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Saudi Journal of Kidney Diseases and Transplantation
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Year : 2012  |  Volume : 23  |  Issue : 2  |  Page : 301-305
Clinical features and outcomes of ANCA-associated renal vasculitis

1 Nephrology Department, University Hospital Aristide, Le Dantec, Dakar, Senegal
2 Nephrology Department, University Hospital La Conception, Marseille, France

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Date of Web Publication28-Feb-2012


To determine the patterns and outcomes of the pauci-immune vasculitis in the nephrology department at hospital La Conception in Marseille, we conducted a retrospective study including all patients with diagnosis of pauci-immune renal vasculitis between January 1, 2000 and December 31, 2007. Among 33 cases, 25 were diagnosed as Wegener granulomatosis (WG), seven as microscopic polyangitis (MPA) and one as Churg-Strauss syndrome (SCS). The median age of the patients was 57.7 years and the sex-ratio (M/F) was 1.6. The visceral mani­festations included kidneys (100% of patients), lungs (75%), ENT (52% of WG), and nervous system (57% of MPA). The mean serum creatinine at admission was 3.3 mg/dL. Renal biopsies revealed a pauci-immune crescentic gromerulonephritis in 96% of the cases. Two patients with WG received plasmapheresis and seven patients required emergency hemodialysis. Induction therapy comprised cyclophosphamide IV and corticosteroids, while maintenance therapy included azathioprine for the majority of patients. Eighty four percent of the patients experienced complete remission after induction therapy. During maintenance therapy relapses were more frequent among patients with MPA (28%) compared to WG cases (12%). After 35 months of follow-up, eight patients ended on chronic hemodialysis, and five patients died. ANCA associated vasculitis are frequent in our patients. Long-term outcomes are relatively good despite a mortality rate of 15% and 25% of the patients entering dialysis after three years of follow-up.

How to cite this article:
Seck SM, Dussol B, Brunet P, Burtey S. Clinical features and outcomes of ANCA-associated renal vasculitis. Saudi J Kidney Dis Transpl 2012;23:301-5

How to cite this URL:
Seck SM, Dussol B, Brunet P, Burtey S. Clinical features and outcomes of ANCA-associated renal vasculitis. Saudi J Kidney Dis Transpl [serial online] 2012 [cited 2021 Sep 28];23:301-5. Available from: https://www.sjkdt.org/text.asp?2012/23/2/301/93162

   Introduction Top

Pauci-immune vasculitis associated with anti-neutrophil cytoplasmic antibodies (ANCA) are characterized by inflammation of small vessels (arterioles, venules and capillaries). [1] They comprise many diseases such as Wegener granulo-matosis (WG), microscopic polyangitis (MPA), Churg and Strauss syndrome (CSS), and renal limited vasculitis. Kidney injury is frequent in ANCA vasculitis and represents a strong prog­nosis factor. [2],[3]

The aim of this study was to determine the clinical and laboratory patterns and outcomes of patients with ANCA associated vasculitis at a referral center in France.

   Patients and Methods Top

We conducted a retrospective observational study between January 1 and December 31, 2007 at the nephrology department in university hospital La Conception in Marseille, France. All patients with diagnosis of ANCA vascu-litis and followed during this period were included. WG and SCS were diagnosed accor­ding to the ACR criteria. [4] Chapel Hill classi­fication was used for diagnosis of MPA. [1] Severity of vasculitis was assessed with the Birmingham Vasculitis Assessment Score (BVAS) [5] and Five Factor Score (FFS). [6] Socio-demographic, clinical, and laboratory data were collected from medical records. Statistical ana­lysis was done with Microsoft Excel 2007.

