Article
Blood-borne IgA-plasma cells in granulomatosis with polyangiitis as a sign for involvement of the upper respiratory tract or gastrointestinal involvement?
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Authors
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Bimba Franziska Hoyer
- Charité - Universitätsmedizin Berlin, Medizinische Klinik mit Schwerpunkt Rheumatologie und klinische Immunologie, Deutsches Rheuma-Forschungszentrum (DRFZ), Berlin
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Anna Klemm
- Deutsches Rheumaforschungs-Zentrum Berlin, Berlin
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Adriano Taddeo
- Deutsches Rheuma-Forschungszentrum (DRFZ), Charité - Universitätsmedizin Berlin, Medizinische Klinik mit Schwerpunkt Rheumatologie und klinische Immunologie, Berlin
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Laleh Khodadadi
- Deutsches Rheuma-Forschungszentrum (DRFZ), Charité - Universitätsmedizin Berlin, Medizinische Klinik mit Schwerpunkt Rheumatologie und klinische Immunologie, Berlin
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Qingyu Cheng
- Deutsches Rheuma-Forschungszentrum (DRFZ), Charité - Universitätsmedizin Berlin, Medizinische Klinik mit Schwerpunkt Rheumatologie und klinische Immunologie, Berlin
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Andreas Radbruch
- Deutsches Rheuma-Forschungszentrum (DRFZ), Berlin
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Falk Hiepe
- Medizinische Klinik mit Schwerpunkt Rheumatologie und klinische Immunologie der Charité - Universitätsmedizin Berlin und Deutsches RheumaForschungszentrum Berlin - ein Institut der Leibniz-Gemeinschaft, Berlin
| Published: | September 12, 2014 |
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Outline
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Background: B cells seem to play a major role in granulomatosis with polyangiitis (GPA, formarly known as Wegener’s disease). This is reflected by the presence of autoantibodies directed against neutrophil granular encymes (ANCA) in a great majority of patients as well as in the success of B cell depleting therapies. Renal manifestations seem to be directly mediated by autoantibodies. Whether mucosal plasma cells play a role in involvement of the respiratory tract or the gastrointestinal tract is yet unknown. IgA-ANCA can be found in about 30% of patients (Kelley et al). For a better understanding of the possible role of B cells in GPA, we measured B and T cells in the peripheral blood of patients and found major changes correlating with disease activity (BVAS).
Methods: 25 patients with GPA (12 with active disease, 13 in remission) were analysed by flow cytometry and compared to 25 healthy donors and 10 with systemic sclerosis (SSc). Stainings for CD19, 20, 27, 3, 4, 8, 138, IgD, IgA and MHCII were performed and analysed by FlowJo. The study was approved by the ethics comitee of the Charité and all patients signed informed consent.
Results: Marked differences (p=0.0018) were found regarding the number and frequency of plasmablasts and – cells in patients with active disease (6.6 ± 5.0/µl) as compared to patients in remission (2.5 ± 1.6/µl) or healthy donors (2.3 ± 1.2/µl). IN patients with GPA a significant higher number of the plasma cells was IgA-producing cells as compared to healthy controls (p=0.0028).
The number of plasma cells as well as their frequency correlate with disease activity (r= 0.9135, p<0.0001). In patients with systemic sclerosis we found comparable changes.
Conclusion: The number of plasma cells in active GPA and SSc is increased which implies a role of plasma cell mediated mechanisms in the pathogenesis of GPA and maybe SSc. A high frequency of these plasma cells is IgA-producing which could play a role in involvement of the upper respiratory tract or gastrointestinal involvement. Further studies are needed including the analysis of biopsies to further understand their role.
