Polyarteritis nodosa (PAN), first described by Küssmaul and Maier, is a well-known form of necrotizing angiitis whose manifestations are weight loss, fever, asthenia, peripheral neuropathy, renal involvement, musculoskeletal and cutaneous manifestations, hypertension, gastrointestinal tract involvement, and cardiac failure. Recently individualized from PAN, microscopic polyangiitis (MPA) is a systemic vasculitis of small-size vessels whose clinical manifestations are very similar to those of PAN, but it is characterized by the presence of rapidly progressive glomerulonephritis (RPGN), which is nearly constant, and pulmonary involvement usually absent in PAN. Churg–Strauss syndrome (CSS) is a disorder characterized by hypereosinophilia and systemic vasculitis similar to that of PAN and occurring in individuals with asthma and allergic rhinitis. Considering the etiologies of PAN, primary and secondary vasculitides can also be distinguished because PAN can be the consequence of hepatitis B virus (HBV) infection and sometimes of other etiologic agents.
The prognosis of systemic vasculitides has been transformed by corticosteroids that are, except in HBV-related PAN, the basic treatment. Immunosuppressive drugs, especially cyclophophamide, have also contributed to improving the prognosis, but their precise role in the management of these vasculitides is still being elucidated.
Küssmaul A, Maier R. Ueber eine bischer nicht beschriebene eigenthümliche Arterienerkrankung (Periarteritis nodosa) die mit Morbus Brightii und rapid forstschreitender allgemeiner Muskellähmung einhergeht. Dtsch Arch Klin Med 1986; 1: 483.
2.
Adu D et al. Polyarteritis and the kidney . Q J Med1987; 62: 221–237 .
3.
D'Agati et al. Idiopathic microscopic polyarteritis nodosa: ultra structural observations on the renal vascular and glomerular lesions . Am J Kidney Dis1986; 7: 95–110 .
4.
Gordon M et al. Relapses in patients with a systemic vasculitis . QJ Mrd1993; 86: 779–789 .
5.
Savage COS et al. Microscopic polyarteritis: presentation, pathology and prognosis . Q J Med1985; 56: 467–483 .
6.
Serra A et al. Vasculitis affecting the kidney: presentation, histopathology and long-term outcome . Q J Med1984; 53: 181–207 .
7.
Guillevin L et al. Microscopic polyangiitis: clinical symptoms, laboratory findings and outcome in 89 patients . Arthritis Rheum1997; 40 (Supplement): S169-S169 (abstract 828).
8.
Jennette JC et al. Nomenclature of systemic vasculitides. Proposal of an international consensus conference . Arthritis Rheum1994; 37: 187–192 .
Churg J , Strauss L . Allergic granulomatosis, allergic angiitis and periarteritis nodosa . Am J Pathol1951; 27: 277–301 .
11.
Lanham et al. Systemic vasculitis with asthma and eosinophilia: a clinical approach to the Churg–Strauss syndrome . Medicine (Baltimore)1984; 63: 65–81 .
12.
Cohen P et al. and the French Polyarteritis Nodosa Study Group Clinical aspects and long survival of 85 patients with Churg–Strauss syndrome . Arthritis Rheum1995; 38 (Supplement 9): S. 391-S. 391 [abstract 1438].
13.
Gocke DJ et al. Association between polyarteritis nodosa and Australia antigen . Lancet1970; 2: 1149–1150 .
14.
Guillevin L et al. Polyarteritis nodosa related to hepatitis B virus. A prospective study with long-term observation of 41 patients . Medicine (Baltimore)1995; 74: 238–253 .
15.
Zeek PM . Periarteritis nodosa: a critical review . Am J Clin Pathol1952; 22: 777–790 .
16.
Fauci AS , Haynes BF , Katz P . The spectrum of vasculitis: clinical, pathologic, immunologic and therapeutic considerations . Ann Intern Med1978; 89: 660–676 .
17.
