Lupus nephritis associated with positive MPO-ANCA in a patient with underlying autoimmune hemolytic anemia

Abstract

A 19-year-old female was admitted with general malaise and systemic edema. She had been diagnosed as having autoimmune hemolytic anemia (AIHA) eight years earlier and was successfully managed with oral prednisolone. During the current admission, she was diagnosed as having systemic lupus erythematosus (SLE) based on the presence of renal involvement, hematological abnormalities, and antinuclear and anti-double-stranded DNA antibodies, along with a recurrence of AIHA; her serology revealed a high myeloperoxydase-antineutrophil cytoplasmic antibody (MPO-ANCA) titer. She was treated with prednisolone (50 mg day(-1)), but her renal function started to deteriorate. She responded to treatment with hemodialysis, plasmapheresis, and methylprednisolone pulse therapy; her MPO-ANCA titer and renal function improved. Treatment with intravenous cyclophosphamide gradually suppressed her AIHA and SLE activity. A renal biopsy revealed a diffuse proliferative lupus nephritis (class IV-G (A)) with necrotizing crescentic glomerulonephritis that was presumed to be associated with MPO-ANCA. The association of MPO-ANCA with SLE in this refractory case is discussed.

AP-VAS 2012 case report: a case of lupus nephritis with predominant synchronous cellular crescent formation and myeloperoxidase-antineutrophil cytoplasmic antibody seropositivity

“…Several cases considered to have ANCA-associated GN concurrent with LN have been reported [2][3][4][5][6][7][8][9][10]. Recently, Yu et al [4] reported that approximately 20 % of 152 patients who were classified as LN IV-G had crescentic GN and that approximately 30 % of cases with crescentic LN were seropositive for ANCA in a retrospective study of 327 patients with LN.…”

“…So far, very few reports have referred to the synchronicity of crescents in patients with ANCA-associated GN overlapping with LN. Of the cases reported to date with detailed pathologic findings, most had only cellular [3,5,7,10] or fibrocellular [6] crescents, although one case with different stages of crescents has been reported [9]. The existence of a link between ANCA seropositivity and SLE in the development of GN has been discussed [2][3][4].…”

“…In the treatment of AAV [15], immunosuppressive therapy with IVCY and corticosteroids is mostly used during induction therapy for this overlapping disease. However, their effects and the adverse events (e.g., infection) varied between cases, partly reflecting the comorbidities in each patient [2][3][4][5][6][7][8][9][10]. Although our patient required intermittent hemodialysis soon after admission, because of her very active renal histologic status and her severe and acute clinical course, we decided to use immunosuppressive therapies (i.e., MP and IVCY followed by oral corticosteroids).…”

“…Yu et al [4] reported that, in patients with LN IV-G treated with immunosuppressive therapy, the complete remission rate and renal outcome were worse in patients with crescents than in those without crescents. Alternatives to conventional immunosuppressive therapy such as intravenous Ig [6], calcineurin inhibitors [7,9], and plasma exchange [10] have been reported to achieve partial or complete remission. To achieve remission while minimizing the risk of immunosuppression-related adverse events, further accumulation of clinical evidence documenting the effects of different treatment strategies against this condition is necessary.…”

“…Recently, however, several cases with an overlap of both diseases have been reported [2][3][4][5][6][7][8][9][10]. Their pathologic findings differ from those of typical LN, showing disproportionate necrotizing and crescent formations compared with the degree of subendothelial immune complex deposits and endocapillary proliferation.…”