Abstract
Systemic vasculitic syndromes are rare in childhood. Vasculitis is the predominant feature of a large number of different clinical entities that are linked by the presence of inflammatory changes in the blood vessels. The nature of these diseases and their relationship to each other remain unclear. The clinical presentation associated with the size of the affected vessels and epidemiological data are very helpful for the diagnosis of those diseases. Recent advances are beginning to shed some light on the etiology and pathogenetic mechanisms involved in the various vasculitides. There is good evidence to support roles for circulating immune complexes, cell-mediated immunity, anti-neutrophil cytoplasmic antibodies and anti-endothelial cell antibodies in the pathogenesis. Renal involvement in vasculitis in children is commonly seen in Henoch-Schönlein purpura, microscopic polyarteritis, Wegener's granulomatosis, Churg-Strauss syndrome and polyarteritis nodosa. However, kidney disease can also be part of the clinical picture of Kawasaki disease and Takayasu arteritis. Recently, with the institution of early and aggressive immunosuppressive treatment of severe cases, significant improvement in the long-term survival of patients has been achieved. This review article addresses the pathological and clinical features (particularly renal involvement), therapeutic intervention and prognosis of the above-mentioned diseases.
Similar content being viewed by others
References
Churg A, Churg J (1991) Systemic vasculitides. Igaku-Shoin, New York, pp 3–5
Salcedo JR, Greenberg GL, Kapur S (1988) Renal histology of mucocutaneous lymph node syndrome (Kawasaki disease). Clin Nephrol 29: 347–351
Hall JB, Wadham B MCN, Wood CJ, Ashton V, Adam WR (1984) Vasculitis and glomerulonephritis, a subgroup with an antineutrophil cytoplasmic antibody. Aust N Z J Med 14: 277–278
Savage COS, Winearls CG, Jones S, Marshall PD, Lockwood CM (1989) Prospective study of radioimmunoassay for antibodies against neutrophil cytoplasma in diagnosis of systemic vasculitis. Lancet I: 1389–1393
Van der Wall Bake AWL, Lobatto S, Jonges L (1987) IgA antibodies directed against cytoplasmic antigens of polymorphonuclear leucocytes in patients with Henoch-Schonlein purpura. In: McGee JR, Mestecky J (eds) Advances in mucosal immunology. Plenum, New York, pp 1593–1598
Savage COS, Tizard EJ, Jayne D, Lockwood CM, Dillon MJ (1989) Antineutrophil cytoplasma antibodies in Kawasaki disease. Arch Dis Child 6: 360–363
Ewert BH, Jennette JC, Falk RJ (1992) Anti-myeloperoxidase antibodies stimulate neutrophils to damage human endothelial cells. Kidney Int 41: 375–383
Kussmaul A, Maier R (1866) Über eine bisher nicht beschriebene eigentümliche Arterienerkrankung (Periarteritis Nodosa). Dtsch Arch Klin Med 1: 484–518
Kurland LT, Chuang T-Y, Hunder GG (1984) The epidemiology of systemic arteritis. In: Current topics in Rheumatology: Epidemiology of the Rheumatic Diseases, pp. 196, New York, Gower Medicial Publisher
Leib ES, Hibrawi H, Chia D, Blaker R, Barnett EV (1981) Correlation of disease activity in systemic necrotizing vasculitis with immune complexes. J Rheumatol 8: 258–265
Ronco P, Verroust P, Mignon F, Kourilsky O, Vanhille P, Meyrier A, Mery JP, Morel-Maroger L (1983) Immunopathological studies of polyarteritis nodosa and Wegener's granulomatosis. A report of 43 patients with 54 renal biopsies. Q J Med 52: 212–223
Brasile L, Kremer JM, Clarke JL (1989) Identification of an autoantibody to vascular endothelial cell-specific antigens in patients with systemic vasculitis. Am J Med 87: 74–80