Lightfoot RW et al. The American College of Rheumatology 1990 criteria for the classification of polyarteritis nodosa . Arthritis Rheum1990; 33: 1088–1093 .
18.
Lie JT . Histopathologic specificity of systemic vasculitis . Rheum Dis Clin North Am1995; 21: 883–910 .
19.
Cohen Tervaert JW , Kallemberg C . Neurologic manifestations of systemic vasculitides . Rheum Dis Clin North Am1993; 19: 913–940 .
20.
Lie JT . Nomenclature and classification of vasculitis: plus ça change, plus c'est la même chose . Arthritis Rheum1994; 37: 181–186 .
21.
Guillevin L , Lhote F . Distinguishing polyarteritis nodosa from microscopic polyangiitis . Curr Opin Rheumatol1995; 7: 20–24 .
22.
Hagen EC et al. Anti-neutrophil cytoplasm autoantibodies: a review of the antigens involved, the assays, and the clinical and possible pathogenetic consequences . Blood1993; 81: 1996–2002 .
23.
Van der Woud FJ et al. Autoantibodies against neutrophils and monocytes; tool for diagnosis and marker of disease activity in Wegener's granulomatosis . Lancet1985; i: 425–429 .
24.
Kallenberg GCM , Mulder AHL , Cohen Tervaert JW . Antineutrophil cytoplasmic autoantibodies: a still-growing class of autoantibodies in inflammatory disorders . Am J Med1992; 93: 675–682 .
25.
Savage COS . Interactions between leukocytes, the vascular endothelium and autoantibodies in the development of systemic vasculitis . Ann Méd Interne (Paris)1993; 144: 353–356 .
26.
Gross WL , Csernok E . Immunodiagnostic pathophysiologic aspects of antineutrophil cytoplasmic antibodies in vasculitis . Curr Opin Rheumatol1995; 7: 11–19 .
27.
Brasile L et al. Identification of an autoantibody to vascular endothelial cell-specific antigens in patients with systemic vasculitis . Am J Med1989; 87: 74–80 .
28.
Direskeneli H et al. Autoantibodies against endothelial cells, extracellular matrix, and human collagen type IV in patients with systemic vasculitis . Clin Immunol Immunopathol1994; 70: 206–210 .
29.
Savage COS et al. Vascular damage in Wegener's granulomatosis and microscopic polyarteritis: presence of anti-endothelial cell antibodies and their relation to anti-neutrophil cytoplasm antibodies . Clin Exp Immunol1991; 85: 14–19 .
30.
Kallenberg CGM . Autoantibodies in vasculitis: current perspectives . Clin Exp Rheumatol1993; 11: 355–360 .
31.
Del Papa N et al. Antibodies to endothelial cells in primary vasculitides mediate in vitro endothelial cytotoxicity in the presence of normal peripheral blood mononuclear cells . Clin Immunol Immunopathol1992; 63: 267–274 .
32.
Del Papa N et al. Anti-endothelial cell IgG antibodies from patients with Wegener's granulomatosis bind to human endothelial cells in vitro and induce molecule expression and cytokine secretion . Arthritis Rheum1996; 39: 758–766 .
33.
Grau GE et al. Serum cytokine changes in systemic vasculitis . Immunology1989; 68: 196–198 .
34.
Kelow J et al. Pro-and anti-inflammatory cytokines in primary systemic vasculitis. In: Gross WL (ed). ANCA-associated vasculitis. Plenum Press: New York and London , 1993 pp 341–344.
35.
Cid MC et al. Immunohistochemical characterization of inflammatory cells and immunologic activation markers in muscle and nerve biopsy specimens from patients with systemic polyarteritis nodosa . Arthritis Rheum1994; 37: 1055–1061 .
36.
Conn DL . In: Klippel JH , Dieppe P (eds). RheumatologyMosby Year Book Europe Ltd: London , 1994, pp 6-17.1–6-17.10.
37.
