Top

BMC Nephrology

Published in:

Open Access 01-12-2016 | Research article

Long-term outcomes in antineutrophil cytoplasmic autoantibody–positive eosinophilic granulomatosis with polyangiitis patients with renal involvement: a retrospective study of 14 Chinese patients

Authors: Yinghua Chen, Yuemei Ding, Zhengzhao Liu, Haitao Zhang, Zhihong Liu, Weixin Hu

Published in: BMC Nephrology | Issue 1/2016

Abstract

Background

The clinic-pathological features and outcomes of Chinese patients with antineutrophil cytoplasmic autoantibody (ANCA)-positive eosinophilic granulomatosis with polyangiitis (EGPA) and renal involvement have not been studied.

Methods

Fourteen EGPA patients with renal involvement were included. All patients underwent renal biopsy. Clinic-pathological features and outcomes were retrospectively analyzed.

Results

The most common initial symptom of EGPA was asthma (57.1 %), followed by hemoptysis (21.4 %), gross hematuria (14.3 %), and arthritis (7.1 %). All patients had positive serum ANCA (anti-MPO in 12, anti-PR3 in 2). Elevated eosinophils (median 15 %, range 10–45 %) were found in all patients. The median serum IgE level was 463 g/L (range 200–1000 g/L). All patients presented with renal dysfunction, with a median SCr of 5.4 mg/dL (range 1.47–11 mg/dL), seven patients (50 %) required initial renal replacement therapy. Thirteen patients showed hematuria and proteinuria (median 1.1 g/24 h, range 0.5–7.8 g/24 h). Renal biopsy showed pauci-immune segmental necrotizing glomerulonephritis with crescents in 13 patients and acute interstitial nephritis in one patient. Twelve patients (85.7 %) showed renal interstitial eosinophil infiltration, among whom three had eosinophilic granuloma. Among seven patients (71.4 %) who required initial dialysis, 5 discontinued dialysis, one died, one received maintenance dialysis after glucocorticoids plus immunosuppressive for induction treatment. Twelve patients were followed up for a median of 43.5 months (range 6–83 months), during follow-up, two patients progressed to end-stage renal disease, nine had chronic kidney disease with eGFR < 60 mL/min, and two patients had normal eGFR.

Conclusions

Renal involvement in ANCA-positive EGPA could be severe and showed varied renal histology. Although intensive immunosuppressive therapy effectively improved the renal function, the long-term renal survival was poor. Early diagnosis and treatment are essential to improve long-term renal survival.

Churg J, Strauss L. Allergic granulomatosis, allergic angiitis, and periarteritisnodosa. Am J Pathol. 1951;27:277–301.PubMedPubMedCentral

Sinico RA, Di Toma L, Maggiore U, Bottero P, Radice A, Tosoni C, Grasselli C, Pavone L, Gregorini G, Monti S, Frassi M, Vecchio F, Corace C, Venegoni E, Buzio C. Prevalence and clinical significance of antineutrophil cytoplasmic antibodies in churg-strauss syndrome. Arthritis Rheum. 2005;52:2926–35.CrossRefPubMed

Sablé-Fourtassou R, Cohen P, Mahr A, Pagnoux C, Mouthon L, Jayne D, Blockmans D, Cordier JF, Delaval P, Puechal X, Lauque D, Viallard JF, Zoulim A, Guillevin L. French vasculitis study group. Antineutrophil cytoplasmic antibodies and the churg-strauss syndrome. Ann Intern Med. 2005;143:632–8.CrossRefPubMed

Keogh KA, Specks U. Churg-strauss syndrome: clinical presentation, antineutrophil cytoplasmic antibodies, and leukotriene receptor antagonists. Am J Med. 2003;115:284–90.CrossRefPubMed

Sinico RA, Di Toma L, Maggiore U, Tosoni C, Bottero P, Sabadini E, Giammarresi G, Tumiati B, Gregorini G, Pesci A, Monti S, Balestrieri G, Garini G, Vecchio F, Buzio C. Renal involvement in churg-strauss syndrome. Am J Kidney Dis. 2006;47:770–9.CrossRefPubMed

Chumbley LC, Harrison Jr EG, DeRemee RA. Allergic granulomatosis and angiitis (churg-strauss syndrome). report and analysis of 30 cases. Mayo Clin Proc. 1977;52:477–84.PubMed

