Poor Renal Outcome of Antineutrophil Cytoplasmic Antibody Negative Pauci-immune Glomerulonephritis in Taiwanese

Hung, Peir-Haur; Chiu, Yen‐Ling; Lin, Wei-Chou; Chiang, Wen‐Chih; Chen, Ming-Fong; Lin, Shih-Chieh; Wu, Kwan‐Dun; Tsai, Tun‐Jun

doi:10.1016/s0929-6646(09)60267-9

Cited by 34 publications

(55 citation statements)

References 18 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Evaluation of larger cohorts from both the European Vasculitis Study Group and the Chapel Hill Group implied that approximately 10 -30% of patients with pauci-immune crescentic glomerulonephritis lack positive ANCAs (21,22). In both the Taiwanese and the Chinese case series, ANCA-negative pauci-immune crescentic glomerulonephritis presented at a much younger age than those with ANCA-positive disease (19,20). For example, ANCA-negative patients were usually much younger than the ANCA-positive patients (mean Ϯ SD age 45.1 Ϯ 13.5 versus 59.1 Ϯ 17.6 years [19] and 39 Ϯ 17 versus 56 Ϯ 14 years [20]).…”

Section: Discussionmentioning

confidence: 94%

“…In both the Taiwanese and the Chinese case series, ANCA-negative pauci-immune crescentic glomerulonephritis presented at a much younger age than those with ANCA-positive disease (19,20). For example, ANCA-negative patients were usually much younger than the ANCA-positive patients (mean Ϯ SD age 45.1 Ϯ 13.5 versus 59.1 Ϯ 17.6 years [19] and 39 Ϯ 17 versus 56 Ϯ 14 years [20]). However, in a study in the UK, no major differences in age at presentation were found between these 2 groups (19).…”

Section: Discussionmentioning

confidence: 94%

“…These differences may reflect variations in disease susceptibilities observed in 2 different ethnic populations. ANCA-negative patients had fewer constitutional symptoms (including weight loss, fevers, myalgias, and arthralgias) and fewer extrarenal manifestations (including respiratory, eye, and ear/nose/throat symptoms) than those with positive ANCAs (19,20). However, ANCA-negative patients had a greater degree of proteinuria (mean Ϯ SD 5.47 Ϯ 3.32 gm/24 hours versus 2.23 Ϯ 2.27 gm/24 hours; P Ͻ 0.001) and a higher prevalence of nephrotic syndrome (46% versus 8.8%; P Ͻ 0.001) than ANCA-positive patients (20).…”

Section: Discussionmentioning

confidence: 96%

“…In a 10-year retrospective study, the annual incidence rate was 3.9 patients per 1 million individuals and approximately 27% of affected patients with pauciimmune crescentic glomerulonephritis were ANCA negative (18). In a Taiwanese and a Chinese case series, 38% and 33% of patients with pauci-immune crescentic glomerulonephritis were ANCA negative, respectively (19,20). However, these studies lacked a unified definition for "crescentic" and "pauci-immune" glomerulonephritis, which limits the comparability of these results.…”

Section: Discussionmentioning

confidence: 99%

“…Limited prevalence data are available on ANCAnegative pauci-immune crescentic glomerulonephritis. A limited number of case series and population-based studies have focused on the clinical spectrum of this disorder (8,(15)(16)(17)(18)(19)(20). In a 10-year retrospective study, the annual incidence rate was 3.9 patients per 1 million individuals and approximately 27% of affected patients with pauciimmune crescentic glomerulonephritis were ANCA negative (18).…”

Section: Discussionmentioning

confidence: 99%

See 4 more Smart Citations

An Unusual Case of Hematuria

Najem

Sfeir

Estrada

et al. 2014

Arthritis Care & Research

View full text Add to dashboard Cite

Section: Discussionmentioning

confidence: 94%

Section: Discussionmentioning

confidence: 94%

Section: Discussionmentioning

confidence: 96%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

See 3 more Smart Citations

An Unusual Case of Hematuria

Najem

Sfeir

Estrada

et al. 2014

Arthritis Care & Research

View full text Add to dashboard Cite

A historical study of American patients with anti-neutrophil cytoplasmic antibody negative pauci-immune glomerulonephritis