   Results Top

Among 40 cases of ANCA vasculitis, we excluded seven patients because of incomplete data. Thirty-three patients were effectively in­cluded in the study and comprised 25 cases with Wegener granulomatosis (WG), seven with microscopic polyangitis (MPA) and one with Churg-Strauss syndrome (SCS). The me­dian age of the patients was 57.7 years and the sex ratio (M/F) was 1.6. Prevalence of ANCA-associated vasculitis during the study period was 0.5 %. Pulmonary-renal syndrome was the most frequent cause of admission (62% of pa­tients). The mean duration between the onset and diagnosis of vasculitis was 14 ± 3.2 weeks (01 - 42 weeks). Clinical and biological cha­racteristics of patients are presented in [Table 1], [Table 2] and.
Table 1: Clinical manifestations of ANCA-associated vasculitis including Wegener granulomatosis
(WG) microscopic polyangitis (MPA), and Churg– Strauss syndrome (CSS).

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Table 2: Biological parameters at admission of patients.

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Renal biopsies were available in 28 cases and revealed pauci-immune crescentic glomerulo-nephritis in 96% of them. Tubulo-interstitial lesions were present in 46% of the cases. Histology did not reveal any granulomas even in WG. The mean BVAS and FFS prognosis scores were 41.7 (20-52), 1.78 (1-3) and 0, for patients with WG, MPA, and CSS, respectively.

Induction therapy consisted of corticosteroids (CTC) 1 mg/kg/day and 0.6 g/m [2] of IV cyclo-phosphamide (CYC) adapted to creatinine clea­rance of the patients. CTC was taken daily at and CYC was administered monthly for six months. After that CYC pulses were given every three months until one year; in two patients with WG, CYC was administrated by oral route at 2 mg/kg/day. Four cases of severe MPA, re­ceived three consecutive pulses of CTC (10 mg/kg/day) during the induction therapy. Two patients with WG and intra-alveolar hemor­rhage underwent plasmapheresis (five sessions) and seven patients required acute emergency hemodialysis. For maintenance therapy, oral CTC (5-10 mg/day) was used in combination with azathioprine (100-150 mg/day) in 93.8% of the WG and MPA cases. Methotrexate (20 mg/week) replaced azathioprine (AZA) in two patients. The mean duration of maintenance therapy was 10.6 ± 6 months (eight to -25 months). The case of CSS was treated with CTC (1 mg/kg/day) during four weeks rapidly tapered to maintenance dose of 10 mg/day and then continued for 12 months.

After induction therapy, the remission rate was 80% and 86% for patients with WG and MPA, respectively. During the maintenance period, relapses occurred in 12% of MPA and 28% of the WG cases. Two cases of resistant WG were treated with rituximab (four injec­tions of 375 mg/m [2] ) and stabilized their renal function. Evolution of patients' serum crea-tinine levels according to type of vasculitis is represented in [Figure 1].
Figure 1: Evolution of serum creatinine during follow-up in our patients with the different vasculitis
including Wegener granulomatosis (WG) microscopic polyangitis (MPA), and Churg-Strauss syndrome (CSS).

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The major complications included azathio-prine-induced bone marrow toxicity (two cases), intercostal herpes zoster (one case) and severe sepsis (eight cases). The number of relapses was not significantly correlated with occur­rence of end-stage renal disease (ESRD) (r=0.46,P=0.15). However, the presence of severe tubulo-interstitial injuries was more frequent in the dialysis-dependent patients. After a follow-up period of 35 months, eight patients with ESRD entered chronic hemodialysis with­out any relapse, and two patients were trans­planted after 17 and 25 months; the grafts func­tion was normal. Five patients died because of septicemia during the induction therapy.

   Discussion Top

Prevalence of ANCA vasculitis in our study was lower than that reported in the general population. [3],[7],[8] This discrepancy relates to the fact that only patients with severe renal injury were included in our study. Most of the pa­tients with ANCA-associated vasculitis were followed by specialists other than nephrolo-gists. Compared to other studies, our patients were younger but male predominance was similar. [7] The clinical presentation was domi­nated by pulmonary-renal syndrome and ENT symptoms as reported by other authors. [3] Intes­tinal vasculitis found in two cases was asso­ciated with severe forms of vasculitis. [9] The immunological findings were very suggestive of the diagnosis in the majority of patients re­vealing anti-PR3 ANCA and anti-MPO ANCA, usually described in WG and MPA, respec-tively. [3],[7] However, atypical ANCA are not rare and seem to be associated with a better prog-nosis. [10] Renal biopsies in ANCA-associated vasculitis classically show crescentic glomerulonephritis as found in the majority of our pa-tients. [8],[15] The tubulo-interstitial lesions were predominantly observed in WG and could ex­plain largely the worse renal outcomes in these patients. [11]

