J Rheum Dis.  2015 Feb;22(1):34-38. 10.4078/jrd.2015.22.1.34.

A Case of True Renal Lupus Vasculitis Combined with Pauci-immune Glomerulonephritis in a Patient with Systemic Lupus Erythematosus

• Park EJ
• Kim S
• Kim H
• Kim J

• KMID: 2222925
• DOI: http://doi.org/10.4078/jrd.2015.22.1.34

Abstract

Renal lupus vasculitis is a rare vascular lesion complicated with systemic lupus erythematosus (SLE). We report an unusual case of true renal lupus vasculitis with antineutrophil cytoplasmic antibodies (ANCA)-negative pauci-immune glomerulonephritis in a patient with SLE. A 32-year-old woman presenting with hematuria and overt proteinuria was admitted to the hospital. She had been diagnosed with SLE at 16 years of age and treated with prednisolone, hydroxychloroquine, and methotraxate. A kidney biopsy revealed 42 glomeruli with ischemic wrinkling, and segmental loop necrosis with fibrin deposition. Prominent inflammatory cell infiltration of interlobular arteries and afferent arterioles with severe necrosis was demonstrated. No electron-dense and immune deposits in the glomeruli were observed by immunofluorescent and electron microscopy; in contrast, those in the renal vascular wall showed a full-house pattern. Antiphospholipid antibodies and ANCA were negative. The patient was treated with monthly intravenous cyclophosphamide pulses and high dose steroid, and showed good response on further follow-up.

Keyword

True renal lupus vasculitis; Pauci-immune glomerulonephritis; Systemic lupus erythematosus

MeSH Terms

Adult
Antibodies, Antineutrophil Cytoplasmic
Antibodies, Antiphospholipid
Arteries
Arterioles
Biopsy
Cyclophosphamide
Female
Fibrin
Follow-Up Studies
Glomerulonephritis*
Hematuria
Humans
Hydroxychloroquine
Kidney
Lupus Erythematosus, Systemic*
Microscopy, Electron
Necrosis
Prednisolone
Proteinuria
Vasculitis*
Antibodies, Antineutrophil Cytoplasmic
Antibodies, Antiphospholipid
Cyclophosphamide
Fibrin
Hydroxychloroquine
Prednisolone

Figure

• 

  Figure 1. Pauci-immune glomerulonephritis. (A) Glomeruli show segmental necrosis with fibrinoid deposits and (B) ischemic wrinkling (H&E; A: ×400, B: ×200). (C) Immunofluoresence demonstrates absence of significant immune-complex deposits (×400).

• 

  Figure 2. True renal lupus vasculitis. (A) Interlobular arteries shows prominent and transmural inflammatory cell infiltration with (B) severe necrosis and fibrinoid deposits (A, B: H&E, ×400). (C) Electron-dense deposits demonstrated in arteriolar wall (electromicroscopy, ×3,000).

• 

  Figure 3. A full-house pattern immune-complex deposits in vessel wall including (A) immunoglobulin G (×400), (B) immunoglobulin M (×400), and (C) C1q, by immunofluoresence microscopy (×400).

Reference

1. Cameron JS. Lupus nephritis: an historical perspective 1968-1998. J Nephrol. 1999; 12(Suppl 2):S29–41.

2. Banfi G, Bertani T, Boeri V, Faraggiana T, Mazzucco G, Monga G, et al. Renal vascular lesions as a marker of poor prognosis in patients with lupus nephritis. Gruppo Italiano per lo Studio della Nefrite Lupica (GISNEL). Am J Kidney Dis. 1991; 18:240–8.

3. Wu LH, Yu F, Tan Y, Qu Z, Chen MH, Wang SX, et al. Inclusion of renal vascular lesions in the 2003 ISN/RPS system for classifying lupus nephritis improves renal outcome predictions. Kidney Int. 2013; 83:715–23.

4. Grishman E, Venkataseshan VS. Vascular lesions in lupus nephritis. Mod Pathol. 1988; 1:235–41.

5. Appel GB, Pirani CL, D'Agati V. Renal vascular complications of systemic lupus erythematosus. J Am Soc Nephrol. 1994; 4:1499–515.

6. Gelfand J, Truong L, Stern L, Pirani CL, Appel GB. Thrombotic thrombocytopenic purpura syndrome in systemic lupus erythematosus: treatment with plasma infusion. Am J Kidney Dis. 1985; 6:154–60.

7. Nasr SH, D'Agati VD, Park HR, Sterman PL, Goyzueta JD, Dressler RM, et al. Necrotizing and crescentic lupus nephritis with antineutrophil cytoplasmic antibody seropositivity. Clin J Am Soc Nephrol. 2008; 3:682–90.

8. Sen D, Isenberg DA. Antineutrophil cytoplasmic autoantibodies in systemic lupus erythematosus. Lupus. 2003; 12:651–8.

9. Tan EM, Cohen AS, Fries JF, Masi AT, McShane DJ, Rothfield NF, et al. The 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum. 1982; 25:1271–7.

10. Pan HF, Fang XH, Wu GC, Li WX, Zhao XF, Li XP, et al. Anti-neutrophil cytoplasmic antibodies in new-onset systemic lupus erythematosus and lupus nephritis. Inflammation. 2008; 31:260–5.

11. Marshall S, Dressler R, D'Agati V. Membranous lupus nephritis with antineutrophil cytoplasmic antibody-associated segmental necrotizing and crescentic glomerulonephritis. Am J Kidney Dis. 1997; 29:119–24.

12. Moeckel GW, Jabs K, Fogo AB. An 11-year-old African-American girl with systemic lupus erythematosus and ANCA-negative renal vasculitis. Am J Kidney Dis. 2002; 39:433–8.

13. Benz K, Büttner M, Rosenthal K, Amann K. Lupus nephritis and fibrinoid vasculitis of the kidney: a very rare alliance. J Clin Pathol. 2010; 63:950–2.

14. Abdellatif AA, Waris S, Lakhani A, Kadikoy H, Haque W, Truong LD. True vasculitis in lupus nephritis. Clin Nephrol. 2010; 74:106–12.

15. Kadikoy H, Haque W, Ahmed S, Abdellatif A. Rituximab in the treatment of refractory lupus nephritis with vasculitis. Saudi J Kidney Dis Transpl. 2012; 23:334–7.