Sokolowska BM, Szczeklik WK, Wludarczyk AA, Kuczia PP, Jakiela BA, Gasior JA, Bartyzel SR, Rewerski PA, Musial J. ANCA-positive and ANCA-negative phenotypes of eosinophilic granulomatosis with polyangiitis (EGPA): outcome and long-term follow-up of 50 patients from a single polish center. Clin Exp Rheumatol. 2014;32(3 Suppl 82):S41–47.PubMed

Lanham JG, Elkon KB, Pusey CD, Hughes GR. Systemic vasculitis with asthma and eosinophilia: a clinical approach to the churg-strauss syndrome. Medicine (Baltimore). 1984;63:65–81.CrossRef

Della Rossa A, Baldini C, Tavoni A, Tognetti A, Neglia D, Sambuceti G, Puccini R, Colangelo C, Bombardieri S. Churg-strauss syndrome: clinical and serological features of 19 patients from a single Italian centre. Rheumatology (Oxford). 2002;41:1286–94.CrossRef

10.

Masi AT, Hunder GG, Lie JT, Michel BA, Bloch DA, Arend WP, Calabrese LH, Edworthy SM, Fauci AS, Leavitt RY. The American College of rheumatology 1990 criteria for the classification of churg-strauss syndrome (allergic granulomatosis and angiitis). Arthritis Rheum. 1990;33:1094–100.CrossRefPubMed

11.

Levey AS, Stevens LA, Schmid CH, Zhang YL, Castro 3rd AF, Feldman HI, Kusek JW, Eggers P, Van Lente F, Greene T, Coresh J. CKD-EPI (chronic kidney disease epidemiology collaboration). a new equation to estimate glomerular filtration rate. Ann Intern Med. 2009;150:604–12.CrossRefPubMedPubMedCentral

12.

Levey AS, de Jong PE, Coresh J, El Nahas M, Astor BC, Matsushita K, Gansevoort RT, Kasiske BL, Eckardt KU. The definition, classification, and prognosis of chronic kidney disease: a KDIGO controversies conference report. Kidney Int. 2011;80:17–28.CrossRefPubMed

13.

Berden AE, Ferrario F, Hagen EC, Jayne DR, Jennette JC, Joh K, Neumann I, Noël LH, Pusey CD, Waldherr R, Bruijn JA, Bajema IM. Histopathologic classification of ANCA-associated glomerulonephritis. J Am SocNephrol. 2010;21:1628–36.

14.

Hu W, Liu C, Xie H, Chen H, Liu Z, Li L. Mycophenolatemofetil versus cyclophosphamide for inducing remission of ANCA vasculitis with moderate renal involvement. Nephrol Dial Transplant. 2008;23:1307–12.CrossRefPubMed

15.

Hu W, Liu Z, Ji D, Xie H, Gong D, Li L. Staphylococcal protein a immunoadsorption for Goodpasture’s syndrome in four Chinese patients. J Nephrol. 2006;19:312–7.PubMed

16.

Chen Y, Gong Z, Chen X, Tang L, Zhao X, Yuan Q, Cai G. Tripterygium wilfordii hook F (a traditional Chinese medicine) for primary nephrotic syndrome. Cochrane Database Syst Rev. 2013;11:8. doi:10.1002/14651858.

17.

Mahr A, Guillevin L, Poissonnet M, Aymé S. Prevalences of polyarteritisnodosa, microscopic polyangiitis, Wegener’s granulomatosis, and churg-strauss syndrome in a french urban multiethnic population in 2000: a capture-recapture estimate. Arthritis Rheum. 2004;51:92–9.CrossRefPubMed

18.

Watts RA, Lane SE, Scott DG, Koldingsnes W, Nossent H, Gonzalez-Gay MA, Garcia-Porrua C, Bentham GA. Epidemiology of vasculitis in Europe. Ann Rheum Dis. 2001;60:1156–7.CrossRefPubMedPubMedCentral

19.

Horai Y, Miyamura T, Hirata A, Nakamura M, Takahama S, Ando H, Minami R, Yamamoto M, Suematsu E. Churg-strauss syndrome: a retrospective study of 11 cases from a single center in Japan. Int J Rheum Dis. 2010;13:e6–e10.CrossRefPubMed

20.

Fauci AS, Haynes B, Katz P. The spectrum of vasculitis: clinical, pathologic, immunologic and therapeutic considerations. Ann Intern Med. 1978;89(5 Pt 1):660–76.CrossRefPubMed

21.