Conn DL (1990) Polyarteritis. Rheum Dis Clin North Am 16: 341–362
White RH, Schambelan M (1980) Hypertension, hyperreninemia, and secondary hyperaldosteronism in systemic necrotizing vasculitis. Ann Intern Med 29: 199–201
Graham PC, Lindop GB (1990) The renin-secreting cell in polyarteritis — an immunocytochemical study. Histopathology 16: 339–345
Genc FA, Mercan S, Belgerden S, Uysal V (1990) Spontaneous perirenal haematoma due to polyarteritis nodosa. Br J Clin Pract 44: 744–745
Smith DL, Wernick R (1989) Spontaneous rupture of a renal artery aneurysm in polyarteritis nodosa: critical review of the literature and report of a case. Am J Med 87: 464–467
Arkin A (1930) A clinical and pathological study of periarteritis nodosa. Am J Pathol 6:401–427
Zeek PM, Smith CC, Weeter JC (1948) Studies on periarteritis nodosa. III. The differentiation between the vascular lesions of periarteritis nodosa and of hypersensitivity. Am J Pathol 24: 889–918
Cohen RD, Conn DL, Ilstsup DM (1980) Clinical features, prognosis, and response to treatment in polyarteritis. Mayo Clin Proc 55: 146–155
Frohnet PP, Sheps SG (1967) Longterm follow-up study of periarteritis nodosa. Am J Med 43: 8–14
Leib ES, Restivo C, Paulus HE (1979) Immunosuppressive and corticosteroid therapy of polyarteritis nodosa. Am J Med 67: 941–947
Fauci AS, Katz P, Haynes BF, Wolff SM (1979) Cyclophosphamide therapy of severe systemic necrotizing vasculitis. N Engl J Med 301: 235–238
Montalbert C, Carvallo A, Broumand B, Noble D, Anstine LA, Currier CB Jr (1980) Successful renal transplantation in polyarteritis nodosa. Clin Nephrol 14: 206–209
Kawasaki T (1967) Acute febrile mucocutaneous lymph node syndrome with lymphoid involvement with specific desquamation of fingers and the toes. Jpn J Allergy 16: 178–222
Hicks RV, Melish ME (1986) Kawasaki syndrome. Pediatr Clin North Am 38: 1151–1175
Leung DYM, Collin T, Lapierre LA, Geba RS, Pober JS (1986) Immunoglobulin and antibodies present in the acute phase of Kawasaki syndrome lyse cultured vascular endothelial cells stimulated by gamma interferon. J Clin Invest 77: 1428–1435
Burns JC, Globe MP, Clarke SH, Wiggins J Jr, Hathaway WE (1984) Coagulopathy and platelet activation in Kawasaki syndrome: identification of patients at high risk for development of coronary artery aneurysms. J Pediatr 105: 206–211
Tizard EJ, Suzuki A, Levin M, Dillon MJ (1991) Clinical aspects of 100 patients with Kawasaki disease. Arch Dis Child 66: 185–188
Sasaguri Y, Kato H (1982) Regression of aneurysms in Kawasaki disease: a pathological study. J Pediatr 100: 225–231
Gribetz D, Landing BH, Larson EJ (1991) Kawasaki disease: Mucocutaneous lymphnode syndrome (MCLS), In: Churg A, Churg J (eds) Systemic vasculitides, chapter 18. Igaku-Shoin, Tokyo
Newburger JW, Takahashi M, Burns JC, Beiser AS, Chung KJ, Duff E, Glode MP, Mason WH, Reddy V, Sanders P, Shulman ST, Wigging JW, Hicks RV, Fulton DR, Lewis AB, Leung DYM, Colton T, Rosen FS, Melish ME (1986) The treatment of Kawasaki syndrome with intravenous immunoglobulin. N Engl J Med 315: 341–347
Rowley AH, Shulman ST (1991) Current therapy for acute Kawasaki syndrome. J Pediatr 118: 987–991
Fauci AS, Haynes BF, Katz P, Wolff SM (1983) Wegener's granulomatosis: prospective clinical and therapeutic experience with 85 patients for 21 years. Ann Intern Med 98: 76–85
Moorthy AV, Chesney RW, Segar WE, Groshong T (1977) Wegener granulomatosis in childhood: prolonged survival following cytotoxic therapy. J Pediatr 91: 616–618
