Case Report

A Case of Systemic Lupus Erythematosus/Antineutrophil Cytoplasmic Antibody-Associated Vasculitis Overlap Syndrome with Dissociated Pathological and Immunological Findings

Table 1

Serological and pathological features of the 21 reported cases described above (case 4 of [2] was excluded because no renal biopsy was reported).

AgeSexHematuriaProteinuria (g/day)Serum creatinine (mg/dL)ANAAnti-dsDNA (UL/mL)Low C3/C4ANCA specificityRenal biopsyPositive immunenoreactanatsDiagnosisTreatment

Case 1 of [1]22FemaleYes1.52.7Positive (1:1286)NegativeNot determinedMPO-ANCAIgG 3+ IgM 2+ IgA+ C3 3+ C1q 2+LN IV-S (A/C)/ANCA GNIntavenous CY, PSL, MMF
Case 2 of [1]37FemaleYes5.910Positive (1:80)PositiveNot determinedMPO-ANCAIgG 3+ C3 3+ C1q 1+LN V/ANCA GNIntravenousCY.PSL, azathioprine
Case 3 of [1]62FemaleYes24.7Positive (1:848)PositiveNot determinedNegativeIgG+ IgM+ C3 2+LN III (A/C)/ANCA GNIntravenousCY, PSL
Case 4 of [1]80FemaleYes44.5Positive (1:2560)NegativeNot determinedNegativeIgG+ IgM+LN II/ANCA GNHydralazine, oral CY, PSL, PLX
Case 5 of [1]19MaleYes49.6Positive (1:160)PositiveNot determinedNegativeIgG 3+ IgA+ C3 2+ C1q 2+LN IV G (A)/ANCA vasculitisM-PRED, intavenous CY, oral CY, PSL
Case 6 of [1]50FemaleYes3.24.4Positive (1:160)NegativeNot determinedMPO-ANCAIgG3+ IgA+ C3 2+ C1q 2+LN III (A)/ANCA GNM-PRED, intravenous CY, PSL
Case 7 of [1]55MaleYes321Positive (1:160)PositiveNot determinedNegativeIgG 2+ C3 2+LN V /ANCA GNPSL,intravenous CY
Case 8 of [1]37FemaleYes6.41.1Positive (1:40)NegativeNot determinedMPO-ANCAIgG 3+ C3 2+LN V /ANCA GNIntravenousCY, PSL
Case 9 of [1]44FemaleYes1.78.8Positive (1:320)PositiveNot determinedNegativeIgG 2+ IgM 1+ IgA 1+ C3 2+LN III (A/C)/ANCA GNIntravenousCY, PSL,MMF, HC
Case 10 of [1]78FemaleYes34.5Positive (1:640)NegativeNot determinedMPO-ANCA,PR-3 ANCAIgG 2+ C3 2+LN III(A/C)/ANCA GNHydralazine, M-PRED, OSL, oral CY
[4]40FemaleNot determinedNot determined8.7Positive (1:640)NegativeNot determinedMPO-ANCANot determinedPauci-immune GNPSL, rituximab, HC, CY
[5]48FemaleYes0.6Not determinedPositive (1:40)NegativeNo/noPR3-ANCAIgG+ C3+LN VPSL, intravenous CY
Case 1 of [3]23FemaleYes0.81.02Positive (1:800)23No/yesNegativeC3+Pauci-immune GNNot determined
Case 2 of [3]43FemaleYes3.27.11Positive (1:1280)NegativeNo/noMPO-ANCAIgG+ IgM+ C3+Pauci-immune GNNot determined
Case 3 of [3]57FemaleYes3.65.63Positive (1:1280)NegativeNo/noMPO-ANCAPauci-immunePauci-immune GNNot determined
Case 4 of [3]29FemaleYes0.31.01Positive (1:640)20No/noMPO-ANCAIgG 3+ IgM 2+ C1q 2+LN II-GNot determined
Case 5 of [3]41FemaleYes41.53Positive (1:2560)50Yes/yesMPO-ANCAIgG 2+ IgA 2+ C3 3+ C1q 2+LN IV-GNot determined
Case 6 of [3]53FemaleYes61.62Positive (1:600)NegativeYes/yesMPO-ANCANegativePauci-immune GNNot determined
Case 7 of [3]27FemaleYes0.83.93Positive (1:200)22No/yesMPO-ANCAIgG+ C3+Pauci-immune GNNot determined
Case 8 of [3]74FemaleYes13.31Positive (1:5240)50No/noMPO-ANCAIgG 2+ C3 2+LN IV-GNot determined
Case 1 of [4]74FemaleYes0.64.1Positive (1:5120)PositiveNo/yesMPO-ANCAIgG+ IgM+ C3+Not determinedNot determined
Case 2 of [4]35FemaleYes>63.4Positive (1:620)PositiveNo/noMPO-ANCAIgG+Not determinedPSL, CY
Case 3 of [4]21FemaleYes2.53.8Positive (1:2560)PositiveNo/noMPO-ANCAIgG+ IgM+ C3+ C1q+Not determinedPSL, oral CY, intravenous CY

ANA: antinuclear antibody, ANCA: antineutrophil cytoplasmic antibodies, LN: lupus nephritis, GN: glomerulonephritis, CY: cyclophosphamide, MMF: mycophenolate mofetil, PSL: prednisolone, M-PRED: pulse methylprednisolone, PLX: plasmapheresis, and HC: hydroxychloroquine.