ANCA-associated vasculitis with renal involvement: an outcome analysis

Weidner, Sven; Geuss, Steffen; Hafezi‐Rachti, Syrus; Wonka, Andrea; Rupprecht, Harald

doi:10.1093/ndt/gfh161

Cited by 141 publications

(125 citation statements)

References 15 publications

(15 reference statements)

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“…Our results extend the observations from the Dutch cohort. Our data are also in agreement with published results from other studies, showing that the renal function at initial presentation is the best predictor for renal function at follow-up (27)(28)(29). However, in contrast to the Dutch study, no significant correlation with the relapse risk was found in our cohort.…”

Section: Discussionsupporting

confidence: 92%

Membrane Proteinase 3 Expression in Patients with Wegener’s Granulomatosis and in Human Hematopoietic Stem Cell–Derived Neutrophils

Schreiber

Otto

et al. 2005

Journal of the American Society of Nephrology

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A large membrane proteinase 3 (mPR3)-positive neutrophil subset (mPR3 high ) is a risk for Wegener's granulomatosis (WG).The relationship between mPR3 expression and clinical manifestations was investigated in 81 WG patients and mPR3 expression was studied in CD34 ؉ stem cell-derived human neutrophils. The mPR3 high neutrophil percentage correlated with renal function, anemia, and albumin at the time of presentation. The mPR3 high neutrophil percentage and renal failure severity correlated directly after 5 yr. For elucidating mechanisms that govern mPR3 expression, studies were conducted to determine whether the genetic information that governs mPR3 expression resides within the neutrophils, even without stimuli possibly related to disease. CD34 ؉ hematopoietic stem cells were differentiated to neutrophils, and their mPR3 expression was determined. A two-step amplification/differentiation protocol was used to differentiate human CD34 ؉ hematopoietic stem cells into neutrophils with G-CSF. The cells progressively expressed the neutrophil surface markers CD66b, CD35, and CD11b. The ferricytochrome C assay demonstrated a strong respiratory burst at day 14 in response to PMA but none at day 0. Intracellular PR3 was detectable from day 4 by Western blotting. An increasing percentage of a mPR3-positive neutrophil subset became detectable by flow cytometry, whereas a second subset remained negative, consistent with a bimodal expression. Finally, human PR3-anti-neutrophil cytoplasmic autoantibodies induced a stronger respiratory burst, compared with human control IgG in stem cell-derived neutrophils. Taken together, these studies underscore the clinical importance of the WG mPR3 phenotype. The surface mPR3 on resting cells is probably genetically determined rather than being dictated by external factors.

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Section: Discussionsupporting

confidence: 92%

Membrane Proteinase 3 Expression in Patients with Wegener’s Granulomatosis and in Human Hematopoietic Stem Cell–Derived Neutrophils

Schreiber

Otto

et al. 2005

Journal of the American Society of Nephrology

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“…Infectious complications threaten patients receiving immunosuppressive therapy in AAV (1,2). In this retrospective study, 53% of patients experienced at least one event and 28% had an episode requiring hospitalization.…”

Section: Discussionmentioning

confidence: 81%

“…Newer therapies, such as rituximab, precisely target this cell (36). However, clinicians should specifically monitor this variable, and the presence of severe lymphopenia must alert the clinicians to its risk and entail appropriate actions: (1) to taper corticosteroids as proposed by current guidelines because this can easily be forgotten (37), (2) to measure CD4 lymphocyte cell count in order to establish the risk of opportunistic infections and start prophylaxis accordingly (35), (3) to reevaluate other risk factors of infection and reinforce preventive measures (hand washing, avoidance of viral contact), and (4) to suspect infections early and institute prompt treatment. Prevention of infectious complications should always be weighed against the need to control disease activity.…”

Section: Discussionmentioning

confidence: 99%

“…Infectious complications are major concerns during treatment of ANCA-associated vasculitis (AAV) and are a leading cause of death (1,2). Risk factors predictive of infections have been identified and include leukopenia, advancing age, deteriorating renal function, the intensity of cyclophosphamide, and corticosteroid therapy (3)(4)(5)(6)(7)(8)(9)(10).…”

Section: Introductionmentioning

confidence: 99%

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Lymphopenia and Treatment-Related Infectious Complications in ANCA-Associated Vasculitis

Goupil

Brachemi

Nadeau‐Fredette

et al. 2013

Clinical Journal of the American Society of Nephrology

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SummaryBackground and objectives ANCA-associated vasculitis (AAV) is treated with potent immunosuppressive regimens. This study sought to determine risk factors associated with infections during first-intention therapy.Design, setting, participants, & measurements This retrospective study involved two separate cohorts of consecutive cases of AAV seen from 2004 to 2011 at two university hospitals. The following were assessed: vasculitis severity; therapy; and periods with no, moderate (lymphocyte count, 0.3-1.03 10 9 /L), or severe (lymphocyte count # 0.3310 9 /L) lymphopenia and neutropenia (neutrophil count # 1.5310 9 /L).Results One hundred patients had a mean age of 57615 years and a Birmingham vasculitis activity score of 7.763.6. Therapy consisted of pulse methylprednisolone (59%), cyclophosphamide (85%), methotrexate (6%), and plasmapheresis (25%) in addition to oral corticosteroids. During follow-up, 53% of patients experienced infection and 28% were hospitalized for infection (severe infection). Only 18% experienced neutropenia, but 72% and 36% presented moderate and severe lymphopenia for a total duration of ,0.1%, 73%, and 8% of the treatment follow-up, respectively. Lower initial estimated GFR, longer duration of corticosteroid use, and presence of lymphopenia were risk factors of infections. The rate was 2.23 events/person-year in the presence of severe lymphopenia compared with 0.41 and 0.19 during periods with moderate or no lymphopenia (P,0.001). Similarly, the rate of severe infections was 1.00 event/person-year with severe lymphopenia and 0.08 and 0.10 with moderate and no lymphopenia (P,0.001). This association remained independent of other risk factors.Conclusions Lymphopenia is frequent during the treatment of AAV, and its severity is associated with the risk of infectious complications.

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“…The patients without thromboembolism were not evaluated for coagulation defects. Mean Ϯ SD platelet counts were 315 Ϯ 168 ϫ 10 3 /mm 3 . None of the patients had nephrotic range proteinuria.…”

Section: Resultsmentioning

confidence: 99%

Thromboembolic events as a complication of antineutrophil cytoplasmic antibody–associated vasculitis

Weidner¹,

Hafezi‐Rachti²,

Rupprecht³

2006

Arthritis & Rheumatism

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ANCA-associated vasculitis with renal involvement: an outcome analysis

Abstract: Our data underscore the pathogenetic potential of ANCA by demonstrating a more aggressive disease state and a poorer outcome in patients with PR3-ANCA.

Cited by 141 publications

References 15 publications

Membrane Proteinase 3 Expression in Patients with Wegener’s Granulomatosis and in Human Hematopoietic Stem Cell–Derived Neutrophils

Membrane Proteinase 3 Expression in Patients with Wegener’s Granulomatosis and in Human Hematopoietic Stem Cell–Derived Neutrophils

Lymphopenia and Treatment-Related Infectious Complications in ANCA-Associated Vasculitis

Thromboembolic events as a complication of antineutrophil cytoplasmic antibody–associated vasculitis

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