ACR Meeting Abstracts

ACR Meeting Abstracts

Abstract Number: 2736

Long-Term Survival in Systemic Necrotizing Vasculitides

Sabine Jardel1, Xavier Puéchal2, Alain Le Quellec3, Matthieu Groh2, Mohamed Hamidou4, Francois Maurier5, Olivier Aumaître6, Achille Aouba7, Thomas Quémeneur8, Jean-François Subra9, Vincent Cottin10, Pascal Godmer11, Patrice Cacoub12, Philippe Delaval13, Anne-Laure Fauchais14, Eric Hachulla15, Delphine Maucort-Boulch16, Loïc Guillevin2 and Jean-Christophe Lega17, 1Lyon 1 University, Lyon, France, 2National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, AP–HP, Université Paris Descartes, Paris, France, 3Montpellier, Montpellier, France, 4Internal Medicine Department, Internal Medicine Department, Nantes University Hospital, Nantes, France, 5Internal Medicine, Sainte-Blandine de Metz Hospital, Metz, France, 6CHU Pitié-Salpêtrière - Department of Internal Medicine 2. Referal center for SLE/APS, Paris, France, 7Department of Internal Medicine, Caen University Hospital, Caen, France, 8Service de néphrologie, médecine interne et vasculaire, Hôpital de Valenciennes, Valenciennes, France, 9Angers, Angers, France, 10Louis Pradel Hospital, Claude Bernard University Lyon 1, Lyon, France, 11Medecine Interne, CH Vannes, Vannes, France, 12Department of Internal Medicine and Clinical Immunology, Groupe Hospitalier Pitié-Salpêtrière, Paris, France, 13Rennes, Rennes, France, 14Internal Medicine, Internal Medicine, Limoges, France, 15CHU Lille, Département de Médecine Interne et Immunologie Clinique, F-59000 Lille, France, Lille, France, 16Biostatistics and bioinformatics, Centre Hospitalier Lyon Sud - Claude Bernard University Lyon 1, Lyon, France, 17Department of Internal and Vascular Medicine, Lyon Sud Hospital, Hospices Civils de Lyon, Lyon, France

Meeting: 2017 ACR/ARHP Annual Meeting

Date of first publication: September 18, 2017

Keywords: ,

Session Information

Date: Tuesday, November 7, 2017

Title: Vasculitis Poster III: Other Vasculitis Syndromes

Session Type: ACR Poster Session C

Session Time: 9:00AM-11:00AM

Background/Purpose:

The aim of the study was to describe the evolution of mortality over recent decades in systemic necrotizing vasculitides, including polyarteritis nodosa (PAN), granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA).

Methods:

The date of diagnosis and the cause of death for patients with PAN, GPA, MPA, and EGPA were analyzed from the French Vasculitis Study Group database. Patients were divided into 5 groups: diagnosis before 1980, 1980-1989, 1990-1999, 2000-2010, and after 2010. The causes of death were classified as either vasculitis, infection, cardiovascular, malignancy, miscellaneous, or unknown.

Results:

Among the 2243 included patients (PAN 16%, GPA 42%, EGPA 23%, MPA 19%), 301 (13%) deaths were reported (Table 1). The 5-year overall survival rate increased from 72.2% (95% confidence interval [CI] 59.7-87.2) for patients diagnosed before 1980, to 94.5% (95% CI 90.4-98.8) after 2010 (p<0.001; Figure 1). The incidence of 5-year mortality between 1980-1989 and the last decade (>2010) decreased from 2.18 to 0.11 per 100 person-years for vasculitis-related deaths (p=0.004), from 1.09 to 0.11 for cardiovascular-related deaths (p=0.03), and from 0.82 to 0.25 (p=0.03) for deaths caused by infection. No death by infection occurred in patients diagnosed after 2010. For deaths related to malignancy, the incidence of 5-year mortality was 0.41 per 100 person-years in 1980-1989, 0.17 in 2000-2009 (p=0.21), and 0.32 after 2010 (p=0.78).

Conclusion:

The results indicate that survival has dramatically improved since the 1980s due to the decrease of vasculitis flares and infections, but that cancer-related mortality remained stable. These results highlight the current targets to improve the overall prognosis.

