Rapidly Progressive Glomerulonephritis

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M J Dillon - One of the best experts on this subject based on the ideXlab platform.

  • retrospective study of plasma exchange in patients with idiopathic Rapidly Progressive Glomerulonephritis and vasculitis
    Archives of Disease in Childhood, 1996
    Co-Authors: A Gianviti, Richard S Trompeter, T M Barratt, M F Lythgoe, M J Dillon
    Abstract:

    A retrospective study of 48 patients was conducted to evaluate the efficacy of plasma exchange in children with idiopathic Rapidly Progressive Glomerulonephritis (IRPGN), and renal or non-renal vasculitis. All patients were followed up at a single centre over a 15 year period. Treatment consisted of corticosteroids and/or cytotoxic agents. Plasma exchange was used in all patients because of severe renal involvement and/or clinical deterioration. One hundred per cent of patients with renal vasculitis who started plasma exchange within one month of disease onset and 58% of cases with IRPGN had significant improvement in renal function. No relapses of vasculitis were observed after treatment with plasma exchange in patients with renal and non-renal vasculitis. The results suggest that plasma exchange associated with immunosuppressive treatment could be of benefit in cases of IRPGN or vasculitis in terms of both renal and extrarenal recovery.

A Gianviti - One of the best experts on this subject based on the ideXlab platform.

  • retrospective study of plasma exchange in patients with idiopathic Rapidly Progressive Glomerulonephritis and vasculitis
    Archives of Disease in Childhood, 1996
    Co-Authors: A Gianviti, Richard S Trompeter, T M Barratt, M F Lythgoe, M J Dillon
    Abstract:

    A retrospective study of 48 patients was conducted to evaluate the efficacy of plasma exchange in children with idiopathic Rapidly Progressive Glomerulonephritis (IRPGN), and renal or non-renal vasculitis. All patients were followed up at a single centre over a 15 year period. Treatment consisted of corticosteroids and/or cytotoxic agents. Plasma exchange was used in all patients because of severe renal involvement and/or clinical deterioration. One hundred per cent of patients with renal vasculitis who started plasma exchange within one month of disease onset and 58% of cases with IRPGN had significant improvement in renal function. No relapses of vasculitis were observed after treatment with plasma exchange in patients with renal and non-renal vasculitis. The results suggest that plasma exchange associated with immunosuppressive treatment could be of benefit in cases of IRPGN or vasculitis in terms of both renal and extrarenal recovery.

David Jayne - One of the best experts on this subject based on the ideXlab platform.

  • plasma exchange for renal vasculitis and idiopathic Rapidly Progressive Glomerulonephritis a meta analysis
    American Journal of Kidney Diseases, 2011
    Co-Authors: Michael Walsh, L. Guillevin, Charles D. Pusey, Fausta Catapano, Wladimir Szpirt, Kristian Thorlund, Annette Bruchfeld, Marion Haubitz, Peter A Merkel, David Jayne
    Abstract:

    Background Plasma exchange may be effective adjunctive treatment for renal vasculitis. We performed a systematic review and meta-analysis of randomized controlled trials of plasma exchange for renal vasculitis. Study Design Systematic review and meta-analysis of articles identified from electronic databases, bibliographies, and studies identified by experts. Data were abstracted in parallel by 2 reviewers. Setting & Population Adults with idiopathic renal vasculitis or Rapidly Progressive Glomerulonephritis. Selection Criteria for Studies Randomized controlled trials that compared standard care with standard care plus adjuvant plasma exchange in adult patients with either renal vasculitis or idiopathic Rapidly Progressive Glomerulonephritis. Intervention Adjuvant plasma exchange. Outcome Composite of end-stage renal disease or death. Results We identified 9 trials including 387 patients. In a fixed-effects model, the pooled RR for end-stage renal disease or death was 0.80 for patients treated with adjunctive plasma exchange compared with standard care alone (95% CI, 0.65-0.99; P = 0.04). No significant heterogeneity was detected (P = 0.5; I2 = 0%). The effect of plasma exchange did not differ significantly across the range of baseline serum creatinine values (P = 0.7) or number of plasma exchange treatments (P = 0.8). The RR for end-stage renal disease was 0.64 (95% CI, 0.47-0.88; P = 0.006), whereas the RR for death alone was 1.01 (95% CI, 0.71-1.4; P = 0.9). Limitations Although the primary result was statistically significant, there is insufficient statistical information to reliably determine whether plasma exchange decreases the composite of end-stage renal disease or death. Conclusions Plasma exchange may decrease the composite end point of end-stage renal disease or death in patients with renal vasculitis. Additional trials are required given the limited data available.

Ronald J Falk - One of the best experts on this subject based on the ideXlab platform.

Eri Muso - One of the best experts on this subject based on the ideXlab platform.

