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Single-cell Analysis of Immunophenotype and Cytokine Production in Peripheral Whole Blood via Mass Cytometry
Published on: June 26, 2018
[Clinical characteristics and treatment outcomes of microscopic polyangiitis combined with autoimmune hemolytic
Dan Liu1, Ru Li2, Jia-yu Liu3
1Department of Rheumatology and Immunology,Peking University People's Hospital, Beijing 100044, China; Department of Rheumatology and Immunology, Fifth Hospital of Xi'an, Xi'an 710082, China.
Objective:
To investigate the clinical characteristics, laboratory features, outcome and prognosis of microscopic polyangiitis (MPA) patients with autoimmune hemolytic anemia (AIHA).
Methods:
In this study, 63 cases diagnosed as MPA from October 2006 to November 2013 in Peking University People's Hospital were reviewed. The clinical characteristics, outcome and prognosis of MPA patients with AIHA were retrospectively analyzed.
Results:
There were 12.7% (8/63) MPA patients with AIHA. The patients with AIHA had higher prevalence of fever, fatigue, hematuresis, albuminuria and new-onset hypertension, compared with non-AIHA group(87.5% vs.29.1%;100% vs. 49.1%; 100% vs. 60%; 75% vs. 20%, all P<0.05). In patients with AIHA, there were significantly lower levels of red blood cell(RBC), hemoglobin(Hb), alanine albumin(Alb) and complement C3[(2.3±0.5)×10(12)/L vs.(3.0±0.7)×10(12)/L;(66.2±13.1) g/L vs.(90.0±20.3) g/L; (26.1±4.4) g/L vs. (33.5±6.4) g/L; (0.7±0.2) g/L vs.(0.9±0.3) g/L,all P<0.05]. However, the levels of erythrocyte sedimentation rate (ESR), IgG and Birmingham Vasculitis Activity Score (BVAS) were higher, compared with those in the patients without AIHA [(102.1±25.7) mm/1 h vs.(76.5±31.1) mm/1 h; (20.9±6.1) g/L vs. (14.5±6.0) g/L; ( 23.7±5.7) vs.(17.3±4.1), all P<0.05]. The study suggested that there were more severe multi-system damage in the patients with AIHA than in those without AIHA. In the MPA patients with AIHA, six cases received treatment of methylprednisolone combined with immunosuppressant, and two received treatment of methylprednisolone only. Among the eight cases, three were recovered from anemia and four improved. One died of severe lung infection. After 4 years follow-up,in the seven MPA patients with AIHA, three received remission, two died,and two suffered from anemia and abnormal renal function.
Conclusion:
MPA with AIHA is more complicated by multi-system damage and not rare in clinical settings. Glucocorticoid combined with immunosuppressant is beneficial to induce remission for MPA patients with AIHA.
Insights
Microscopic polyangiitis (MPA) with autoimmune hemolytic anemia (AIHA) presents with more severe multi-system damage. Combination therapy with glucocorticoids and immunosuppressants shows benefit in treating MPA patients with AIHA.
Area of Science:
- Rheumatology
- Hematology
- Internal Medicine
Context:
- Microscopic polyangiitis (MPA) is a systemic vasculitis.
- Autoimmune hemolytic anemia (AIHA) is a condition where the immune system attacks red blood cells.
- The co-occurrence of MPA and AIHA presents unique clinical challenges.
Purpose:
- To investigate the clinical characteristics, laboratory features, outcomes, and prognosis of patients with microscopic polyangiitis (MPA) and autoimmune hemolytic anemia (AIHA).
Summary:
- This retrospective study analyzed 63 MPA patients, identifying 12.7% (8/63) with concurrent AIHA.
- MPA patients with AIHA exhibited a higher prevalence of fever, fatigue, hematuria, albuminuria, and new-onset hypertension, alongside lower RBC, hemoglobin, albumin, and C3 levels.
- These patients also showed higher ESR, IgG, and BVAS scores, indicating more severe multi-system damage compared to non-AIHA MPA patients.
Impact:
- MPA with AIHA is a complex condition associated with significant multi-system involvement.
- Treatment with methylprednisolone combined with immunosuppressants demonstrated positive outcomes in inducing remission for anemia and improving overall condition.
- The findings underscore the importance of recognizing and managing AIHA in MPA patients for improved prognosis.
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