Gherardi R et al. The spectrum of vasculitis in human immunodeficiency virus-infected patients. A clinicopathologic evaluation . Arthritis Rheum1993; 36: 1164–1174 .
38.
Gaches F et al. Périartérite noueuse et infection par le parvovirus B 19 . Rev Méd Interne1993; 14: 323–325 .
39.
Finkel TH et al. Chronic parvovirus B19 infection and systemic necrotising vasculitis: opportunistic infection or aeteological agent?Lancet1994; 343: 1255–1258 .
40.
Vernant JC et al. Vascularites et manifestations neurologiques liées au HTLV-1 . Presse Méd1994; 23: 1421–1427 .
41.
Carson CW et al. Frequency and significance of antibodies to hepatitis C virus in polyarteritis nodosa . J Rheumatol1993; 20: 304–309 .
42.
Frohnert PP , Sheps SG . Long-term follow-up study of periarteritis nodosa . Am J Med1967; 43: 8–14 .
43.
Sach M , Cassidy JT , Bole GG . Prognostic factors in polyarteritis . J Rheumatol1975; 2: 411–420 .
44.
Leib ES , Restivo C , Paulus HE . Immunosuppressive and corticosteroid therapy of polyarteritis nodosa . Am J Med1979; 67: 941–947 .
Guillevin L et al. Clinical findings and prognosis of polyarteritis nodosa and Churg–Strauss angiitis: a study in 165 patients . Br J Rheumatol1988; 27: 258–264 .
47.
Guillevin L et al. Treatment of polyarteritis nodosa and Churg–Strauss syndrome. A meta-analysis of 3 prospective controlled trials including 182 patients over 12 years . Ann Méd Interne (Paris)1992; 143: 405–416 .
48.
Fortin PR et al. Prognosis factors in systemic necrotizing vasculitis of the polyarteritis nodosa group. A review of 45 cases . J Rheumatol1995; 22: 78–84 .
49.
Azar N et al. Symptomatic urogenital manifestations of polyarteritis nodosa and Churg–Strauss angiitis . J Urol1989; 142: 136–138 .
50.
Smith DL , Wernick R . Spontaneous rupture of a renal artery aneurysm in polyarteritis nodosa: critical review of the literature and report of a case . Am J Med1989; 87: 464–467 .
51.
Guillevin L et al. Gastro-intestinal tract involvement in polyarteritis nodosa and Churg–Strauss syndrome . Ann Méd Interne (Paris)1995; 146: 260–267 .
52.
Lê Thi Huong et al. Les manifestations digestives de la périartérite noueuse dans une série de 120 case . Gastroentérol Clin Biol1985; 9: 697–703 .
53.
Géneéreau T , Lortholary O , Royer I , Lhote F , Darras-Joly C , Guillevin . Les manifestations digestives de la périartérite noueuse . Gastroentérol Clin Biol1997; 21: 503–510 .
54.
Bleétry O et al. Manifestations cardiaques de la périartérite noueuse . Arch Mal Coeur1980; 73: 1027–1036 .
55.
Lhote F et al. Les manifestations pleurales au cours de la périartérite noueuse, de la maladie de Wegener et du lupus mateux systémique . Ann méd Interne (Paris)1992; 143: 228–232 .
56.
Guillevin et al. Systemic vasculitis of the polyarteritis nodosa group and infections with hepatitis B virus: a study in 98 patients . Eur J Intern Med1990; 1: 97–105 .
57.
Guillevin et al. Polyarteritis nodosa related to hepatitis B virus. A retrospective study of 66 patients . Ann Méd Interne (Paris)1992; 143 (suppl 1): 63–74 .
58.
Piette JC et al. Systemic polyarteritis nodosa diagnosed at hysterectomy . Am J Med1987; 82: 836–838 .
59.
Trépo C et al. The role of circulating hepatitis B antigen/antibody immune complexes in the pathogenesis of vascular and hepatic manifestations in polyarteritis nodosa . J Clin Pathol1974; 27: 863–886 .
