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Successful treatment of fulminant pulmonary hemorrhage associated with systemic lupus erythematosus

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Abstract

We report a patient with systemic lupus erythematosus (SLE) who developed fulminant pulmonary hemorrhage. This patient also showed liver dysfunction, bicytopenia and hyperferritinemia, with an increase in serum levels of interleukin (IL)-1β, IL-6 and tumor necrosis factor-α (TNF-α) at the onset of pulmonary symptoms, probably indicating an associated hemophagocytic syndrome. Despite an acute progressive course temporarily requiring mechanical ventilation the patient was successfully treated with continuous drip infusion of tacrolimus, plasmapheresis and intravenous high-dose immunoglobulin and corticosteroid. In this patient increased inflammatory cytokines ascribable to activation of macrophages and/or helper T cells were considered to play an important role in the pathogenesis of the pulmonary hemorrhage. Because this complication is frequently fatal in SLE, intensive therapy, including immunosuppressants and plasmapheresis, should be actively considered as early as possible after onset.

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Abbreviations

HPS:

Hemophagocytic syndrome

SLE:

Systemic lupus erythematosus

TNF:

Tumor necrosis factor

References

  1. D’Cruz D, Khamashta M, Hughes G (2002) Pulmonary manifestations of systemic lupus erythematosus. In: DJ Wallace et al (eds) Dubois’ lupus erythematosus, 6th edn. Lippincott Williams & Wilkins, Philadelphia, pp 663–683

  2. Domingo-Pedrol P, Rodriguez de la Serna A, Mancebo-Cortes J, Sanchez-Segura JM (1985) Adult respiratory distress syndrome caused by acute systemic lupus erythematosus. Eur J Respir Dis 67:141–144

    CAS  PubMed  Google Scholar 

  3. Asherson RA, Ridley M, Fletcher CD, Hughes GR (1988) Systemic lupus erythematosus, pulmonary hypertension and adult respiratory distress syndrome (ARDS). Clin Exp Rheumatol 6:301–304

    CAS  PubMed  Google Scholar 

  4. Kim WU, Kim SI, Yoo WH et al. (1999) Adult respiratory distress syndrome in systemic lupus erythematosus: causes and prognostic factors: a single center, retrospective study. Lupus 8:552–557

    Article  CAS  PubMed  Google Scholar 

  5. Hochberg MC (1997) Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 40:1725

    CAS  Google Scholar 

  6. Hallegua DS, Wallace DJ (2002) Gastrointestinal and hepatic manifestations. In: DJ Wallace et al (eds) Dubois’ lupus erythematosus, 6th edn. Lippincott Williams & Wilkins, Philadelphia, pp 843–861

  7. Henter J-I, Elinder G, Öst Å (1999) Diagnostic guidelines for hemophagocytic lymphohistiocytosis. Semin Oncol 18:29–33

    Google Scholar 

  8. Ravelli A (2002) Macrophage activation syndrome. Curr Opin Rheumatol 14:548–552

    Article  CAS  PubMed  Google Scholar 

  9. Green RJ, Ruoss SJ, Kraft SA, Duncan SR, Berry GJ, Raffin TA (1996) Pulmonary capillaritis and alveolar hemorrhage. Update on diagnosis and management. Chest 110:1305–1316

    CAS  PubMed  Google Scholar 

  10. Jara LJ, Vera-Lastra O, Calleja MC (2003) Pulmonary-renal vasculitic disorders: differential diagnosis and management. Curr Rheumatol Rep 5:107–115

    PubMed  Google Scholar 

  11. Lauqua D, Cadranel J, Lazor R et al. (2000) Microscopic polyangiitis with alveolar hemorrhage. A study of 29 cases and review of the literature. Medicine (Baltimore) 79:222–233

  12. Eagen JW, Memoli VA, Roberts JL, Matthew GR, Schwartz MM, Lewis EJ (1978) Pulmonary hemorrhage in systemic lupus erythematosus. Medicine (Baltimore) 57:545–560

    Google Scholar 

  13. Moss M, Ingram RH Jr (2001) Acute respiratory distress syndrome. In: E Braunwald et al. (eds) Harrison’s principles of internal medicine, 15th edn. McGraw-Hill, New York, pp 1523–1526

  14. Lewis EJ, Hunsicker LG, Lan SP, Rohde RD, Lachin JM (1992) A controlled trial of plasmapheresis therapy in severe lupus nephritis. N Engl J Med 326:1373–1379

    PubMed  Google Scholar 

  15. Erickson RW, Franklin WA, Emlen W (1994) Treatment of hemorrhagic lupus pneumonitis with plasmapheresis. Semin Arthritis Rheum 24:114–123

    CAS  PubMed  Google Scholar 

  16. Dalakas MC (1998) Mechanism of action of intravenous immunoglobulin and therapeutic considerations in the treatment of autoimmune neurologic diseases. Neurology 51(Suppl): S2–8

    CAS  Google Scholar 

  17. Kilian K, Banyai A, Karadi A, Miklos K, Patranyi GG, Paloczi K (1998) FK-506 (tacrolimus) therapy for an unusual SLE-like disease. Transplant Proc 30:4130–4131

    Article  CAS  PubMed  Google Scholar 

  18. Griffiths B, Emery P (2001) The treatment of lupus with cyclosporin A. Lupus 10:165–170

    Article  CAS  PubMed  Google Scholar 

  19. Manger K, Kalden JR, Manger B (1996) Cyclosporin A in the treatment of systemic lupus erythematosus: results of an open clinical study. Br J Rheumatol 35:669–675

    CAS  PubMed  Google Scholar 

  20. Schreiber SL, Crabtree GR (1992) The mechanism of action of cyclosporin A and FK506. Immunol Today 13:136–142

    PubMed  Google Scholar 

  21. Moon JI, Park SG, Cheon KO (2000) Pregnancy in renal transplant patients. Transplant Proc 32:1869–1870

    Article  CAS  PubMed  Google Scholar 

  22. Yasuhara M, Hashida T, Toraguchi M et al. (1995) Pharmacokinetics and pharmacodynamics of FK506 in pediatric patients receiving living-related donor liver transplantation. Transplantation Proc 27:1108–1110

    CAS  Google Scholar 

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Authors and Affiliations

  1. Third Department of Medicine, Shinshu University School of Medicine, 3–1-1 Asahi, 390–8621, Matsumoto, Japan

    Ken-ichi Hoshi, Masayuki Matsuda, Mariko Ishikawa, Shigeaki Mitsuhashi, Takahisa Gono, Takao Hashimoto & Shu-ichi Ikeda

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  1. Ken-ichi Hoshi
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  2. Masayuki Matsuda
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  3. Mariko Ishikawa
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  4. Shigeaki Mitsuhashi
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  5. Takahisa Gono
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  6. Takao Hashimoto
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  7. Shu-ichi Ikeda
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Correspondence to Masayuki Matsuda.

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Hoshi, Ki., Matsuda, M., Ishikawa, M. et al. Successful treatment of fulminant pulmonary hemorrhage associated with systemic lupus erythematosus. Clin Rheumatol 23, 252–255 (2004). https://doi.org/10.1007/s10067-003-0859-2

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  • DOI: https://doi.org/10.1007/s10067-003-0859-2

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