  • age dependent survival in Rapidly Progressive Glomerulonephritis a nationwide questionnaire survey from children to the elderly
    PLOS ONE, 2020
    Co-Authors: Mayumi Takahashikobayashi, Hirofumi Makino, Yoshihiro Arimura, Takashi Wada, Kosaku Nitta, Eri Muso, Joichi Usui, Shuzo Kaneko, Hitoshi Sugiyama, Seiichi Matsuo
    Abstract:

    Background Rapidly Progressive Glomerulonephritis (RPGN) has been known to have a poor prognosis. Although evidence across adult RPGN cases has accumulated over many years, the number of case series in adolescents and young adults has been limited, requiring further studies. Methods A total of 1,766 cases from 1989 to 2007 were included in this nationwide questionnaire survey, led by Intractable (former name, Progressive) Renal Diseases Research, Research on intractable disease, from the Ministry of Health, Labour and Welfare of Japan. To elucidate age-related differences in 2-year patient and renal survival rates, the cases were divided into the following four groups: children (0–18 years), young adults (19–39 years), the middle-aged (40–64 years), and the elderly (over 65 years). Results Of the 1,766 total RPGN cases, antineutrophil cytoplasmic antibody (ANCA)-associated Glomerulonephritis comprised 1,128 cases (63.9% of all RPGN cases), showing a tendency to increase with age. Two-year patient survival for RPGN was 93.9% among children, 92.6% in young adults, 83.2% in the middle-aged, and 68.8% in the elderly. The younger group (children plus young adults) showed a clearly higher survival rate compared to the older group (middle-aged plus elderly) (p<0.05). ANCA-associated Glomerulonephritis also showed similar age-related results with all RPGN cases. The comparison of renal prognosis showed no statistically significant differences both in RPGN and in ANCA-associated GN. Conclusion The present study described the age-dependent characteristics of the classification of RPGN, especially focusing on a better prognosis of the younger group in patient survival both in RPGN and in ANCA-associated GN.

  • evidence based clinical practice guidelines for Rapidly Progressive Glomerulonephritis 2014
    Clinical and Experimental Nephrology, 2016
    Co-Authors: Yoshihiro Arimura, Eri Muso, Shoichi Fujimoto, Midori Hasegawa, Shinya Kaname, Joichi Usui, Toshiko Ihara, Masaki Kobayashi, Mitsuyo Itabashi, Kiyoki Kitagawa
    Abstract:

    The World Health Organization defines Rapidly Progressive Glomerulonephritis (RPGN)/Rapidly Progressive nephritic syndrome as an abrupt or insidious onset of macroscopic hematuria, proteinuria, anemia, and Rapidly progressing renal failure. The Research Committee of Progressive Glomerular Disease of the Ministry of Health, Labor and Welfare of Japan and the Japanese Society of Nephrology defined RPGN as Rapidly progressing renal failure within several weeks to several months that is associated with urinary findings such as proteinuria, hematuria, red blood cell casts, and granular casts indicating Glomerulonephritis. Without treatment, most patients will develop end-stage renal disease. RPGN is one of the clinical syndromes resulting from Glomerulonephritis. In most cases of RPGN, the histopathological diagnosis is necrotizing crescentic Glomerulonephritis (NCGN). NCGN is classified into three types—linear, granular, and paucity-immune pattern—based on immunofluorescence microscopic findings. A linear pattern indicates anti-glomerular basement disease, including in situ immune complex formation disease based on the Chapel Hill consensus criteria (2012). Granular staining is seen in circulating immune complex diseases such as systemic lupus erythematosus and IgA vasculitis. Most cases with the paucity-immune pattern are Glomerulonephritis induced by antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis. Myeloperoxidase (MPO)-specific ANCA-associated vasculitis is more widely known than proteinase 3 ANCA-associated vasculitis in Japan.

  • a nationwide survey of Rapidly Progressive Glomerulonephritis in japan etiology prognosis and treatment diversity
    Clinical and Experimental Nephrology, 2009
    Co-Authors: Akio Koyama, Kunihiro Yamagata, Hirofumi Makino, Yoshihiro Arimura, Takashi Wada, Kosaku Nitta, Hiroshi Nihei, Eri Muso, Yoshio Taguma, Hidekazu Shigematsu
    Abstract:

    Background The etiology, prevalence, and prognosis of Rapidly Progressive Glomerulonephritis (RPGN) including renal vasculitis vary among races and periods.

  • intravenous immunoglobulin ivig therapy in mpo anca related polyangiitis with Rapidly Progressive Glomerulonephritis in japan
    Japanese Journal of Infectious Diseases, 2004
    Co-Authors: Eri Muso, Kunihiro Yamagata, Toshiko Itoihara, Takahiko Ono, Enyu Imai, Akira Akamatsu, Kazuo Suzuki
    Abstract:

    For 30 myeloperoxidase (MPO) antineutrophil cytoplasmic antibody (ANCA) related Rapidly Progressive Glomerulonephritis patients (male 17, female 13, average age of 68 +/- 11.8 years old), intravenous immunoglobulin (IVIg) (400 mg/kg/day) was administered for 5 consecutive days before or along with conventional immunosuppressive therapy in Japan. Twenty patients were treated with IVIg before the start or newly increase of conventional therapy and evaluated the independent effect of this therapy. In these patients, just after IVIg, significant decrease of CRP from 8.61 +/- 5.77 to 5.47 +/- 4.50 mg/dl (P < 0.001) was noted with improvement of elevated serum creatinine in 12 out of 19 patients (63%). In the analysis of the overall outcome of 30 patients, at 3 months after IVIg and following conventional therapy, no patients showed renal death except 4 for whom hemodialysis had been started before IVIg. At 6 months, renal survival rate were 92% (newly renal death 2 out of 26) and 2 patients died due to cerebral bleeding (survival rate was 93%). No fatal infection was noted. IVIg might be the potent inducible therapy which can be promoted before the beginning of conventional immunosuppressant treatment for relatively aged and lower immunopotent MPO-ANCA patients in Japan.