Guillevin L , Ronco P , Verroust P . Circulating immune complexes in systemic necrotizing vasculitis of the polyarteritis nodosa group. Comparison of HBV-related polyarteritis nodosa and Churg–Strauss angiitis . J Autoimmun1990; 3: 789–792 .
62.
Guillevin L et al. Antineutrophil cytoplasm antibodies in systemic polyarteritis nodosa with and without hepatitis B virus infection and Churg–Strauss syndrome: 62 patients . J Rheumatol1993; 20: 1345–1349 .
63.
Pusey CD , Gaskin G . Disease association with anti-neutrophil cytoplasmic antibodies. In: Gross WL (ed). ANCA-associated vasculitisPlenum Press: New York and London , 1993, pp 145–155.
64.
Hauschild S et al. ANCA in systemic vasculitis, collagen vascular disorders and inflammatory bowel diseases. In: Gross WL (ed). ANCA-associated vasculitisPlenum Press: New York and London , 1993, pp 245–251.
65.
Bron KM , Strott CA , Shapiro AP . The diagnostic value of angiographic observations in polyarteritis nodosa. A case of multiple aneurysms in the visceral organs . Arch Intern Med1965; 116: 450–454 .
66.
Hekali P et al. Diagnostic significance of angiographically observed visceral naeurysms with regard to polyarteritis nodosa . Acta Radiol1991; 32: 143–148 .
67.
Giullevin L et al. Regressing aneurysms in polyarteritis nodosa related to hepatitis B virus . Eur J Intern Med1990; 1: 267–272 .
68.
Guillevin et al. Antineutrophil cytoplasm antibodies, abnormal angiograms and pathological findings in polyarteritis nodosa and Churg–Strauss syndrome: indications for the classification of vasculitides of the polyarteritis nodosa group . Br J Rheum1996; 35: 958–964 .
69.
Cosserat J et al. Embolies multiples de cholestérol simulant une périartérite noueuse . Presse Méd1992; 21: 557–564 .
70.
Davson J , Ball J , Platt R . The kidney in periarteritis nodosa . Q J Med1948; 17: 175–202 .
71.
Dupré-Goudable C . et al Micropolyartérite . Nephrologie1992; 3: 283–286 .
72.
Wainwright J , Davson J . The renal appearances in the microscopic form of periarteritis nodosaJ Pathol Bacteriol1948; 62: 189–196 .
73.
Haworth SJ et al. Pulmonary haemorrhage complicating Wegener's granulomatosis and microscopic polyarteritis . Br Med J1985; 290: 1775–1778 .
74.
Nada AK et al. Pulmonary fibrosis as an unusual clinical manifestation of a pulmonary-renal vasculitis in elderly patients . Mayo Clin Proc1990; 65: 847–856 .
75.
Matsumoto T et al. The lung is polyarteritis nodosa: a pathologic study of 10 cases . Hum Pathol1993; 24: 717–724 .
Niles JL . Value of test for antineutrophil cytoplasmic autoantibodies in the diagnosis and treatment of vasculitis . Curr Opin Rheumatol1993; 5: 18–24 .
78.
Lhote F , Cohen P , Généreau T , Gayraud M , Guillevin L . Microscopic polyangiitis. Clinical aspects and treatment . Ann Méd Interne (Paris)1996; 147: 165–177 .
79.
Falk RJ et al. Clinical course of anti-neutrophil cytoplasmic autoantibody-associated glomerulonephritis and systemic vasculitis . Ann Intern Med1990; 113: 656–663 .
80.
Csernok E et al. Membrane surface proteinase and intracytoplasmic immunoglobulin on neutrophils from patients with ANCA-associated vaculitides. In: Gross WL (ed). ANCA-associated vasculitisPlenum Press: New York and London , 1993, pp 45–50.
81.
Cohen-Tervaert JW , Elema JD , Kallenberg CGM . Clinical histopathological association of 29kD-ANCA and MPO-ANCA . APMIS1990; 98 (Suppl 19): 35-35 .
