scholarly journals Effect of Treatment on Damage and Hospitalization in Elderly Patients with Microscopic Polyangiitis and Granulomatosis with Polyangiitis

2019 ◽  
Vol 47 (4) ◽  
pp. 580-588 ◽  
Author(s):  
Maria Weiner ◽  
Su Mein Goh ◽  
Aladdin J. Mohammad ◽  
Zdenka Hrušková ◽  
Anisha Tanna ◽  
...  
Keyword(s):  
Elderly Patients ◽  
Cause Of Death ◽  
Granulomatosis With Polyangiitis ◽  
Microscopic Polyangiitis ◽  
Immunosuppressive Treatment ◽  
Damage Index ◽  
Positive Association ◽  
Oral Prednisolone ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Organ Damage

Objective.Age is a risk factor for organ damage, adverse events, and mortality in microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA). However, the relationship between treatment and damage, hospitalizations, and causes of death in elderly patients is largely unknown.Methods.Consecutive patients from Sweden, the United Kingdom, and the Czech Republic diagnosed between 1997 and 2013 were included. Inclusion criteria were a diagnosis of MPA or GPA and age 75 years or more at diagnosis. Treatment with cyclophosphamide (CYC), rituximab (RTX), and corticosteroids the first 3 months was registered. Outcomes up to 2 years from diagnosis included Vasculitis Damage Index (VDI), hospitalization, and cause of death.Results.Treatment data were available for 167 of 202 patients. At 2 years, 4% had no items of damage. There was a positive association between VDI score at 2 years and Birmingham Vasculitis Activity Score at onset, and a negative association with treatment using CYC or RTX. Intravenous methylprednisolone dose was associated with treatment-related damage. During the first year, 69% of patients were readmitted to hospital. Myeloperoxidase–antineutrophil cytoplasmic antibody positivity and lower creatinine levels decreased the odds of readmission. The most common cause of death was infection, and this was associated with cumulative oral prednisolone dose.Conclusion.Immunosuppressive treatment with CYC or RTX in elderly patients with MPA and GPA was associated with development of less permanent organ damage and was not associated with hospitalization. However, higher doses of corticosteroids during the first 3 months was associated with treatment-related damage and fatal infections.

Incidence of Malignancy Prior to Antineutrophil Cytoplasmic Antibody–associated Vasculitis Compared to the General Population

2017 ◽  
Vol 44 (3) ◽  
pp. 314-318 ◽  
Author(s):  
Emma E. van Daalen ◽  
Chinar Rahmattulla ◽  
Ron Wolterbeek ◽  
Jan A. Bruijn ◽  
Ingeborg M. Bajema
Keyword(s):  
General Population ◽  
Causal Relationship ◽  
Granulomatosis With Polyangiitis ◽  
Microscopic Polyangiitis ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Malignancy Risk ◽  
Time Period ◽  
Study Results ◽  
Significant Difference ◽  
Overall Risk

Objective.Previous studies have reported an increased malignancy risk preceding antineutrophil cytoplasmic antibody–associated vasculitis (AAV), suggesting common pathogenic pathways in these 2 entities. However, the study results were conflicting and often limited to patients with granulomatosis with polyangiitis (GPA). Here, we study the malignancy risk prior to AAV diagnosis [either GPA or microscopic polyangiitis (MPA)] to elaborate on the putative association between malignancy and AAV.Methods.A total of 203 patients were selected for the current study. Malignancies prior to AAV diagnosis were identified using a nationwide pathology database, and their occurrence was verified by reviewing the medical files of 145 patients (71.4%). The malignancy incidence was compared to the general population by calculation of standardized incidence ratios (SIR), matching for sex, age, and time period. SIR were calculated for 2 intervals: < 2 years and ≥ 2 years prior to AAV diagnosis. Separate analyses were performed for GPA and MPA.Results.The overall risk for malignancy prior to AAV diagnosis was similar to that of the general population (SIR 0.96, 95% CI 0.55–1.57), as was true when risks were analyzed by malignancy type, including skin, bladder, kidney, lung, stomach, rectum, and uterus (SIR ranged from 1.64 to 4.14). We found no significant difference in malignancy risk between patients with GPA and MPA.Conclusion.Our findings do not support the hypothesis that preceding malignancies and AAV have a causal relationship or shared pathogenic pathways.

