Abstract
The inflammatory process of systemic lupus erythematosus (SLE) vasculitis occurs in up to 56% of patients with SLE, whereas the vaso-occlusive incidence of antiphospholipid syndrome in SLE (which is not exclusive of SLE vasculitis) reaches up to 15%. Both situations can be life-threatening, requiring the appropriate therapeutic approach. Aggressive anti-inflammatory therapy is used in the case of SLE vasculitis and aggressive antithrombotic/anticoagulant treatment in the other. However, often the differential diagnosis between the two entities is difficult to achieve, especially when a tissue biopsy to help establish the diagnosis is topographically difficult to obtain or is clinically contraindicated. Such situations are discussed and appropriate guidelines for a correct diagnosis suggested.
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References and Recommended Reading
Drenkard C, Villa AR, Reyes E, et al.: Vasculitis in systemic lupus erythematosus. Lupus 1997, 6:235–242. An important study of SLE vasculitis in a large cohort of patients.
Perez-Vazquez ME, Villa AR, Drenkard C, et al.: Influence of disease duration, continued follow-up and further antiphospholipid testing on the frequency and classification category of antiphospholipid syndrome in a cohort of patients with SLE. J Rheumatol 1993, 20:437–442.
Simioni P, Prandoni P, Zanon E, et al.: Deep venous thrombosis and lupus anticoagulant. A case-control study. Haemost Thromb 1996, 76:187–189.
Gladman DD, Urowitz MB: Systemic lupus erythematosus: clinical features. In Rheumatology. Edited by Klippel JH, Dieppe PA. London: Mosby; 1998:1–18.
Flores-Suarez LF, Alarcon-Segovia D: Vasculitis in connective tissue diseases. Curr Rheumatol Reports 2000, 2:396–401.
Cuellar MC, Espinoza LR: Laboratory testing in the evaluation and diagnosis of vasculitis. Curr Rheumatol Reports 2000, 2:417–422. A very good presentation of the wide range of clinical tests available for the diagnosis of vasculitis, and their use in its differential diagnosis.
Sundy JS, Haynes BF: Cytokines and adhesion molecules in the pathogenesis of vasculitis. Curr Rheumatol Reports 2000, 2:402–410.
Carvvalho D, Savage COS, Isenberg D, Pearson JD: IgG antiendothelial cell autoantibodies from patients with SLE stimulate the release of two endothelial cell-derived mediators manner, which enhance adhesion molecule expression and leukocyte adhesion in an autocrine manner. Arthritis Rheum 1999, 42:631–640.
Moore PM: Vasculitis of the central nervous system. Curr Rheumatol Reports 2000, 2:376–382.
Shintani S, Ono K, Hinoshita H, et al.: Unusual neuroradiological findings in systemic lupus erythematosus. Eur Neurol 1993, 33:13–16.
Ko SF, Lee TY, Cheng TT, et al.: CT findings at lupus mesenteric vasculitis. Acta Radiol 1997, 38:115–120.
Harris KG, Tran DD, Sickels WJ, et al.: Diagnosing intracranial vasculitis: the role of MR and angiography. Am J Neuroradiol 1994, 15:317–330.
Imbes SG: Diffuse cerebral vasculitis with normal results on brain MR imaging. Am J Roentgenol 1999, 173:1494–1496.
Cloft HJ, Philips CD, Dix JE, et al.: Correlation of angiography and MR imaging in cerebral vasculitis. Acta Radiol 1999, 40:83–87.
Yuh WT, Ueda T, Maley JE, et al.: Diagnosis of microvasculopathy in CNS vasculitis: value of perfusion and diffusion. J MRI 1999, 10:310–313.
Stenson AW: Roentgenographic findings in major vasculitis syndromes. Rheum Dis Clin North Am 1990, 16:293–308.
Boey ML, Colaco CB, Gharavi AE, et al.: Thrombosis in SLE: striking association with the presence of circulating lupus anticoagulant. Br Med J 1983, 287:1021–1023.
Hughes GR: Thrombosis, abortion, cerebral disease and the lupus anticoagulant. Br Med J 1983, 287:1088–1089.
Hughes GR: The anticardiolipin syndrome. Clin Exp Rheumatol 1985, 3:285–286.
Wilson WA, Gharavi EA, Koike T, et al.: International consensus statement on preliminary classification criteria for definite antiphospholipid syndrome. Report of an International Workshop. Arthritis Rheum 1999, 42:1309–1311. This is the formal paper on which the diagnosis of definite APS is based.
Godfrey T, D’Cruz D: Antiphospholipid syndrome: General features. In Hughes Syndrome. Edited by Khamashta MA. London: Springer; 2000:8–19.
Levine SR, Brey RL, Sawaya KL, et al.: Recurrent stroke and thrombo-occlusive events in the antiphospholipid syndrome. Ann Neurol 1995, 38:119–124.
Navarrete MG, Brey RL, Levine SR: Cerebral disease in the antiphospholipid syndrome. In Hughes Syndrome. Edited by Khamashta MA. London, Springer; 2000:43–58. A very good review about clinical manifestations of cerebral disease in APS with emphasis on its differential diagnosis.
Shah NM, Khamashta MA, Atsumi T, Hughes GVR: Outcome of patients with anticardiolipin antibodies: a 10 year follow-up of 52 patients. Lupus 1998, 7:3–6.