82.
Gaskin G , Clutterbuck EJ , Pusey CD . Renal disease in the Churg–Strauss syndrome . Contrib Nephrol1991; 94: 58–65 .
83.
Cohen P , Gayraud M , Lhote F , Jarrousse B , Guillevin L . Possible triggering factors for Churg–Strauss syndrome: a retrospective analysis of 97 patients . Arthritis Rheum1997; 40 (Supplement): S166-S166 (abstract 812).
84.
Phanuphak P , Kohler PF . Onset of polyarteritis nodosa during allergic hyposensitization treatment . Am J Med1980; 68: 479–485 .
85.
Guillevin L . Amouroux J , Arbeille B , Boura R . Churg–Strauss angiitis. Arguments favoring the responsibility of inhaled antigens . Chest1991; 100: 1472–1473 .
86.
Chumbley LC , Harrison EG , De Remee RA . Allergic granulomatosis and angiitis (Churg–Strauss syndrome) . Mayo Clin Proc1977; 52: 477–484 .
87.
Guillevin L , Guittard T , Blétry O , Godeau P , Rosenthal P . Systemic necrotizing angiitis with asthma: causes and precipitating factors in 43 cases . Lung1987; 165: 165–172 .
88.
Hass C et al. L'angéite allergique avec granulomatose: syndrome de Churg et Strauss. Etude reétrospective de 16 observations . Ann Méd Interne (Paris)1991; 142: 335–342 .
89.
Abu-Shakra M et al. Outcome of polyarteritis nodosa and Churg–Strauss syndrome. An analysis of twenty-five patients . Arthritis Rheum1994; 37: 1798–1803 .
90.
Lhote F , Guillevin L . Polyarteritis nodosa, microscopic polyangiitis and Churg–Strauss syndrome . Clinical aspects and treatment. Rheum Dis Clin North Am1995; 21: 911–947 .
91.
Guillevin L et al. Prognostic factors in polyarteritis nodosa and Churg–Strauss syndrome. A prospective study in 342 patients . Medicine1996; 75: 17–28 .
92.
Allen JN , Davis WB . Eosinophilic lung diseases . Am J Respir Crit Care Med1994; 150: 1423–1438 .
93.
Bagenstoss AH , Shick RM , Polley HF . The effect of cortisone on the lesions of periarteritis nodosa [abstract] . Am J Pathol1950; 26: 709-709 .
94.
Fauci AS , Doppman JL , Wolff SM . Cyclophosphamide-induced remission in advanced polyarteritis nodosa . Am J Med1978; 64: 890–894 .
95.
Fauci AS et al. Cyclophosphamide therapy of severe systemic necrotizing vasculitis . New Engl J Med1979; 301: 235–238 .
96.
Guillevin L et al. Long-term follow-up after treatment of periarteritis nodosa and Churg–Strauss angiitis with comparison of steroids, plasma exchanges and cyclophosphamide to steroids and plasma exchanges. A prospective randomized trial of 71 patients . J Rheumatol1991; 18: 567–574 .
97.
Guillevin L et al. Lack of superiority of steroids and plasma exchanges to steroids in the treatment of polyarteritis nodosa and Churg–Strauss angiitis. A prospective randomized trial in 78 patients . Arthritis Rheum1992; 35: 208–215 .
98.
Giullevin L et al. Treatment of polyarteritis nodosa related to hepatitis B virus with short-term steroid therapy associated with antiviral agents and plasma exchanges. A prospective trial in 33 patients . J Rheumatol1993; 20: 289–298 .
99.
Guillevin L et al. Treatment of polyarteritis nodosa related to hepatitis B virus with interferon-alpha and plasma exchanges . Ann Rheum Dis1994; 53: 334–337 .
100.