Antineutrophil Cytoplasmic Antibody-Associated Vasculitis: Toward an Individualized Approach

Nephron ◽  
2021 ◽  
pp. 1-17
Author(s):  
Javier Villacorta ◽  
Laura Martinez-Valenzuela ◽  
Irene Martin-Capon ◽  
Juliana Bordignon-Draibe
Keyword(s):  
Alternative Pathway ◽  
Treatment Options ◽  
Immunosuppressive Treatment ◽  
Standard Of Care ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Organ Damage ◽  
Cumulative Exposure ◽  
Clinical Approach ◽  
Clinical Syndromes ◽  
Individualized Approach

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), characterized by the presence of autoantibodies to neutrophil cytoplasmic antigens, proteinase 3 (PR3), and myeloperoxidase (MPO), typically involves small blood vessels of the respiratory tract and kidneys. It includes distinct clinical syndromes: microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA), and eosinophilic GPA. GPA is commonly associated with PR3-ANCA, while MPA is associated with MPO-ANCA. AAVs have a complex pathogenesis, influenced by genetics and environmental factors. There is evidence for a loss of tolerance to neutrophil proteins, which leads to ANCA-mediated neutrophil activation and injury, with effector T cells and activation of the alternative pathway of the complement also involved. Advances in immunosuppressive treatment have drastically reduced mortality of AAV in the past decades, opting for a more individualized approach. Careful assessment of ANCA specificity, disease activity, organ damage, and quality of life allows for a tailored immunosuppressive therapy. Contemporary AAV treatment is characterized by regimens that minimize the cumulative exposure to glucocorticoids and cyclophosphamide, and novel approaches including complement blockage and immunosuppressant combinations might be the standard of care in the future. In this review, we examine the pathogenesis, clinical approach, and evidence-based treatment options for the management of AAV patients.

scholarly journals Granulomatosis with polyangiitis in a patient with biopsy-proven IgG4-related pulmonary disease and coincident small cell lung cancer

2019 ◽  
Vol 12 (3) ◽  
pp. e226280 ◽  
Author(s):  
Khurram Abbass ◽  
Hollis Krug
Keyword(s):  
Lung Cancer ◽  
Small Cell Lung Cancer ◽  
Cell Lung Cancer ◽  
Granulomatosis With Polyangiitis ◽  
Immunosuppressive Treatment ◽  
Rapidly Progressive Glomerulonephritis ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Small Cell ◽  
Small Cell Lung ◽  
Igg4 Related Disease

Granulomatosis with polyangiitis (GPA) was diagnosed in a patient with a 16-month history of IgG4-related lung disease that spontaneously became asymptomatic. Cytoplasmic antineutrophil cytoplasmic antibody (ANCA) was positive at the time of diagnosis of IgG4-related disease (IgG4-RD), but there was no vasculitis or kidney disease. Sixteen months later he developed rapidly progressive glomerulonephritis that responded to cyclophosphamide treatment. While undergoing treatment for GPA, he was found to have a lung mass identified as small cell lung cancer. This mass was present at the time of the IgG4-RD diagnosis. GPA can be confused with IgG4-RD histologically and they rarely coexist. ANCA antibodies are primarily IgG4 subclass. IgG4-RD has been associated with cancer and may improve prognosis. We speculate that this patient may have had small cell lung cancer that incited an IgG4 predominant immune response with coexistent ANCA antibodies that eventually resulted in GPA. Immunosuppressive treatment of GPA likely accelerated the progression of the lung cancer.

Efficacy and Safety of Rituximab Treatment in Patients with Antineutrophil Cytoplasmic Antibody-associated Vasculitides: Results from a German Registry (GRAID)

2012 ◽  
Vol 39 (11) ◽  
pp. 2153-2156 ◽  
Author(s):  
PETRA ROLL ◽  
EVA OSTERMEIER ◽  
MARION HAUBITZ ◽  
SVJETLANA LOVRIC ◽  
LEONORE UNGER ◽  
...  
Keyword(s):  
Treatment Option ◽  
Clinical Efficacy ◽  
Granulomatosis With Polyangiitis ◽  
Microscopic Polyangiitis ◽  
Safety Data ◽  
Antineutrophil Cytoplasmic Antibody ◽  
National Registry ◽  
Efficacy And Safety ◽  
Anca Associated Vasculitis ◽  
German Registry

Objective.Rituximab (RTX) therapy is a treatment option in patients with refractory antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). We investigated the tolerability and clinical efficacy of RTX in a cohort of patients with refractory AAV.Methods.Clinical and safety data of patients with AAV treated with RTX were retrospectively assessed from the data of a German national registry.Results.In total, 58 patients were included in this analysis (50/58 with granulomatosis with polyangiitis; 8/58 with microscopic polyangiitis who received at least 1 cycle, 17 patients who received 2 cycles, and 3 patients who received 3 cycles of RTX). Response was classified as complete and partial in 22 (40%) and in 29 cases (52.7%), respectively. Four patients (7.3%) were classified as nonresponders.Conclusion.RTX was well tolerated with good clinical efficacy in patients with refractory AAV.