Schwartz M, Rochas M, Weller B, et al.: High association of anti-cardiolipin antibodies with psychosis. J Clin Psychiatry 1998, 59:20–23.
Wolkowitz OM, Reus VI, Canick J, et al.: Glucocorticoid medication, memory and steroid psychosis in medical illness. Ann NY Acad Sci 1997, 823:81–96.
Specker C, Siebler M, Schneider M: Imaging of microemboli in antiphospholipid syndrome. In Hughes Syndrome. Edited by Khamashta MA. London: Springer; 2000:195–200.
Toubi E, Khamashta MA, Pannara A, Hughes GRV: Association of antiphospholipid antibodies with central nervous system disease in SLE. Am J Med 1995, 99:397–401.
Sailer M, Burchert W, Ehrenheim C, et al.: Positron emission tomography and magnetic resonance imaging for cerebral involvement in patients with SLE. J Neurol 1997, 244:186–193.
Battagliotti CA: Skin manifestations of the antiphospholipid antibody syndrome. In Hughes Syndrome. Edited by Khamashta MA. London: Springer; 2000:59–69. A very well-illustrated review of various forms of skin manifestations in APS.
Amigo MC, Garcia-Torres R: Kidney disease in antiphospholipid syndrome. In Hughes Syndrome. Edited by Khamashta MA. London: Springer; 2000:70–81. A very good classification of renal involvement in APS and its clinical and histopathologic manifestations.
Nochy D, Daugas E, Droz D, et al.: The intrarenal vascular lesions associated with primary antiphospholipid syndrome. J Am Soc Nephrol 1999, 10:507–518.
Font J, Cervera R: Cardiac manifestations in the antiphospholipid syndrome. In Hughes Syndrome. Edited by Khamashta MA. London: Springer; 2000:32–42. A very good presentation of the cardiac involvement in APS, with emphasis on its differential diagnosis.
Ziporen L, Goldberg I, Arad M, et al.: Libman-Sacks endocarditis in the antiphospholipid syndrome: immunopathogenic findings in deformed heart valves. Lupus 1996, 5:196–205.
Amital H, Langevitz P, Levy Y, et al.: Valvular deposition of antiphospholipid antibodies in the antiphospholipid syndrome: A clue to the origin of the disease. Clin Exp Rheumatol 1999, 17:99–102.
Pierangeli SS, Gharavi AE, Harris EN: Anticardiolipin testing. In Hughes Syndrome. Edited by Khamashta MA. London: Springer; 2000:205–213.
Montecucco C, Caporali R: Hemocytopenias in antiphospholipid syndrome. In Hughes Syndrome. Edited by Khamashta MA. London: Springer; 2000:20–31.
Gharavi AE, Pierangeli SS: Infections and antiphospholipid antibodies. In Hughes Syndrome. Edited by Khamashta MA. London: Springer; 2000:135–143. An important overview on the significance of postinfectious aCL-positive findings and their clinical implications.
Sammaritano LR: Drug-induced antiphospholipid antibodies. In Hughes Syndrome. Edited by Khamashta MA. London: Springer; 2000:144–154.
Mackie IJ, Donohoe S, Machin SJ: Lupus anticoagulant measurements. In Hughes Syndrome. Edited by Khamashta MA. London: Springer; 2000:214–224.
Lie JT: Vasculopathy of the antiphospholipid syndrome revisited: thrombosis is the culprit and vasculitis the consort. Lupus 1996, 5:368–371.
Kim JH, Ha HK, Yoon KH, et al.: CT features of abdominal manifestations of primary antiphospholipid syndrome. J Comput Assist Tomog 1999, 5:678–683.
Isshi K, Hirohata S: Differential roles of the anti-ribosomal P antibody and antineuronal antibody in the pathogenesis of central nervous system involvement in systemic lupus erythematosus. Arthritis Rheum 1998, 41:1819–1827.
West SG, Emlen W, Wener MH, Kotzin BL: Neuropsychiatric lupus erythematosus: a 10-year prospective study on the value of diagnostic tests. Am J Med 1995, 99:153–162.
Georgescu L, Mevorach D, Arnett FC, et al.: Anti-P antibodies and neuropsychiatric lupus erythematosus. Ann NY Acad Sci 1997, 823:263–269.
Belmont HM: Catastrophic antiphospholipid syndrome. In Hughes Syndrome. Edited by Khamashta MA. London: Springer; 2000:27–134.
Asherson RA: The catastrophic antiphospholipid syndrome, 1998. A review of the clinical features, possible pathogenesis and treatment. Lupus 1998, 7:S55–62.
Roubey RAS: Antiphospholipid antibody-negative syndrome: Other phospholipids. In Hughes Syndrome. Edited by Khamashta MA. London: Springer; 2000:253–260.
Hunt BJ, Ames PRJ: Antiphospholipid syndrome: differential diagnosis. In Hughes Syndrome. Edited by Khamashta MA. London: Springer; 2000:449–456. An important overview about the differential diagnosis of APS with other thromboembolic (venous and arterial) risk factors.
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Golan, T.D. Lupus vasculitis: Differential diagnosis with antiphospholipid syndrome. Curr Rheumatol Rep 4, 18–24 (2002). https://doi.org/10.1007/s11926-002-0019-8
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DOI: https://doi.org/10.1007/s11926-002-0019-8