Baldini C, Talarico R, Della Rossa A, Bombardieri S. Clinical manifestations and treatment of churg-strauss syndrome. Rheum Dis Clin North Am. 2010;36:527–43.CrossRefPubMed

22.

Shimoi T, Shojima K, Murota A, Takizawa Y, Maruyama J, Setoguchi K. Clinical and pathological features of churg Strauss syndrome among a Japanese population: a case series of 18 patients. Asian Pac J Allergy Immunol. 2012;30:61–70.PubMed

23.

Sharma BK, Daga MK, Sharma M. A limited form of churg-strauss syndrome presenting without asthma and eosinophilia. Med J Aust. 2004;181:498–9.PubMed

24.

Solans R, Bosch JA, Pérez-Bocanegra C, Selva A, Huguet P, Alijotas J, Orriols R, Armadans L, Vilardell M. Churg-strauss syndrome: outcome and long-term follow-up of 32 patients. Rheumatology (Oxford). 2001;40:763–71.CrossRef

25.

Clutterbuck EJ, Evans DJ, Pusey CD. Renal involvement in churg-strauss syndrome. Nephrol Dial Transplant. 1990;5:161–7.CrossRefPubMed

26.

Kikuchi Y, Ikehata N, Tajima O, Yoshizawa N, Miura S. Glomerular lesions in patients with churg-strauss syndrome and the anti-myeloperoxidase antibody. Clin Nephrol. 2001;55:429–35.PubMed

27.

Park SY, Chang JH, Kim HW, Kim DK, Kim EY, Park JT, Chang TI, Park JW, Jeong HJ, Han DS, Kang SW. A case of nephrotic syndrome in a patient with churg-strauss syndrome. Rheumatol Int. 2010;30:1385–8.CrossRefPubMed

28.

Guillevin L, Lhote F, Cohen P, Jarrousse B, Lortholary O, Généreau T, Léon A, Bussel A. Corticosteroids plus pulse cyclophosphamide and plasma exchanges versus corticosteroids plus pulse cyclophosphamide alone in the treatment of polyarteritisnodosa and churg-strauss syndrome patients with factors predicting poor prognosis. A prospective, randomized trial in sixty-two patients. Arthritis Rheum. 1995;38:1638–45.CrossRefPubMed

29.

Ribi C, Cohen P, Pagnoux C, Mahr A, Arène JP, Lauque D, Puéchal X, Letellier P, Delaval P, Cordier JF, Guillevin L. French VasculitisStudy group. Treatment of churg-strauss syndrome without poor-prognosis factors: a multicenter, prospective, randomized, open-label study of seventy-two patients. Arthritis Rheum. 2008;58:586–94.CrossRefPubMed

30.

Mukhtyar C, Flossmann O, Hellmich B, Bacon P, Cid M, Cohen-Tervaert JW, Gross WL, Guillevin L, Jayne D, Mahr A, Merkel PA, Raspe H, Scott D, Witter J, Yazici H, Luqmani RA. European vasculitis study group (EUVAS). outcomes from studies of antineutrophil cytoplasm antibody associated vasculitis: a systematic review by the European league against rheumatism systemic vasculitis task force. Ann Rheum Dis. 2008;67:1004–10.CrossRefPubMed

31.

JonesR HL, BallarinJ BD, Brogan P, Bruchfeld A, Cid M, Dahlsveen K, Dezoysa J, Lanyon P, Peh CA, Tesar V, Vaglio A, Walsh M, Walsh D, Walters G, Jayne D. A randomized trial of mycophenolate mofetil versus cyclophosphamide for remission induction of ANCA-associated vasculitis: “MYCYC”.on behalf of the European vasculitis study group [abstract]. Presse Med. 2013;42:678–9.CrossRef

32.

Silva F, Specks U, Kalra S, Hogan MC, Leung N, Sethi S, Fervenza FC. Mycophenolate mofetil for induction and maintenance of remission in microscopic polyangiitis with mild to moderate renal involvement–a prospective, open-label pilot trial. Clin J Am SocNephrol. 2010;5:445–53.CrossRef

33.

Joy MS, Hogan SL, Jennette JC, Falk RJ, Nachman PH. A pilot study using mycophenolate mofetil in relapsing or resistant ANCA small vessel vasculitis. Nephrol Dial Transplant. 2005;20:2725–32.CrossRefPubMed

34.

Koukoulaki M, Jayne DR. Mycophenolate mofetil in anti-neutrophil cytoplasm antibodies-associated systemic vasculitis. Nephron Clin Pract. 2006;102:c100–107.CrossRefPubMed

35.

