Abstract
ANCA associated vasculitides are a group of disorders characterized by the presence of necrotizing inflammation of blood vessels that involve venules, capillaries, arterioles, arteries, and veins. They are characterized by the presence of antineutrophil cytoplasmic antibodies (ANCA). ANCA associated vasculitis commonly affects the kidney producing proteinuria, hematuria and variable degrees of renal failure. Other organs involved include the lung, skin and peripheral nervous system. We present a patient with limited systemic sclerosis, who later developed pauci-immune glomerulonephritis with p-ANCA (antimyeloperoxidase) specificity and, whose disease was later complicated by cerebral vasculitis with multiple intracerebral hemorrhages.
References
Jennette JC (1991) Antineutrophil cytoplasmic autoantibody-associated diseases: a pathologist’s perspective. Am J Kidney Dis 18:164–170
Ara J, Mirapeix E, Rodriguez R, Saurina A, Darnell A (1999) Relationship between ANCA and disease activity in small vessel vasculitis patients with anti-MPO ANCA. Nephrol Dial Transplant 14:1667–1672. doi:10.1093/ndt/14.7.1667
Falk RJ, Jennette JC (2002) ANCA are pathogenic–oh yes they are! J Am Soc Nephrol 13:1977–1979
Jennette JC, Falk RJ, Andrassy K et al (1994) Nomenclature of systemic vasculitides proposal of an international consensus conference. Arthritis Rheum 37:187–192. doi:10.1002/art.1780370206
Jennette JC, Thomas DB, Falk RJ (2001) Microscopic polyangiitis (microscopic polyarteritis). Semin Diagn Pathol 18:3–13
Endo H, Hosono T, Kondo H (1994) Antineutrophil cytoplasmic autoantibodies in 6 patients with renal failure and systemic sclerosis. J Rheumatol 21:864–870
Locke IC, Worrall JG, Leaker B, Black CM, Cambridge G (1997) Autoantibodies to myeloperoxidase in systemic sclerosis. J Rheumatol 24:86–89
Wutzl AL, Foley RN, O’Driscoll BR, Reeve RS, Chisholm R, Herrick AL (2001) Microscopic polyangiitis presenting as pulmonary-renal syndrome in a patient with long-standing diffuse cutaneous systemic sclerosis and antibodies to myeloperoxidase. Arthritis Rheum 45:533–536. doi :10.1002/1529-0131(200112)45:6<533::AID-ART379>3.0.CO;2-D
Jennette JC, Falk RJ (1997) Small-vessel vasculitis. N Engl J Med 337:1512–1523. doi:10.1056/NEJM199711203372106
Niles JL, Bottinger EP, Saurina GR et al (1996) The syndrome of lung hemorrhage and nephritis is usually an ANCA-associated condition. Arch Intern Med 156:440–445. doi:10.1001/archinte.156.4.440
Deshpande PV, Gilbert R, Alton H, Milford DV (2000) Microscopic polyarteritis with renal and cerebral involvement. Pediatr Nephrol 15:134–135. doi:10.1007/s004670000428
Sasaki A, Hirato J, Nakazato Y, Tanaka T, Takeuchi H (1998) An autopsy case of P-ANCA-positive microscopic polyangiitis with multiple cerebral hemorrhagic infarction. No To Shinkei 50:56–60
Honda H, Hasegawa T, Morokawa N, Kato N, Inoue K (1996) A case of MPO-ANCA related vasculitis with transient leukoencephalopathy and multiple cerebral hemorrhage. Rinsho Shinkeigaku 36:1089–1094
Ito Y, Suzuki K, Yamazaki T, Yoshizawa T, Ohkoshi N, Matsumura A (2006) ANCA-associated vasculitis (AAV) causing bilateral cerebral infarction and subsequent intracerebral hemorrhage without renal and respiratory dysfunction. J Neurol Sci 240:99–101. doi:10.1016/j.jns.2005.07.003
Caramaschi P, Biasi D, Carletto A, Bambara LM (2003) A case of ANCA-associated vasculitis with predominant involvement of central nervous system. Joint Bone Spine 70:380–383. doi:10.1016/S1297-319X(03)00098-8
Jacobi D, Maillot F, Hommet C et al (2005) P-ANCA cranial pachymeningitis: a case report. Clin Rheumatol 24:174–177. doi:10.1007/s10067-004-1022-4
Dandekar SS, Narendran NN, Edmunds B, Graham EM (2004) Ocular involvement in systemic vasculitis associated with perinuclear antineutrophil cytoplasmic antibodies. Arch Ophthalmol 122:786–787. doi:10.1001/archopht.122.5.786
Altaie R, Ditizio F, Fahy GT (2005) Microscopic polyangitis presenting with sub-acute reversible optic neuropathy. Eye 19:363–365. doi:10.1038/sj.eye.6701479
Nachman PH, Hogan SL, Jennette JC, Falk RJ (1996) Treatment response and relapse in antineutrophil cytoplasmic autoantibody-associated microscopic polyangiitis and glomerulonephritis. J Am Soc Nephrol 7:33–39
Molloy ES, Langford CA (2006) Advances in the treatment of small vessel vasculitis. Rheum Dis Clin North Am 32:157–172. doi:10.1016/j.rdc.2005.12.002
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Akkara Veetil, B.M., Schimmer, B.M. A case of limited systemic sclerosis with p-ANCA, complicated by multiple cerebral hemorrhages. Rheumatol Int 29, 325–329 (2009). https://doi.org/10.1007/s00296-008-0675-8
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DOI: https://doi.org/10.1007/s00296-008-0675-8