et al. 2015

View full text Add to dashboard Cite

Anti-neutrophil cytoplasmic antibodies (ANCA) play an important role in the pathogenesis of ANCA-associated vasculitis. The lack of ANCA antibodies may indicate a variation in clinical presentation and outcomes of this disease. We identified 74 adult patients between 1995 and 2009 with the diagnosis of pauci-immune glomerulonephritis. Demographics, histological features, and treatment outcomes were compared between ANCA-positive and ANCA-negative patients. These factors were correlated with renal function at presentation and follow-up. Of the 74 patients, 57 were ANCA-positive, and 17 were ANCA-negative. Demographics and mean Birmingham Vasculitis Activity Score were similar between ANCA-negative and ANCA-positive patients at presentation. Renal function was significantly worse at presentation in the ANCA-negative patients (eGFR 16.59 vs. 31.89 ml/min/1.73 m(2), p = 0.03). Patients in the ANCA-negative group had a significantly higher interstitial fibrosis score compared to the ANCA-positive group (2.1 vs.1.6, p = 0.04). The median time to remission was shorter in the ANCA-negative patients (51 vs. 78 days, p = 0.01). Long-term renal function and 1-year patient and renal survival were similar between ANCA-negative and ANCA-positive patients. Baseline eGFR, percentage of normal glomeruli, glomerular sclerosis, and tubulointerstitial scarring predicted eGFR at 1 year in both groups similarly. This is the first historical review of American patients with pauci-immune glomerulonephritis, comparing patients with ANCA-negative and ANCA-positive serology. Although ANCA-negative patients present with lower eGFR and more interstitial fibrosis, 1-year and long-term outcomes in both groups are similar.

show abstract

A case of a 6-year-old girl with anti-neutrophil cytoplasmic autoantibody-negative pauci-immune crescentic glomerulonephritis

et al. 2011

View full text Add to dashboard Cite

A 6-year-old girl was admitted to our hospital with proteinuria, hematuria, skin rash and joint pain of the lower limbs. Due to rapid progression of renal insufficiency, hemodialysis and peritoneal dialysis were performed. She was diagnosed with rapidly progressive glomerulonephritis. Kidney biopsy showed severe crescent formation (50% of glomeruli) and no deposition of any immunoglobulins or complements. Serologically, anti-neutrophil cytoplasmic autoantibody (ANCA) was negative not only by ELISA against proteinase-3 and myeloperoxidase-ANCA but also by indirect immunofluorescent assay against cytoplasmic and perinuclear ANCA. Anti-glomerular basement membrane antibody was also negative. In the acute phase, proinflammatory cytokines such as soluble tumor necrosis factor receptor 1 (sTNFR1), soluble interleukin (IL)-2 receptor (sIL2R), IL-6 and chemokine IL-8 were elevated. The patient was diagnosed with ANCA-negative pauci-immune crescentic glomerulonephritis (CrGN). Intensive treatment with methylprednisolone pulse therapy, plasma exchange, and multiple drug therapy including prednisolone and cyclophosphamide resulted in histopathological improvement and complete remission of proteinuria. There was a possibility that sTNFR1, sIL2R, IL-6 and IL-8 might be involved in the initiation and progression of ANCA-negative pauci-immune CrGN, and to remove and suppress these cytokines might be an effective way to treat ANCA-negative pauci-immune CrGN.

show abstract

Poor Renal Outcome of Antineutrophil Cytoplasmic Antibody Negative Pauci-immune Glomerulonephritis in Taiwanese

Abstract: This study illustrates the necessity for pathologic diagnosis of pauci-immune GN despite ANCA negativity. The poor prognosis associated with ANCA negativity in this study may be partly due to delayed diagnosis since these patients frequently lacked systemic involvement.

Cited by 34 publications

References 18 publications

An Unusual Case of Hematuria

An Unusual Case of Hematuria

A historical study of American patients with anti-neutrophil cytoplasmic antibody negative pauci-immune glomerulonephritis

A case of a 6-year-old girl with anti-neutrophil cytoplasmic autoantibody-negative pauci-immune crescentic glomerulonephritis

Contact Info

Product

Resources

About