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Life-Threatening Complications of Systemic Lupus Erythematosus

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Autoimmune Diseases
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Abstract

The major cause of death in longstanding lupus is accelerated atherosclerosis leading to cardiovascular disease. Early in lupus, the major causes of death are active lupus and infection. Life-threatening or organ-threatening lupus is not rare. Multiple organs including skin (necrosis), hematologic (thrombocytopenia, hemolytic anemia, neutropenia, catastrophic antiphospholipid syndrome, and thrombotic thrombocytopenic purpura), heart (pericardial tamponade, myocarditis), lung (alveolar hemorrhage, pulmonary hypertension), gastrointestinal (vasculitis, pancreatitis), adrenal insufficiency, and neurologic (myelitis) may be encountered.

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References

  1. Liu A, Zhang W, Tian X, Zhang X, Zhang F, Zeng X. Prevalence, risk factors and outcome of digital gangrene in 2684 lupus patients. Lupus. 2009;18(12):1112-1118.

    Article  PubMed  CAS  Google Scholar 

  2. Frances C, Tribout B, Boisnic S. Cutaneous necrosis associated with the lupus anticoagulant. Dermatologica. 1989;178:194-201.

    Article  PubMed  CAS  Google Scholar 

  3. Tan EM, Cohen AS, Fries JF, et al. The 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum. 1982;25:1271-1277.

    Article  PubMed  CAS  Google Scholar 

  4. Alba P, Karim MY, Hunt BJ. Mycophenolate mofetil as a treatment for autoimmune haemolytic anaemia in patients with systemic lupus erythematosus and antiphospholipid syndrome. Lupus. 2003;12(8):633-635.

    Article  PubMed  CAS  Google Scholar 

  5. Cervera H, Jara LJ, Pizarro S, et al. Danazol for systemic lupus erythematosus with refractory autoimmune thrombocytopenia or Evans’ syndrome. J Rheumatol. 1995;22(10):1867-1871.

    PubMed  CAS  Google Scholar 

  6. Abdwani R, Mani R. Anti-CD20 monoclonal antibody in acute life threatening haemolytic anaemia complicating childhood onset SLE. Lupus. 2009;18(5):460-464.

    Article  PubMed  CAS  Google Scholar 

  7. Gomard-Mennesson E, Ruivard M, Koenig M, et al. Treatment of isolated severe immune hemolytic anaemia associated with systemic lupus erythematosus: 26 cases. Lupus. 2006;15(4):223-231.

    Article  PubMed  CAS  Google Scholar 

  8. Gruenberg JC, VanSlyck EJ, Abraham JP. Splenectomy in systemic lupus erythematosis. Am Surg. 1986;52(7):366-370.

    PubMed  CAS  Google Scholar 

  9. Keeling DM, Isenberg DA. Haematological manifestations of systemic lupus erythematosus. Blood Rev. 1993;7(4):199-207.

    Article  PubMed  CAS  Google Scholar 

  10. Martinez-Banos D, Crispin JC, Lazo-Langner A, Sanchez-Guerrero J. Moderate and severe neutropenia in patients with systemic lupus erythematosus. Rheumatology (Oxford). 2006;45(8):994-998.

    Article  CAS  Google Scholar 

  11. Vasiliu IM, Petri MA, Baer AN. Therapy with granulocyte colony-stimulating factor in ­systemic lupus erythematosus may be associated with severe flares. J Rheumatol. 2006;33(9):1878-1880.

    PubMed  Google Scholar 

  12. Asherson RA, Cervera R, Piette JC, et al. Catastrophic antiphospholipid syndrome. Clinical and laboratory features of 50 patients. Medicine. 1998;77(3):195-207.

    Article  PubMed  CAS  Google Scholar 

  13. Asherson RA, Cervera R, de Groot PG, et al. Catastrophic antiphospholipid syndrome: international consensus statement on classification criteria and treatment guidelines. Lupus. 2003;12(7):530-534.

    Article  PubMed  CAS  Google Scholar 

  14. Asherson RA, Cervera R, Font J. Multiorgan thrombotic disorders in systemic lupus erythematosus: a common link? Lupus. 1992;1(4):199-203.

    Article  PubMed  CAS  Google Scholar 

  15. Musio F, Bohen EM, Yuan CM, Welch PG. Review of thrombotic thrombocytopenic purpura in the setting of systemic lupus erythematosus. Semin Arthritis Rheum. 1998;28(1):1-19.

    Article  PubMed  CAS  Google Scholar 

  16. Kwok SK, Ju JH, Cho CS, Kim HY, Park SH. Thrombotic thrombocytopenic purpura in ­systemic lupus erythematosus: risk factors and clinical outcome: a single centre study. Lupus. 2009;18(1):16-21.

    Article  PubMed  CAS  Google Scholar 

  17. Nesher G, Hanna VE, Moore TL, Hersh M, Osborn TG. Thrombotic microangiopathic hemolytic anemia in systemic lupus erythematosus. Semin Arthritis Rheum. 1994;24:165-172.

    Article  PubMed  CAS  Google Scholar 

  18. Miyamura T, Watanabe H, Takahama S, et al. Thrombotic thrombocytopenic purpura in patients with systemic lupus erythematosus. Nihon Rinsho Meneki Gakkai Kaishi. 2008;31(3):159-165.

    PubMed  Google Scholar 

  19. Letchumanan P, Ng HJ, Lee LH, Thumboo J. A comparison of thrombotic thrombocytopenic purpura in an inception cohort of patients with and without systemic lupus erythematosus. Rheumatology (Oxford). 2009;48(4):399-403.

