlap syndrome is defined as systemic vasculitis that does not fit precisely into a single category of classical vasculitis classification and/or overlaps with more than one category. Several polyangiitis overlap syndromes have been identified, however, there are very few case reports of an overlap syndrome involving both GPA and EGPA in the medical literature.

We conducted a PUBMED literature review using key words ‘granulomatosis with polyangiitis,’ ‘Wegener’s,’ ‘GPA,’ ‘eosinophilic granulomatosis with polyangiitis,’ ‘Churg-Strauss,’ ‘EGPA,’ ‘overlap syndrome,’ ‘Wegener’s with eosinophilia,’ and ‘GPA with eosinophilia’ in English only journals from 1986 to 2017. Relevant case reports and review articles of overlap syndromes of GPA and EGPA were identified. We aim to report a unique case of GPA and EGPA overlap syndrome and review the cases that have been previously described.

Between 1986 and 2017, we identified 15 cases that represent an overlap syndrome with compelling features of both GPA and EGPA. Patients ranged in age between 21 and 78. Of those whose gender was identified, 80 % of the patients were female. All cases described involved the lungs, 60 % reported sinus involvement, and more than 50 % displayed renal involvement. An overwhelming majority of patients were positive for c-ANCA and demonstrated eosinophilia (peripheral blood or tissue eosinophilia). A preponderance of the cases described were treated with systemic corticosteroids combined with an immunosuppressive/cytotoxic agents.

To our knowledge, there have been very few cases reported of an overlap syndrome of GPA and EGPA. Identification of patients with a polyangiitis overlap syndrome of GPA and EGPA is imperative as prognosis, longitudinal management and treatment modalities may differ between these entities.

• Churg-Strauss
• EGPA
• Eosinophilic granulomatosis with polyangiitis
• GPA
• GPA with eosinophilia
• Granulomatosis with polyangiitis
• Overlap syndrome
• Wegener’s
• Wegener’s with eosinophilia

• Chronic kidney disease
• Cost effectiveness analysis
• Disease modeling
• Health economics

• Biomedical Research
• Computer Simulation
• Cost-Benefit Analysis
• Disease Progression
• Glomerular Filtration Rate/physiology
• Humans
• Models, Biological
• Renal Insufficiency, Chronic/complications
• Renal Insufficiency, Chronic/mortality
• Renal Insufficiency, Chronic/physiopathology
• Sensitivity and Specificity

• Kyoto University
• Advanced Science, Technology & Management Research Institute of KYOTO

• Acteoside
• IgA nephropathy
• Th22 Cell
• chemotaxis
• inflammation

• Adult
• Biopsy
• Cell Line
• Cell Proliferation/drug effects
• Chemokine CCL20/immunology
• Chemokine CCL20/metabolism
• Chemokine CCL22/immunology
• Chemokine CCL22/metabolism
• Chemokine CCL27/immunology
• Chemokine CCL27/metabolism
• Chemotaxis/drug effects
• Chemotaxis/immunology
• Coculture Techniques
• Disease Progression
• Drug Therapy, Combination/methods
• Female
• Glomerulonephritis, IGA/drug therapy
• Glomerulonephritis, IGA/immunology
• Glomerulonephritis, IGA/pathology
• Glucosides/pharmacology
• Glucosides/therapeutic use
• Humans
• Immunosuppressive Agents/pharmacology
• Immunosuppressive Agents/therapeutic use
• Male
• Mesangial Cells/drug effects
• Mesangial Cells/immunology
• Mesangial Cells/pathology
• Middle Aged
• Phenols/pharmacology
• Phenols/therapeutic use
• Proteinuria/drug therapy
• Rehmannia/chemistry
• T-Lymphocytes, Helper-Inducer/drug effects
• T-Lymphocytes, Helper-Inducer/immunology
• T-Lymphocytes, Helper-Inducer/metabolism
• Transforming Growth Factor beta1/immunology
• Transforming Growth Factor beta1/metabolism
• Treatment Outcome
• Valsartan/therapeutic use
• Young Adult

The diagnosis of antineutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) is rare in pregnancy but potentially life threatening. There are no randomized controlled trials to guide the management of AAV in pregnancy and fetal safety data remains limited. Rituximab administration, a treatment for AAV, has been reported in pregnant women with reassuring fetal outcomes in the oncology and rheumatology literature; however, no published reports describe its use in AAV.

We present a case of de novo myeloperoxidase positive (MPO) AAV diagnosed at 22 weeks gestation. Clinical presentation included elevated serum creatinine at 177 μmol/L, hematuria and nephrotic range proteinuria along with high-titre MPO. Diagnosis was confirmed by renal biopsy. Patient was treated with methylprednisolone IV followed by oral prednisone 70 mg daily and Rituximab 650 mg IV weekly for four weeks followed by azathioprine maintenance therapy and prednisone taper. Delivery occurred at 29 weeks gestation via cesarean section for maternal neurologic symptoms concerning for preeclampsia. Maternal and fetal CD + 19 cells were depleted at time of delivery with associated fetal lymphopenia in the absence of infection or other complications related to Rituximab use. The patient experienced a reduction in proteinuria and inflammatory markers following Rituximab therapy; however, serum creatinine increased to 375 μmol/L by 11 weeks post-partum.

We report the first use, to our knowledge, of Rituximab with corticosteroids for induction therapy of AAV in pregnancy.

• Antineutrophil cytoplasmic antibody (ANCA)
• Case report
• Myeloperoxidase (MPO)
• Pregnancy
• Rituximab
• Vasculitis

• IgA nephropathy
• Oxford Classification
• mycophenolate mofetil

• IgA nephropathy
• albuminuria
• chronic renal failure
• immunosuppressive drugs
• prognosis