Orlowski JP, Clough JD, Dyment PG (1978) Wegener's granulomatosis in the pediatric age group. Pediatrics 61: 83–90
Harriwon HL, Lishaw MA, Lindsley CB, Cuppage FE (1980) Bolus corticosteroids and cyclophosphamide for initial treatment of Wegener's granulomatosis. JAMA 244: 1599–1600
Brandwein S, Esdaile J, Danoff D, Tannenbaum H (1983) Wegener's granulomatosis: clinical features and outcome in 13 patients. Arch Intern Med 143: 476–479
DeRemee RA (1988) Extrapulmonary manifestations of Wegener's granulomatosis and other respiratory vasculitides. Semin Respir Med 9: 403–408
Pinching AJ, Lockwood CM, Pussell BA, Rees AJ, Sweny P, Evans DJ, Bowley N, Peters DK (1983) Wegener granulomatosis: observations on 18 patients with severe renal disease. Q J Med 52: 435–460
Givens CD Jr, Newman JH, McCurley TL (1985) Diagnosis of Wegener's granulomatosis by transbronchial biopsy. Chest 88: 794–796
Travis WD, Hoffman GS, Leavitt RY, Pass HI, Fauci AS (1991) Surgical pathology of the lung in Wegener's granulomatosis. Am J Surg Pathol 15: 315–333
Specks U, DeRemee RA (1990) Granulomatous vaseulitis: Wegener's granulomatosis and Churg-Strauss syndrome. Rheum Dis Clin North Am 16: 377–397
Lieberman K, Churg A (1991) Wegener's granulomatosis. In: Churg A, Churg J (eds) Systemic vasculitides. Igaku-Shoin, New York, pp 79–100
Specks U, Wheatley CL, McDonald T, Rohrbach MS, DeRemee RA (1989) Anti-cytoplasmic autoantibodies in the diagnosis and follow-up of Wegener's granulomatosis. Mayo Clin Proc 64: 28–36
Nolle B, Specks U, Ludemann J, Rohrbach MS, De Remee RA, Gross WL (1989) Anti-cytoplasmic auto-antibodies: their immunodiagnostic value in Wegener granulomatosis. Ann Intern Med 111: 28–40
Andrassy K, Erb A, Koderisch J, Waldherr R, Ritz E (1991) Wegener's granulomatosis with renal involvement: patient survival and correlations between initial renal function, renal histology, therapy and renal outcome. Clin Nephrol 25: 139–147
DeRemee RA, McDonald TJ, Weiland LH (1985) Wegener's granulomatosis: observations on treatment with antimicrobial agents. Mayo Clin Proc 60: 27–32
Appel GB, Gee B, Kashgarian M, Hayslett JP (1981) Wegener's granulomatosis-clinical-pathologic correlations and long-term course. Am J Kidney Dis 1: 27–37
Oberhuber G, Prior C, Boxmuller C, Dietze O, Margreiter R (1988) Early recurrence of Wegener's granulomatosis in a kidney allograft under cyclosporine treatment. Transplant Int I: 49–50
Jacquot C, Thoua Y, Dupont E, Vereerstraeten P (1990) Recidive de granulomatose de Wegener sur une greffe de rein de cadavre. Nephrologie 11: 97–103
Tai P-C, Holt ME, Denny P, Gibbs AR, Williams BD, Spry CJF (1984) Deposition of eosinophil cationic protein in granulomas in allergic granulomatosis and vasculitis: the Churg-Strauss syndrome. BMJ 289: 400–402
Wathen CW, Harrison DJ (1987) Circulating anti-neutrophilic antibodies in systemic vasculitis. Lancet I: 1037
Fink CW (1986) Vasculitis. Pediatr Clin North Am 33: 1203–1219
Jessurun J, Azevedo M, Saldana M (1986) Allergic angiitis and granulomatosis (Churg-Strauss syndrome): report of a case with massive thymic involvement in a nonasthmatic patient. Hum Pathol 17: 637–639
Clutterbuck EJ, Evans DJ, Pusey CD (1990) Renal involvement in Churg-Strauss syndrome. Nephrol Dial Transplant 5: 161–167
Davenport A, McDicken J, Goldsmith HJ (1988) Reversible acute renal failure due to Churg-Strauss syndrome. Postgrad Med J 64: 713–715
Antiga G, Volpi A, Battini G, Bignotti GG, Giordano F, Meroni M, Minazzi M, Sinico RA, Sessa A (1991) Acute renal failure in a patient affected with Churg-Strauss syndrome. Nephron 57: 113–114
