Session Title: Vasculitis I
Session Type: Abstract Submissions (ACR)
Background/Purpose: Autoimmune hemolytic anemia (AIHA) and Microscopic polyangiitis (MPA) are both rare autoimmune conditions. AIHA is caused by autoantibody-induced hemolysis (the premature destruction of circulating red blood cells) while MPA is characterized by necrotizing glomerulonephritis and pulmonary capillaritis. Both diseases are mediated by activation of lymphocytes and complement. Here we describe the clinical and laboratory characteristics of MPA patients combined with AIHA, investigate their treatment and prognosis, so as to help to improve the prognosis of such patients.
Methods: MPA was diagnosed in 51 patients referred to Department of Rheumatology, People’s Hospital, Peking University. All patients of MPA were divided into 2 groups, non-AIHA group (n=35), and MPA combined with AIHA group (n=16). Clinical and laboratory data were retrospectively analyzed. Disease activity was evaluated by Birmingham Vacuities Activity Score-version 3 (BVAS [V3]). Therapeutic response and prognosis were systemically reviewed during a two-year follow-up period.
Results: Sixteen of the 51 patients were combined with AIHA(31.4%) and 13 of them manifested as the first symptome. Incidence of fever, hypertension, and severe lung injury (i.e. interstitial lung disease, pneumorrhagia) of MPA combined with AIHA group were higher than that in non-AIAH group, fever (81.3% vs. 15%, X2=7.50, P<0.01), hypertension (50% vs. 17.1%, X2=5.95, P<0.01) and severe lung injury (100% vs. 68.9%, X2=4.69, P<0.01), respectively. Compared with non-AIHA group, MPA combined with AIHA group had significantly lower levels of RBC (2.3 ± 0.3×109/L vs. 3.0 ± 0.7 × 109/L), Hb (72.8 ± 15.3 vs. 102.3 ± 20.7g/L), and C3 (0.6 ± 0.2 g/L vs. 1 ± 0.2g/L), while higher level of ESR (103.4 ± 27.9mm/H vs. 76.5 ± 31.1mm/H), IgG (18.5 ± 6.1mg/ml vs. 13.9 ± 6.0mg/ml) and BVAS [V3] (22.3 ± 2.7 vs. 18.3 ± 5.1). In MPA combined with AIHA group, Methylprednisolone of 500mg/day and 40mg/day were used in nine and seven cases, respectively, in which 13 cases had combined with cyclophosphamide, and 1 case combined with mycophenolate mofetil. After 3 months’ treatment, Hb level of MPA combined with AIHA group significantly increased (from 72.8 ± 15.3 to 100.1 ± 16.9g/L) while BAVS (V3) has remarkably decreased (from 22.3 ± 2.7 to 3.6 ± 3.2). Five of 16 cases of MPA-AIHA patients died of severe anemia, renal failure and pulmonary fungal infection, another five were rehospitalized for anemia or renal dysfunction; the remaining six underwent gradual improvements of disease.
Conclusion: AIHA can be the first manifestation of MPA. MPA-AIHA patients with hypocomplementemia and multiple organ damage had high mortality. Start treatment with sufficient glucocorticoid combined with immunosuppressant is beneficial to induce remission.
Q. P. Chen,
H. H. Yao,
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ACR Meeting Abstracts - https://acrabstracts.org/abstract/clinical-research-of-microscopic-polyangiitis-combined-with-autoimmune-hemolytic-anemia/