Objective: To assess the clinical features, pathological presentations, treatments and outcomes of lupus nephritis (LN) with anti-neutrophil cytoplasmic antibody (ANCA) positivity.
Design: A case-control study.
Methods: Patients (n=49) were retrospectively included from Jinling Hospital in China if presenting with biopsy-proven ANCA-positive LN between 1985 and 2008. Clinicopathological characteristics and outcomes were analysed and compared with those of a control group (n=1279). We further compared treatment responses and outcomes of ANCA-positive LN patients based on the treatment issued.
Results: The study included 40 women and 9 men (median age 33 years at biopsy): 38 with myeloperoxidase (MPO)-ANCA, 7 with proteinase 3 PR3)-ANCA and 4 with double positivity. ANCA-positive LN patients exhibited higher haematuria, serum creatinine levels and systemic lupus erythematosus disease activity index scores. On pathological evaluation, class IV LN was predominant, accounting for 61.22% of cases. Light microscopy revealed significantly higher activity index and chronicity index scores, including cellular crescents, interstitial inflammation, tubular atrophy and interstitial fibrosis. ANCA-positive LN patients receiving mycophenolate mofetil as induction therapy had a higher remission rate and better renal outcomes than those receiving cyclophosphamide. During follow-up, end-stage renal disease developed in seven (14.29%) ANCA-positive LN patients, all of them were MPO-ANCA positive.
Conclusions: The characteristics of ANCA-positive LN were massive haematuria and advanced renal insufficiency. We observed a higher remission rate and better prognoses when using mycophenolate mofetil than when using cyclophosphamide as induction therapy.
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http://dx.doi.org/10.1136/bmjopen-2016-015668 | DOI Listing |
Mod Rheumatol Case Rep
December 2024
Department of Rheumatology and Clinical Immunology, Wakayama Medical University, Wakayama, Japan.
Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic vasculitis preceded by bronchial asthma or allergic sinusitis and accompanied by peripheral blood eosinophilia. Immunosuppressive drugs, such as cyclophosphamide in addition to high-dose glucocorticoids, are recommended for induction of remission in patients with severe EGPA. Although mepolizumab is widely recognized as remission induction therapy in non-fatal/non-organ disabling or relapsed/refractory EGPA, its efficacy and safety in induction of remission for severe cases have been ambiguous.
View Article and Find Full Text PDFRheumatology (Oxford)
December 2024
Renal Division, Department of Medicine, Peking University First Hospital, Peking University Institute of Nephrology, Key Laboratory of Renal Disease, Ministry of Health of China, Key Laboratory of Chronic Kidney Disease Prevention and Treatment (Peking University), Ministry of Education, State Key Laboratory of Vascular Homeostasis and Remodeling, Peking University, Beijing, China.
Objectives: Podocyte bridging may be a key initial event occurring early in crescent formation. This study aims to probe the underlying mechanism of atypical protein kinase C (aPKC)/protease-activated receptor 3(Par3)/Par6 polarity complexes on podocyte motility and crescent formation during the progression of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV).
Methods: The effects of anti-TNF-α monoclonal antibody (mAb) on the crescent formation, localization and expression of aPKC/Par3/Par6 polarity complexes, and activities of small GTPases (Rho/Rac1/Cdc42) were explored in an AAV mouse model.
Arthritis Rheumatol
December 2024
BC Children's Hospital and BC Children's Hospital Research Institute, Vancouver, BC, Canada.
Objectives: We aimed to study the disease course, outcomes, and predictors of outcome in pediatric-onset anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) affecting the kidneys.
Methods: Patients eligible for this study had a diagnosis of granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), or ANCA positive pauci-immune glomerulonephritis, were ≤ 18 years at diagnosis, had renal disease defined by biopsy or dialysis dependence, and had clinical data at diagnosis and either 12- or 24-months. Ambispective data from the ARChiVE/PedVas Registry was used.
Respir Investig
November 2024
Department of Respiratory Medicine, Tokyo Medical and Dental University, 1-5-45 Yushima, Bunkyo-ku, Tokyo, 113-8519, Japan.
Introduction: Microscopic polyangiitis (MPA) with interstitial lung disease (ILD) has a significant impact on morbidity and mortality. This study evaluated the clinical characteristics, treatment responses, and prognostic factors of patients with ANCA-associated vasculitis with ILD (AAV-ILD) and pulmonary limited vasculitis (PLV).
Patients And Methods: A retrospective chart review of ILD patients positive for MPO-ANCA was conducted from 2008 to 2021.
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