Kaohsiung Journal of Medical Sciences
Volume 24, Issue 10 , Pages 531-535, October 2008

Pauci-Immune Lupus Nephritis: A Case Report

  • Fei-Ching Li

      Affiliations

    • Department of Nephrology, Kaohsiung Medical University Hospital, Kaohsiung, Taiwan
    • ,
  • Daw-Yang Hwang

      Affiliations

    • Department of Nephrology, Kaohsiung Medical University Hospital, Kaohsiung, Taiwan
    • ,
  • Chi-Chih Hung

      Affiliations

    • Department of Nephrology, Kaohsiung Medical University Hospital, Kaohsiung, Taiwan
    • ,
  • Hung-Chun Chen

      Affiliations

    • Department of Nephrology, Kaohsiung Medical University Hospital, Kaohsiung, Taiwan
    • Faculty of Renal Care, College of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan
    • Corresponding Author InformationAddress correspondence and reprint requests to: Dr Hung-Chun Chen, Department of Nephrology, Kaohsiung Medical University Hospital, 100 Tzyou 1st Road, Kaohsiung 807, Taiwan

Received 31 December 2007; accepted 20 February 2008.

Article Outline

A 26-year-old female with systemic lupus erythematosus was admitted because of dyspnea and progressive lower extremity edema. Laboratory testing showed blood urea nitrogen levels of 147 mg/dL, creatinine of 6.7 mg/dL, serum albumin of 1.7 g/dL and the daily protein loss was 12.7 g. Her C3 level was 60.4 mg/dL and C4 level was 10.2 mg/dL. The antinuclear antibody titer was 1:320, with a homogeneous pattern, but she was negative for anti-dsDNA. ELISA testing for anti-PR3 antibodies and anti-MPO antibodies were all negative. She was also negative for circulating lupus anticoagulant. Renal biopsy revealed diffuse proliferation of glomerular cells, but immunofluorescent microscopy showed no immune deposits and electron microscopy revealed only scanty electron-dense deposits. She received 1 g/day of methylprednisolone intravenously for 3 days, followed by 60 mg/day of prednisolone. She was discharged with serum creatinine decreased to 4.7mg/dL, and a great improvement in dyspnea. Diffuse proliferative lupus nephritis that contains little or no subendothelial deposits is rare. The differential diagnosis, possible mechanisms and treatment are discussed.

Key Words:  crescentic glomerulonephritis , lupus nephritis , pauci-immune glomerulonephritis , systemic lupus erythematosus

No full text is available. To read the body of this article, please view the PDF online.

 

Back to Article Outline

References 

  1. Waldman M , Madaio MP . Pathogenic autoantibodies in lupus nephritis . Lupus . 2005;14:19–24
  2. Lewis EJ , Schwartz MM . Pathology of lupus nephritis . Lupus . 2005;14:31–38
  3. Akhtar M , al-Dalaan A , el-Ramahi KM . Pauci-immune necrotizing lupus nephritis: report of two cases . Am J Kidney Dis . 1994;23:320–325
  4. The nephropathies of systemic lupus erythematosus and other rheumatologic disorders . In:  William FK ,  Fred GS ,  Zoltan GL , et al. editor. Atlas of Renal Pathology . Philadelphia: W.B. Saunders; 1999;p. 78–96
  5. Charney DA , Nassar G , Truong L , et al.   “Pauci-immune” proliferative and necrotizing glomerulonephritis with thrombotic microangiopathy in patients with systemic lupus erythematosus and lupus-like syndrome . Am J Kidney Dis . 2000;35:1193–1206
  6. Schwartz MM , Roberts JL , Lewis EJ . Necrotizing glomerulitis of systemic lupus erythematosus . Hum Pathol . 1983;14:158–167
  7. Savige J , Davies D , Falk RJ , et al.   Antineutrophil cytoplasmic antibodies and associated diseases: a review of the clinical and laboratory features . Kidney Int . 2000;57:846–862
  8. Little MA , Pusey CD . Glomerulonephritis due to antineutrophil cytoplasm antibody-associated vasculitis: an update on approaches to management . Nephrology (Carlton) . 2005;10:368–376
  9. Cunningham MA , Huang XR , Dowling JP , et al.   Prominence of cell-mediated immunity effectors in “pauci-immune” glomerulonephritis . J Am Soc Nephrol . 1999;10:499–506
  10. Couser WG . Sensitized cells come of age: a new era in renal immunology with important therapeutic implications . J Am Soc Nephrol . 1999;10:664–665

PII: S1607-551X(09)70012-9

doi:10.1016/S1607-551X(09)70012-9

Kaohsiung Journal of Medical Sciences
Volume 24, Issue 10 , Pages 531-535, October 2008

Article Tools