About: Long-term outcome of severe alveolar haemorrhage in ANCA-associated vasculitis: a retrospective cohort study     Goto   Sponge   NotDistinct   Permalink

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  • Objectives: Alveolar haemorrhage (AH) is a major cause of early death in anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). There is a paucity of information regarding the outcomes of AAV patients presenting with severe AH. Method: A retrospective cohort study. Patients with severe AH were identified from a case review of 824 AAV patients. Demography, presenting features, treatment, and outcomes are described. Results: Fifty-three patients (33 males, 20 females; median age 59 years) with severe AH were identified: 37 (69.8%) with granulomatosis with polyangiitis (Wegener's) and 16 with microscopic polyangiitis [36 proteinase 3 (PR3)-ANCA positive and 17 myeloperoxidase (MPO)-ANCA positive]. AH was the first disease manifestation in 46 (86.8%) patients. Assisted ventilation was required in 36 (67.9%), renal involvement was present in 52 (98.1%), and 28 (52.8%) required dialysis. Forty (75.5%) received plasma exchange. At 3 months, 44/53 (83.0%) were alive. The mean follow-up was 49 months when 31 (58.5%) were alive and 24 (45.3%) dialysis independent. Mortality was higher in those requiring dialysis at entry (57.1% vs. 24%, p = 0.02) and in patients aged > 65 years (71.4% vs. 30.8%, p = 0.01), and tended to be higher in those requiring intubation (54.5% vs. 32.2%, p = 0.1). Conclusions: Severe AH was more commonly associated with PR3-ANCA (vs. MPO-ANCA) and strongly correlated with renal vasculitis. Current treatment of severe AH leads to remission but long-term mortality remains high. Concurrent renal failure and older age were associated with higher mortality.
  • Objectives: Alveolar haemorrhage (AH) is a major cause of early death in anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). There is a paucity of information regarding the outcomes of AAV patients presenting with severe AH. Method: A retrospective cohort study. Patients with severe AH were identified from a case review of 824 AAV patients. Demography, presenting features, treatment, and outcomes are described. Results: Fifty-three patients (33 males, 20 females; median age 59 years) with severe AH were identified: 37 (69.8%) with granulomatosis with polyangiitis (Wegener's) and 16 with microscopic polyangiitis [36 proteinase 3 (PR3)-ANCA positive and 17 myeloperoxidase (MPO)-ANCA positive]. AH was the first disease manifestation in 46 (86.8%) patients. Assisted ventilation was required in 36 (67.9%), renal involvement was present in 52 (98.1%), and 28 (52.8%) required dialysis. Forty (75.5%) received plasma exchange. At 3 months, 44/53 (83.0%) were alive. The mean follow-up was 49 months when 31 (58.5%) were alive and 24 (45.3%) dialysis independent. Mortality was higher in those requiring dialysis at entry (57.1% vs. 24%, p = 0.02) and in patients aged > 65 years (71.4% vs. 30.8%, p = 0.01), and tended to be higher in those requiring intubation (54.5% vs. 32.2%, p = 0.1). Conclusions: Severe AH was more commonly associated with PR3-ANCA (vs. MPO-ANCA) and strongly correlated with renal vasculitis. Current treatment of severe AH leads to remission but long-term mortality remains high. Concurrent renal failure and older age were associated with higher mortality. (en)
Title
  • Long-term outcome of severe alveolar haemorrhage in ANCA-associated vasculitis: a retrospective cohort study
  • Long-term outcome of severe alveolar haemorrhage in ANCA-associated vasculitis: a retrospective cohort study (en)
skos:prefLabel
  • Long-term outcome of severe alveolar haemorrhage in ANCA-associated vasculitis: a retrospective cohort study
  • Long-term outcome of severe alveolar haemorrhage in ANCA-associated vasculitis: a retrospective cohort study (en)
skos:notation
  • RIV/00023001:_____/13:00058665!RIV14-MZ0-00023001
http://linked.open...avai/riv/aktivita
http://linked.open...avai/riv/aktivity
  • I, V, Z(MSM0021620806)
http://linked.open...iv/cisloPeriodika
  • 3
http://linked.open...vai/riv/dodaniDat
http://linked.open...aciTvurceVysledku
http://linked.open.../riv/druhVysledku
http://linked.open...iv/duvernostUdaju
http://linked.open...titaPredkladatele
http://linked.open...dnocenehoVysledku
  • 85403
http://linked.open...ai/riv/idVysledku
  • RIV/00023001:_____/13:00058665
http://linked.open...riv/jazykVysledku
http://linked.open.../riv/klicovaSlova
  • wegeners-granulomatosis; microscopic polyangiitis (en)
http://linked.open.../riv/klicoveSlovo
http://linked.open...odStatuVydavatele
  • GB - Spojené království Velké Británie a Severního Irska
http://linked.open...ontrolniKodProRIV
  • [B51C0761E515]
http://linked.open...i/riv/nazevZdroje
  • Scandinavian journal of rheumatology
http://linked.open...in/vavai/riv/obor
http://linked.open...ichTvurcuVysledku
http://linked.open...cetTvurcuVysledku
http://linked.open...UplatneniVysledku
http://linked.open...v/svazekPeriodika
  • 42
http://linked.open...iv/tvurceVysledku
  • Lánská, Věra
  • Tesar, V.
  • Casian, A. L.
  • Frausova, D.
  • Hruskova, Z.
  • Jayne, D. R. W.
  • Konopasek, P.
  • Svobodova, B.
http://linked.open...ain/vavai/riv/wos
  • 000319036800008
http://linked.open...n/vavai/riv/zamer
issn
  • 0300-9742
number of pages
http://bibframe.org/vocab/doi
  • 10.3109/03009742.2012.754939
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