The combination of CTC and CYC at induc­tion phase followed by azathioprine was the most used therapeutic regimen and associated with high remission rate (80%) in our patients. This regimen is widely used in many cen-ters. [3],[8],[12],[13] CYC pulses are as efficient as oral route and well tolerated. [12] Methotrexate is equivalent to AZA for maintenance therapy but adverse effects are not lower. [14] Its pres­cription should be restricted to patients with localized form of WG and during maintenance period. [13],[14] Plasma exchanges are currently recommended in patients with severe renal damages (serum creatinine ≥ 600 μmol/L). [13] Monoclonal anti-CD20 antibodies improved renal function in two cases of WG resistant to the usual drug therapy. Rituximab-induced remission was also reported in small-sized studies of patients with ANCA vasculitis resistant to CYC, but more randomized controlled trials are needed. [16] The remission rate and long-term survival in our study were lower than usually reported in other studies. [17],[18],[19] Twenty of our patients became dialysis inde­pendent after a 35-months follow-up. These bad outcomes are linked to bad prognosis scores (BVAS and FFS) in our patients, less aggressive induction therapy, and short du­ration of treatment in some of them. A mini­mal maintenance period of 18 months is gene­rally required. [13] Mycophenolate mophetil may be safe and efficient for maintenance the-rapy. [19] Better prognosis in patients with MPA and SCS compared to WG was already noticed in previous studies. [8],[17],[19]

After remission, monitoring of ANCA titers was used for early detection of relapses. How­ever, the predictive value of ANCA antibodies is not well demonstrated and increased level should not justify escalation of therapy. [8],[20] High mortality rate in our study was compatible with previous studies which reported an im­portant number of deaths during first year of treatment. [18],[21],[22] In a large retrospective study by Slot et al, dialysis need at diagnosis was identified as a major risk factor of death during the first year. [23] However, the first year mor­tality rate in our patients was much lower than in other studies. [19],[23]

