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• Early diagnosis will further add years to life of patients with vasculitis
  Piero Stratta
  Published on: 13 April 2016

• Published on: 13 April 2016

  Early diagnosis will further add years to life of patients with vasculitis

  • Piero Stratta, Director Chair of Nephrology
  • Other Contributors:
    • Marco Quaglia, Roberta Fenoglio

  Dear Editor,

  In a recent paper dealing with long term outcome of vasculitis, the authors conclude that "despite recent advances in treatment, patients with ANCA-associated vasculitis continue to have an excess mortality, ..." and ..."it is unclear to what extent improved treatments will have an impact on later mortality" [1].
  On the basis of our experience on 101 patients with vasculitis, 94 with histological dia...

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  Dear Editor,

  In a recent paper dealing with long term outcome of vasculitis, the authors conclude that "despite recent advances in treatment, patients with ANCA-associated vasculitis continue to have an excess mortality, ..." and ..."it is unclear to what extent improved treatments will have an impact on later mortality" [1].
  On the basis of our experience on 101 patients with vasculitis, 94 with histological diagnosis ( 15 classic polyarteritis, 29, microscopic polyangiitis, 24 Wegener granulomatosis and 33 pauci-immune necrotizing glomerulonephritis) diagnosed from 1975 to 2002 with a mean follow-up time of 7 years, we would like to comment that, even if still sub-optimal, patients survival form the onset of the disease has considerably improved over time when compared with the general age-and sex-matched population of the same area [2].

  In fact, risk of death decreased by 10% at each year of follow-up after 1975, and Poisson-based multinomial analyses confirmed the highly significant risk during the periods of diagnosis from 1975-1983 (HR 4.381, 95%CI 1.274-15.061) and 1984-1992 (HR 3.462, 95%CI 1.052-11.397), as compared to 1993-2002. In particular, life expectancy of patients young women with vasculitis has undergone a dramatic improvement over time, now approaching that of the sex- and age-matched general population: in fact, relative survival of the cohort of women under age 60 was sharply lower in the first period (relative survival at 10 years = 0.339) and increased in the second (0.929), as it did during the 5 years in the third period, when they experienced a survival of 91.6%, comparable to that of the general population (98.1%), the opposite of that observed for men of all ages, showing a relative survival at 5 years from 0.491 to 0.546. The increasing survival rate is chronologically related to two breakthrough advancements at our center: the availability of the ANCA test (after 1986) allowing earlier diagnosis, and the implementation of combined oral pulse steroid/cyclophosphamide association as a first-line strategy for induction (after 1989).
  Also in our patients the need for dialysis at diagnosis was a significant risk facto for patient mortality (HR 4.055, 95%CI 1.493-11.013). Therefore, beside improved treatment, a crucial point to be stressed in order to obtain further improvement in prognosis is the need for early diagnosis. In our experience, delay in diagnosis is a significant risk factor for a worse prognosis, as mean delay from clinical manifestation to diagnosis for all vasculitis significantly decreased during the three periods (from 283? 260 days to 166?170, and to 100 ?64, p<0.01). With respect to renal function, significant associations with renal survival were the percentage of glomeruli involved by crescents (?2=16.5, p=0.0009) and the percentage of sclerosed glomeruli (?2=9.701, p=0.007), and multivariate Cox analysis showed that risk of renal death decreased by 6% at each year of follow-up after 1975, and increased by 5.7% for each year of patient age after 22 years. In sum, cases diagnosed in the later period and with fewer severe lesions at biopsy progressed better, underscoring the importance of an early diagnosis for prompt treatment.

  Therefore, kidney involvement must be seen as a key feature in planning the intensity of treatment the prevention of end-stage renal disease could be seen as a means not only to reduce disability but also to improve survival.

  References

  1. Flossmann O, Berden A, de Groot K, et al. Long-term patient survival in ANCA-associated vasculitis. Ann Rheum Dis 2011;70(3):488-94.

  2. Stratta P, Marcuccio C, Campo A, Sandri et al. Improvement in relative survival of patients with vasculitis: study of 101 cases compared to the general population. Int J Immunopathol Pharmacol 2008;21(3):631- 42.

  Conflict of Interest:

  None declared

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  Conflict of Interest:
  None declared.

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