Azar N, Guillevin L, Huong-Du LT, Herreman G, Meyrier A, Godeau P (1989) Symptomatic urogenital manifestations of polyarteritis nodosa and Churg-Strauss angiitis: analysis of 8 of 165 patients. J Urol 142: 136–138
Cortellini P, Manganelli P, Poletti F, Sacchini P, Ambanelli U, Bezzi E (1988) Ureteral involvement in the Churg-Strauss syndrome: a case report. J Urol 140: 1016–1018
Lanham JG, Churg J (1991) Churg-Strauss syndrome. In: Churg A, Churg J (eds) Systemic vasculitides. Igaku-Shoin, New York, pp 101–120
Churg J (1991) Nomenclature of vasculitic syndromes: a historical perspective. Am J Kidney Dis 18: 148–153
Ross RS, McKusick VA (1953) Aortic arch syndromes: diminished or absent pulses in arteries arising from arch of aorta. Arch Intern Med 92: 701–740
Nakao K, Ikeda M, Kimata S, Nutani H, Miyahara M, Ishimi Z, Hashiba K, Takeda Y, Ozawa T, Matsushita S, Kuramochi M (1967) Takayasu's arteritis: clinical report of eighty-four cases and immunological studies of seven cases. Circulation 35: 1141–1155
Nasu T (1975) Takayasu's trunk arteritis in Japan: a statistical observation of 76 autopsy cases. Pathol Microbiol 43: 140–146
Lande A, Berkman YM (1976) Aortitis: pathological, clinical and arteriographic review. Radiol Clin North Am 14: 219–240
Lupi-Herrera E, Sanchez-Torres G, Morcushamer J, Mispireta J, Horwitz S, Vela JE (1977) Takayasu's arteritis. Clinical study of 107 cases. Am Heart J 93: 94–103
Wiggelinkhuizen J, Cremin BJ (1978) Takayasu arteritis and renovascular hypertension in childhood. Pediatrics 62: 209–217
Matsumura K, Hirano T, Takeda K, Matsuda A, Nakagawa T, Yamaguchi N, Yuasa H, Kusakawa M, Nakano T (1991) Incidence of aneurysms in Takayasu's arteriris. Angiology 42: 308–315
Hall S, Buchbinder R (1990) Takayasu's arteritis. Rheum Dis Clin North Am 16: 411–422
Sabba C, Pugriese D, Caruso G, Bianco M, Centonze V, Pennella A, Albano O (1990) Takayasu's arteritis: a case report and discussion of differences in Eastern and Western cases. Am J Cardiovasc Pathol 3: 95–99
Arend WP, Michel BA, Bloch DA, Hunder GG, Calabrese LH, Edworthy SM, Fauci AS, Leavitt RY, Lie JT, Lightfoot RW Jr, Masi AT, McShane DJ, Mills JA, Stevens MB, Wallace SL, Zvaifler NJ (1990) The American College of Rheumatology 1990 criteria for the classification of Takayasu arteritis. Arthritis Rheum 33: 1129–1134
Eke F, Balfe JW, Hardy BE (1984) Three patients with arteritis. Arch Dis Child 59: 877–883
Strachan RW (1964) The natural history of Takayasu's arteriopathy. Q J Med 33: 57–69
Bonventre MV (1974) Takayasu's disease revisited. N Y State J Med 74: 1960–1967
Sandring H, Welin G (1961) Aortic arch syndrome with special reference to rheumatoid arthritis. Acta Med Scand 170: 1–19
Hall S, Nelson AM (1986) Takayasu's arteritis and juvenile rheumatoid arthritis. J Rheumatol 13: 431–433
Wilson WA, Morgan OS, Bain B, Taylor JE (1979) Takayasu's arteritis: association with Still's disease in an adult. Arthritis Rheum 22: 684–688
Lie JT (1991) Takayasu's arteritis. In: Churg A, Churg J (eds) Systemic vasculitides. Igaku-Shoin, Tokyo, pp 159–179
Lupi F II, Sanches G, Horowitz S, Gutierrez E (1975) Pulmonary artery involvement in Takayasu's arteritis. Chest 67: 69–74
Cohle SD, Lie JT (1988) Inflammatory aneurysm of the aorta, aortitis, and coronary arteritis. Arch Pathol Lab Med 112: 1121–1125
Milner LS, Jacobs DW, Thomson PD, Kala UK, Franklin J, Beale P, Levin SE (1991) Management of severe hypertension in childhood Takayasu's arteritis. Pediatr Nephrol 5: 38–41
Yoshimura M, Kida H, Saito Y, Yokoyama H, Tomosugi N, Abe T, Hattori N (1985) Peculiar glomerular lesions in Takayasu's arteritis. Clin Nephrol 24: 120–127