Guillevin L et al. Corticosteroids plus pulse cyclophosphamide and plasma exchanges versus steroids plus pulse cyclophosphamide alone in the treatment of polyarteritis nodosa and Churg–Strauss syndrome patients with factors predicting poor prognosis. A prospective, randomized trial in sixty-two patients . Arthritis Rheum1995; 38: 1638–1645 .
101.
Gayraud M et al and the French Cooperative Study Group for Vasculitides. Treatment of good-prognosis polyarteritis nodosa and Churg–Strauss syndrome: comparison of steroids and oral or pulse cyclophosphamide in 25 patients . Br J Rheumatol1997; 36: 1290–1297 .
102.
Arlet J . De la corticothérapie classique aÁ l'assaut cortisonique . Ann Méd Interne (Paris)1994; 145 (suppl 2): 5–10 .
103.
Renoux M , Hilliquin P , Menkes CJ . Les bolus de méthylprednisolone en rhumatologie . Ann Méd Interne (Paris)1994; 145: 133–139 .
104.
Stillwell TJ et al. Cyclophosphamide-induced bladder toxicity in Wegener's granulomatosis . Arthritis Rheum1988; 31: 465–470 .
105.
Baker GL et al. Malignancy following treatment of rheumatoid arthritis with cyclophosphamide. Long-term case-control follow-up study . Am J Med1987; 83: 1–9 .
106.
Godeau B et al. Pneumocystis carinii pneumonia in the course of connective tissue disease: report of 34 cases . J Rheumatol1994; 21: 246–251 .
107.
Scott DGI , Bacon PA . Intravenous cyclophosphamide plus methyl prednisolone treatment in systemic rheumatoid vasculitis . Am J Med1984; 76: 377–384 .
108.
De Vita S , Neri R , Bombardieri S . Cyclophosphamide pulses in the treatment of rheumatic diseases: an update . Clin Exp Rheumatol1991; 9: 179–193 .
109.
Généreau T et al. Treatment of systemic vasculitis with cyclophosphamide and steroids: daily oral low dose cyclophosphamide after failure of pulse IV high dose regimen in 4 patients . Br J Rheumatol1994; 33: 959–962 .
110.
Lhote F , Guillevin L . Indications of plasma exchanges in the treatment of polyarteritis nodosa, Churg–Strauss syndrome and other systemic vasculitides . Transfus Sci1996; 17: 211–213 .
111.
Gayraud M , Giullevin L . Cohen P , Jarrousse B . Analysis of long-term follow-up of 278 patients with perarteritis nodosa (PAN) or Churg–Strauss syndrome (CSS) . Arthritis Rheum1997; 40 (Supplement): S166-S166 (abstract 811).
112.
Savage COS , Harper L , Adu D . Primary systemic vasculitis . Lancet1997; 349: 553–558 .
113.
Jayne DR , Rasmussen N for the European Community Systemic Vasculitis Clinical Trial Study Group (ECSYSVASTRIAL) . Treatment of antineutrophil cytoplasm autoantibody-associated systemic vasculitis: initiatives of the European Community systemic vasculitis clinical trials study group . Mayo Clin Proc1997; 72: 737–747 .
114.
Pusey CD et al. Plasma exchange in focal necrotizing glomerulonephritis without anti-GBM antibodies . Kidney Int1991; 40: 757–763 .
115.
Newbuerger JW et al. The treatment of Kawasaki syndrome with intravenous gamma globulin . New Engl J Med1986; 315: 341–347 .
116.
Schifferli JA . Adverse effects of intravenous immunoglobulins . Transfus Sci1992; 13: 331–338 .
117.
Jayne DRW . Intravenous immunoglobulins in the therapy of systemic vasculitis . Transfus Sci1992; 13: 317–324 .
118.
Richter C et al. Treatment of ANCA-associated systemic vasculitis with high-dose intravenous immunoglobulin . Arthritis Rheum1994; 37 (suppl 9): S353-S353 (abstract 1154).
119.
Mathieson PW et al. Monoclonal-antibody therapy in systemic vasculitis . New Engl J Med1990; 323: 250–254 .