Stassen PM, Tervaert JW, Stegeman CA. Induction of remission in active anti-neutrophil cytoplasmic antibody-associated vasculitis with mycophenolate mofetil in patients who cannot be treated with cyclophosphamide. Ann Rheum Dis. 2007;66:798–802.CrossRefPubMed

36.

Comarmond C, Pagnoux C, Khellaf M, Cordier JF, Hamidou M, Viallard JF, Maurier F, Jouneau S, Bienvenu B, Puéchal X, Aumaître O, Le Guenno G, Le Quellec A, Cevallos R, Fain O, Godeau B, Seror R, Dunogué B, Mahr A, Guilpain P, Cohen P, Aouba A, Mouthon L, Guillevin L. French vasculitis study group. Eosinophilic granulomatosis with polyangiitis (churg-strauss): clinical characteristics and long-term follow up of the 383 patients enrolled in the French vasculitis study group cohort. Arthritis Rheum. 2013;65:270–81.CrossRefPubMed

37.

Hiemstra TF, Walsh M, Mahr A, Savage CO, de Groot K, Harper L, Hauser T, Neumann I, Tesar V, Wissing KM, Pagnoux C, Schmitt W, Jayne DR. European vasculitis study group (EUVAS). mycophenolate mofetil vs azathioprine for remissionmaintenance in antineutrophil cytoplasmic antibody-associated vasculitis: a randomized controlled trial. JAMA. 2010;304:2381–8.CrossRefPubMed

38.

Mukhtyar C, Guillevin L, Cid MC, Dasgupta B, de Groot K, Gross W, Hauser T, Hellmich B, Jayne D, Kallenberg CG, Merkel PA, Raspe H, Salvarani C, Scott DG, Stegeman C, Watts R, Westman K, Witter J, Yazici H, Luqmani R. European vasculitis study group. EULAR recommendations for the management of primary small and medium vessel vasculitis. Ann Rheum Dis. 2009;68:310–7.CrossRefPubMed

Title: Long-term outcomes in antineutrophil cytoplasmic autoantibody–positive eosinophilic granulomatosis with polyangiitis patients with renal involvement: a retrospective study of 14 Chinese patients
Authors: Yinghua Chen
Yuemei Ding
Zhengzhao Liu
Haitao Zhang
Zhihong Liu
Weixin Hu
Publication date: 01-12-2016
Publisher: BioMed Central
Published in: BMC Nephrology / Issue 1/2016
Electronic ISSN: 1471-2369
DOI: https://doi.org/10.1186/s12882-016-0319-2

ACC 2024 Congress Coverage

Heart Failure Video

At a glance: The ONWARDS insulin icodec trials

Springer Medicine

Long-term outcomes in antineutrophil cytoplasmic autoantibody–positive eosinophilic granulomatosis with polyangiitis patients with renal involvement: a retrospective study of 14 Chinese patients

Abstract

Background

Methods

Results

Conclusions

ACC 2024 Congress Coverage

Year in Review: Heart failure and cardiomyopathies

Semaglutide benefits people with type 2 diabetes and obesity-related heart failure

Incentivised to exercise

New intervention for STEMI-related cardiogenic shock

» View more highlights on the ACC 2024 congress hub page

At a glance: The ONWARDS insulin icodec trials

Springer Medicine

Abstract

Background

Methods

Results

Conclusions

Please log in to get access to this content

Other articles of this Issue 1/2016

The effect of continuous venovenous hemofiltration on neutrophil gelatinase-associated lipocalin plasma levels in patients with septic acute kidney injury

Mesangial cells from patients with IgA nephropathy have increased susceptibility to galactose-deficient IgA1

Haemodiafiltration elicits less platelet activation compared to haemodialysis

Novel methods for microCT-based analyses of vasculature in the renal cortex reveal a loss of perfusable arterioles and glomeruli in eNOS-/- mice

Differential impact of smoking on mortality and kidney transplantation among adult Men and Women undergoing dialysis

The possible involvement of intestine-derived IgA1: a case of IgA nephropathy associated with Crohn’s disease

ACC 2024 Congress Coverage

Year in Review: Heart failure and cardiomyopathies

Semaglutide benefits people with type 2 diabetes and obesity-related heart failure

Incentivised to exercise

New intervention for STEMI-related cardiogenic shock

» View more highlights on the ACC 2024 congress hub page