Hellman DB, Hardy K, Lindefield S (1987) Takayasu's arteritis associated with crescentic glomerulonephritis. Arthritis Rheum 30: 451–454
Ishikawa K (1988) Diagnostic approach and proposed criteria for the clinical diagnosis of Takayasu's arteriopathy. J Am Coll Cardiol 12: 964–972
Subramanian R, Joy J, Balakrishnah KG (1989) Natural history of aortoarteritis (Takayasu's disease). Circulation 80: 429–437
Ito I (1966) Aortitis syndrome with reference to detection of antiaorta antibody from patients' sera. Jpn Circ J 30: 75–78
Yoshikawa Y, Truong LD, Mattioli CA, Lederer E (1988) Membranoproliferative glomerulonephritis in Takayasu's arteritis. Am J Nephrol 8: 240–244
Sharma S, Rajani M, Kamalakar T, Kumar A, Talwar KK (1990) The association between aneurysm formation and systemic hypertension in Takayasu's arteritis. Clin Radiol 42: 182–187
Sharma S, Rajani M, Shrivastova S, Kaul V, Kamolokas T, Talwar KK, Soxena A (1991) Non-specific-aorta-arteritis (Takayasu's disease) in children. Br J Radiol 64: 690–698
Kumar S, Subramanian R, Mandalom KR, Rao VR, Gupta AK, Joseph S, Unvi NM, Rao AS (1990) Aneurysmal form of aortoarteritis (Takayasu's disease): analysis of thirty cases. Clin Radiol 42: 342–347
Liu YO, Ling J, Wang ZL (1990) Intravenous digital subtraction angiography in patients with aorto-arteritis (Takayasu's). Cardiovasc Intervent Radiol 13: 83–87
Lai KN, Chan KW, Ho CP (1986) Glomerulonephritis associated with Takayasu's arteritis: report of three cases and review of the literature. Am J Kidney Dis 7: 197–204
Weaver FA, Yellin AE, Campen DH, Oberg J, Foran J, Kitridov RC, Lee SE, Kohl RD (1990) Surgical procedures in the management of Takayasu's arteritis. J Vasc Surg 12: 429–437
Sharma S, Rajani M, Kaul V, Talwar KK, Dev V, Shrivastova S (1990) Initial experience with percutaneous transluminal angioplasty in the management of Takayasu's arteritis. Br J Radiol 63: 517–522
White RHR (1991) Henoch-Schonlein purpura. In: Churg A, Churg J (eds) Systemic vasculitides, chapter 14. Igaku-Shoin, Tokyo
Meadow SR, Glasgow EF, White RHR, et al (1972) Schonlein Henoch nephritis. Q J Med 41: 241–258
Levy M, Broyer M, Arsan A, et al (1976) Anaphylactoid purpura nephritis in childhood: natural history and immunopathology. Adv Nephrol 6: 183–224
Meadow SR, Scott DG (1985) Berger disease: Henoch-Schonlein syndrome without the rash. J Pediatr 106: 27–32
Gairdner D (1948) Schonlein-Henoch syndrome. Q J Med 17: 95–122
Van Es LA, Kauffmann RH, Valentijn RM (1987) Renal manifestations of systemic disease: Henoch-Schonlein purpura. In: Holliday MA, Barratt TM, Vernier RL (eds) Pediatric nephrology, 2nd edn. Williams and Wilkins, Baltimore, pp 492–498
Churg J, Churg A (1989) Idiopathic and secondary vasculitis: a review. Mod Pathol 2: 144–160
Baart de la Faille-Kuyper EH, Kater L, Kuijten RH, et al (1976) Occurrence of vascular IgA deposits in clinically normal skin of patients with renal disease. Kidney Int 9: 424–429
Touchard G, Maire P, Beauchant M, et al (1983) Vascular IgA and C3 deposition in gastrointestinal tract of patients with Henoch-Schonlein purpura. Lancet I: 771–772
Hall SL, Miller LC, Duggan E, Mauer SM, Beatty EC, Hellerstein S (1985) Wegener granulomatosis in pediatric patients. J Pediat 106: 739–744
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Roberti, I., Reisman, L. & Churg, J. Vasculitis in childhood. Pediatr Nephrol 7, 479–489 (1993). https://doi.org/10.1007/BF00857580
Received:
Revised:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF00857580