    Article  Google Scholar 

  20. Rosenbaum E, Krebs E, Cohen M, Tiliakos A, Derk CT. The spectrum of clinical manifestations, outcome and treatment of pericardial tamponade in patients with systemic lupus erythematosus: a retrospective study and literature review. Lupus. 2009;18(7):608-612.

    Article  PubMed  CAS  Google Scholar 

  21. Kahl LE. The spectrum of pericardial tamponade in systemic lupus erythematosus: report of ten patients. Arthritis Rheum. 1992;35:1343-1349.

    PubMed  CAS  Google Scholar 

  22. Badui E, Garcia-Rubi D, Robles E, et al. Cardiovascular manifestations in systemic lupus erythematosus. Prospective study of 100 patients. Angiology. 1985;36:431-441.

    Article  PubMed  CAS  Google Scholar 

  23. Borenstein DG, Fye WB, Arnett FC, Stevens MB. The myocarditis of systemic lupus erythematosus: association with myositis. Ann Intern Med. 1978;89:619-624.

    PubMed  CAS  Google Scholar 

  24. Fairfax MJ, Osborn TG, Williams GA, Tsai CC, Moore TL. Endomyocardial biopsy in patients with systemic lupus erythematosus. J Rheumatol. 1988;15:593-596.

    PubMed  CAS  Google Scholar 

  25. Gottenberg JE, Roux S, Assayag P, Clerc D, Mariette X. Specific cardiomyopathy in lupus patients: report of three cases. Joint Bone Spine. 2004;71(1):66-69.

    Article  PubMed  Google Scholar 

  26. Law WG, Thong BY, Lian TY, Kong KO, Chng HH. Acute lupus myocarditis: clinical features and outcome of an oriental case series. Lupus. 2005;14(10):827-831.

    Article  PubMed  CAS  Google Scholar 

  27. Koh WH, Thumboo J, Boey ML. Pulmonary haemorrhage in Oriental patients with systemic lupus erythematosus. Lupus. 1997;6(9):713-716.

    Article  PubMed  CAS  Google Scholar 

  28. Canas C, Tobon GJ, Granados M, Fernandez L. Diffuse alveolar hemorrhage in Colombian patients with systemic lupus erythematosus. Clin Rheumatol. 2007;26(11):1947-1949.

    Article  PubMed  Google Scholar 

  29. Zamora MR, Warner ML, Tuder R, Schwarz MI. Diffuse alveolar hemorrhage and systemic lupus erythematosus. Clinical presentation, histology, survival, and outcome. Medicine. 1997;76(3):192-202.

    Article  PubMed  CAS  Google Scholar 

  30. Chung SM, Lee CK, Lee EY, Yoo B, Lee SD, Moon HB. Clinical aspects of pulmonary hypertension in patients with systemic lupus erythematosus and in patients with idiopathic pulmonary arterial hypertension. Clin Rheumatol. 2006;25(6):866-872.

    Article  PubMed  Google Scholar 

  31. Kamata Y, Iwamoto M, Minota S. Consecutive use of sildenafil and bosentan for the treatment of pulmonary arterial hypertension associated with collagen vascular disease: sildenafil as reliever and bosentan as controller. Lupus. 2007;16(11):901-903.

    Article  PubMed  CAS  Google Scholar 

  32. Makol A, Petri M. Pancreatitis in systemic lupus erythematosus: frequency and associated factors – a review of the Hopkins Lupus Cohort. J Rheumatol. 2010;37(2):341-345.

    Article  PubMed  Google Scholar 

  33. Serrano Lopez MC, Yebra-Bango M, Lopez Bonet E. Acute pancreatitis and systemic lupus erythematosus: necropsy of a case and review of pancreatic vascular lesions. Am J Gastroenterol. 1991;86:764-767.

    PubMed  CAS  Google Scholar 

  34. Espinosa G, Santos E, Cervera R, et al. Adrenal involvement in the antiphospholipid syndrome: clinical and immunologic characteristics of 86 patients. Medicine. 2003;82(2):106-118.

    Article  PubMed  Google Scholar 

  35. Chen SY, Xu JH, Shuai ZW, et al. A clinical analysis 30 cases of lupus mesenteric vasculitis. Zhonghua Nei Ke Za Zhi. 2009;48(2):136-139.

    PubMed  Google Scholar 

  36. Zizic TM, Classen JN, Stevens MB. Acute abdominal complications of systemic lupus erythematosus and polyarteritis nodosa. Am J Med. 1982;73:525-531.

    Article  PubMed  CAS  Google Scholar 

  37. Ko SF, Lee TY, Cheng TT, et al. CT findings at lupus mesenteric vasculitis. Acta Radiol. 1997;38(1):115-120.

    PubMed  CAS  Google Scholar 

  38. Birnbaum J, Petri M, Thompson R, Izbudak I, Kerr D. Distinct subtypes of myelitis in ­systemic lupus erythematosus. Arthritis Rheum. 2009;60(11):3378-3387.

    Article  PubMed  CAS  Google Scholar 

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Correspondence to Michelle Petri .

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Petri, M. (2011). Life-Threatening Complications of Systemic Lupus Erythematosus. In: Khamashta, M., Ramos-Casals, M. (eds) Autoimmune Diseases. Springer, London. https://doi.org/10.1007/978-0-85729-358-9_2

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  • DOI: https://doi.org/10.1007/978-0-85729-358-9_2

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