   References Top

1.Jennette JC, Falk RJ, Andrassy K, et al. Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum 1994;37:187-92.  Back to cited text no. 1
2.Gayraud M, Guillevin L, Le Toumelin P, et al. Long-term follow-up of polyarteritis nodosa, microscopic polyangitis and Churg-Strauss syndrome: Analysis of four prospective trials including 278 patients. Arthritis Rheum 2001; 44:666-75.  Back to cited text no. 2
3.Pagnoux C. Wegener's granulomatosis and microscopic polyangitis. Rev Prat 2008;58(5): 522-32.  Back to cited text no. 3
4.Leavitt RY, Fauci AS, Bloch DA, et al. The American College of Rheumatology 1990 criteria for the classification of Wegener's granulo-matosis. Arthritis Rheum 1990;33(8): 1101-7.  Back to cited text no. 4
5.Luqmani RA, Bacon PA, Moots RJ, et al. Birmingham Vasculitis Activity Score (BVAS) in systemic necrotizing vasculitis. Q J Med 1994;87:671-8.  Back to cited text no. 5
6.Guillevin L, Lhote F, Gayraud M, et al. Prog­nostic factors in polyarteritis nodosa and Churg-Strauss syndrome. A prospective study in 342 patients. Medicine (Baltimore) 1996;75(1):17-28.  Back to cited text no. 6
7.Watts RA, Scott DG. Epidemiology of the vas-culitides. Semin Respir Crit Care Med 2004; 25:455-64.  Back to cited text no. 7
8.Puéchal X. Antineutrophil cytoplasmic antibody-associated vasculitis. Rev Rhum 2007;74:824-32.  Back to cited text no. 8
9.Pagnoux C, Mahr A, Guillevin L. Abdominal and digestive manifestations in systemic vasculitides. Ann Med Intern 2003;154(7):457-67.  Back to cited text no. 9
10.Drooger JC, Dees A, Swaak AJ. ANCA-positive patients: the influence of PR3 and MPO Anti­bodies on survival rate and the association with clinical and laboratory characteristics. Open Rheumatol J 2009;3:14-7.  Back to cited text no. 10
11.Son D, Kanda H, Yamaguchi A, et al. Myelo-peroxidase antineutrophil cytoplasmic antibody-associated vasculitis with diffuse tubulointers-titial nephritis. J Nephrol 2009;22(3):417-20.  Back to cited text no. 11
12.de Groot K, Harper L, Jayne DR, et al. Pulse versus daily oral cyclophosphamide for induction of remission in antineutrophil cytoplasmic antibody-associated vasculitis: a randomized trial. Ann Intern Med 2009;150(10): 670-80.  Back to cited text no. 12
13.Pagnoux C, Mahr A, Hamidou MA, et al. Azathioprine or methotrexate maintenance for ANCA-associated vasculitis. N Engl J Med 2008; 359(26):2790-803.  Back to cited text no. 13
14.Nachman PH. Vasculitis syndromes: which main­tenance therapy for ANCA vasculitis? Nat Rev Nephrol 2009;5(5):254-6.  Back to cited text no. 14
15.Savage CO, Winearls CG, Evans DJ, Rees AJ, Lockwood CM. Microscopic polyarteritis: pre­sentation, pathology and prognosis. Q J Med 1985;56:467-83.  Back to cited text no. 15
16.Pallan L, Savage CO, Harper L. ANCA-associated vasculitis: from bench research to novel treat­ments. Nat Rev Nephrol 2009;5(5):278-86.  Back to cited text no. 16
17.Westman KW, Bygren PG, Olsson H, Ranstam J, Wieslander J. Relapse rate, renal survival, and cancer morbidity in patients with Wegener's granulomatosis or microscopic polyangiitis with renal involvement. J Am Soc Nephrol 1998;9 (5):842-52.  Back to cited text no. 17
18.Booth AD, Almond MK, Burns A, et al. Outcome of ANCA-associated renal vasculitis: a 5-year retrospective study. Am J Kidney Dis 2003;41 (4):776-84.  Back to cited text no. 18
19.Iatrou C, Zerbala S, Revela I, et al. Mycophe-nolate mofetil as maintenance therapy in patients with vasculitis and renal involvement. Clin Nephrol 2009;72(1):31-7.  Back to cited text no. 19
20.Lin W, Chen M, Zhao MH. Follow-up of avidity and titer of anti-myeloperoxidase antibodies in sera from patients with primary ANCA-asso-ciated vasculitis. Autoimmunity 2009;42 (3):198-202.  Back to cited text no. 20
21.Little MA, Nightingale P, Verburgh CA, et al. Early mortality in systemic vasculitis: relative contribution of adverse events and active vas-culitis. Ann Rheum Dis 2010;69(6):1036-43.  Back to cited text no. 21
22.Oh JS, Lee CK, Kim YG, Nah SS, Moon HB, Yoo B. Clinical features and outcomes of microscopic polyangiitis in Korea. J Korean Med Sci 2009;24(2):269-74.  Back to cited text no. 22
23.Slot MC, Tervaert JW, Franssen CF, Stegeman CA. Renal survival and prognostic factors in patients with PR3-ANCA associated vasculitis with renal involvement. Kidney Int 2003;63 (2):670-7.  Back to cited text no. 23

Correspondence Address:
Sidy Mohamed Seck
Nephrology Department, University Hospital Aristide Le Dantec, 30 Avenue Pasteur, BP 3001 Dakar
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PMID: 22382223

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