Table 1 – Baseline characteristic of patients

TOTAL
 (n = 2243)

< 1980
 (n = 51)

1980-1989
 (n = 196)

1990-1999
 (n = 610)

2000-2009
 (n = 986)

³ 2010
 (n = 400)

p-value

Male sex ,%

53.5

52.9

53.1

56.2

52.0

54.3

0.6

Mean age at diagnosis, years ± SD

52.6 ± 16.8

38.6 ± 16.2

48.4 ± 16.3

51.2 ± 16.6

53.8 ± 16.6

55.6 ± 16.6

<0.001

Diagnosis, n  (%)

PAN

358 (16.0)

28 (54.9)

84 (42.8)

118 (19.3)

93 (9.4)

35 (8.8)

<0.001

GPA

938 (41.8)

0 (0)

17 (8.7)

245 (40.2)

493 (50.0)

183 (45.8)

<0.001

EGPA

511 (22.8)

15 (29.4)

64 (32.7)

125 (20.5)

212 (21.5)

95 (23.8)

0.004

MPA

436 (19.4)

8 (15.7)

31 (15.8)

122 (20.0)

188 (19.1)

87 (21.8)

0.48

Mean duration of follow-up, years ± SD

6.2 ± 5.8

9.6 ± 10.1

10.1 ± 9.1

8.2 ± 6.3

5.6 ± 4.0

2.4 ± 1.8

<0.001

Total number of deaths at 5 years
 (%a)

185
 (8.2)

11
 (21.6)

37
 (18.9)

80
 (13.1)

48
 (4.9)

9
 (2.3)

<0.001

Vasculitis
 (%b)

60
 (32.4)

3
 (27.3)

16
 (43.2)

29
 (36.3)

11
 (22.9)

1
 (11.1)

0.18

Infection
 (%b)

34
 (18.4)

1
 (9.1)

6
 (16.2)

18
 (22.5)

9
 (18.8)

0
 (0)

0.57

Cardiovascular
 (%b)

34
 (18.4)

1
 (9.1)

8
 (21.6)

13
 (16.3)

11
 (22.9)

1
 (11.1)

0.79

Malignancy
 (%b)

19
 (10.3)

0
 (0)

3
 (8.1)

7
 (8.8)

6
 (12.5)

3
 (33.3)

0.19

Miscellaneous
 (%b)

19
 (10.3)

0
 (0)

3
 (8.1)

8
 (10.0)

6
 (12.5)

2
 (22.2)

0.54

Unknown
 (%b)

19
 (10.3)

6
 (54.5)

1
 (2.7)

5
 (6.3)

5
 (10.4)

2
 (22.2)

<0.001

EGPA: eosinophilic granulomatosis with polyangiitis, GPA: granulomatosis with polyangiitis, MPA: microscopic polyangiitis, PAN: polyarteritis nodosa, SD: standard deviation.

aPercentage calculated from the total number of patients.

bPercentage calculated from the total number of deaths at 5 years in the same decade.

Disclosure: S. Jardel, None; X. Puéchal, None; A. Le Quellec, None; M. Groh, None; M. Hamidou, None; F. Maurier, None; O. Aumaître, None; A. Aouba, None; T. Quémeneur, None; J. F. Subra, None; V. Cottin, None; P. Godmer, None; P. Cacoub, None; P. Delaval, None; A. L. Fauchais, None; E. Hachulla, None; D. Maucort-Boulch, None; L. Guillevin, Hoffmann-La Roche, Inc., 2; J. C. Lega, None.

To cite this abstract in AMA style:

Jardel S, Puéchal X, Le Quellec A, Groh M, Hamidou M, Maurier F, Aumaître O, Aouba A, Quémeneur T, Subra JF, Cottin V, Godmer P, Cacoub P, Delaval P, Fauchais AL, Hachulla E, Maucort-Boulch D, Guillevin L, Lega JC. Long-Term Survival in Systemic Necrotizing Vasculitides [abstract]. Arthritis Rheumatol. 2017; 69 (suppl 10). https://acrabstracts.org/abstract/long-term-survival-in-systemic-necrotizing-vasculitides/. Accessed .

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