Spectrum and Prognosis of Antineutrophil Cytoplasmic Antibody–associated Vasculitis-related Bronchiectasis: Data from 61 Patients

2019 ◽  
Vol 47 (10) ◽  
pp. 1522-1531 ◽  
Author(s):  
Raphael Lhote ◽  
Marie Chilles ◽  
Matthieu Groh ◽  
Xavier Puéchal ◽  
Philippe Guilpain ◽  
...  
Keyword(s):  
Median Time ◽  
Granulomatosis With Polyangiitis ◽  
Microscopic Polyangiitis ◽  
Fold Increase ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Mononeuritis Multiplex ◽  
Biological Features ◽  
Anca Associated Vasculitis ◽  
Worse Prognosis

ObjectiveTo report on a large series of patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and bronchiectasis, with a specific focus on the timeline of occurrence of both features.MethodsRetrospective nationwide multicenter study of patients diagnosed with both AAV and bronchiectasis.ResultsSixty-one patients were included, among whom 27 (44.25%) had microscopic polyangiitis (MPA), 27 (44.25%) had granulomatosis with polyangiitis (GPA), and 7 (11.5%) had eosinophilic GPA. Thirty-nine (64%) had myeloperoxidase (MPO)-ANCA and 13 (21%) had proteinase 3–ANCA. The diagnosis of bronchiectasis either preceded (n = 25; median time between both diagnoses: 16 yrs, IQR 4–54 yrs), was concomitant to (n = 12), or followed (n = 24; median time between both diagnoses: 1, IQR 0–6 yrs) that of AAV. Patients in whom bronchiectasis precedes the onset of AAV (B-AAV group) have more frequent mononeuritis multiplex, MPA, MPO-ANCA, and a 5-fold increase of death. The occurrence of an AAV relapse tended to be protective against bronchiectasis worsening (HR 0.6, 95% CI 0.4–0.99, P = 0.049), while a diagnosis of bronchiectasis before AAV (HR 5.8, 95% CI 1.2–28.7, P = 0.03) or MPA (HR 18.1, 95% CI 2.2–146.3, P = 0.01) were associated with shorter survival during AAV follow-up.ConclusionThe association of bronchiectasis with AAV is likely not accidental and is mostly associated with MPO-ANCA. Patients in whom bronchiectasis precedes the onset of AAV tend to have distinct clinical and biological features and could carry a worse prognosis.

scholarly journals A case of ANCA associated vasculitis presented with mononeuritis multiplex

2019 ◽  
Vol 9 (3) ◽  
pp. 257-260
Author(s):  
Tanbin Rahman ◽  
Md Rashedul Islam ◽  
Mohammad Sakhawat Hossen Khan ◽  
Sharif Mohammad Ruhul Quddus ◽  
Dilruba Alam ◽  
...  
Keyword(s):  
Inflammatory Cell ◽  
Granulomatosis With Polyangiitis ◽  
Microscopic Polyangiitis ◽  
Systemic Vasculitis ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Mononeuritis Multiplex ◽  
Good Clinical Response ◽  
Standard Regimen ◽  
Anca Associated Vasculitis ◽  
Common Manifestation

Mononeuritis multiplex is a common manifestation of many illnesses which includes diabetes, leprosy, malignancy and certain types of systemic vasculitis. The antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of rare diseases which show typical characteristic inflammatory cell infiltration and blood vessel wall necrosis. AAV syndromes include granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilicgranulomatosis with polyangiitis (EGPA). Here we present a patient who presented with mononeuritis multiplex and had features ofEGPA. The patient was treated with standard regimen of steroids and pulsed cyclophosphamide and she achieved good clinical response. Birdem Med J 2019; 9(3): 257-260

Disease and treatment-related morbidity in young and elderly patients with granulomatosis with polyangiitis and microscopic polyangiitis

2020 ◽  
Vol 50 (6) ◽  
pp. 1441-1448 ◽  
Author(s):  
Alvise Berti ◽  
Mara Felicetti ◽  
Sara Monti ◽  
Augusta Ortolan ◽  
Roberto Padoan ◽  
...  
Keyword(s):  
Elderly Patients ◽  
Granulomatosis With Polyangiitis ◽  
Microscopic Polyangiitis

Vascular endothelial growth factor (VEGF)-A and VEGF-A165b are associated with time to remission of granulomatosis with polyangiitis in a nationwide Japanese prospective cohort study

2020 ◽  
pp. 000456322096837
Author(s):  
Ryosuke Kikuchi ◽  
Naotake Tsuboi ◽  
Ken-Ei Sada ◽  
Masahiro Nakatochi ◽  
Yuki Yokoe ◽  
...  
Keyword(s):  
Vascular Endothelial Growth Factor ◽  
Cohort Study ◽  
Growth Factor ◽  
Granulomatosis With Polyangiitis ◽  
Microscopic Polyangiitis ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Vascular Endothelial ◽  
Serum Samples ◽  
Endothelial Growth Factor

Background Effective prognostic markers are needed for antineutrophil cytoplasmic antibody-associated vasculitis (AAV). This study evaluated the clinical associations of serum vascular endothelial growth factor-A (sVEGF-A) and sVEGF-A165b (an antiangiogenic isoform of VEGF-A) concentrations with time to remission of AAV in a nationwide Japanese prospective follow-up cohort. Methods We collected samples from patients with AAV who were enrolled in the nationwide Japanese cohort study (RemIT-JAV-RPGN). We measured sVEGF-A and sVEGF-A165b concentrations using enzyme-linked immunosorbent assays in 57 serum samples collected 6 months before and after initiation of AAV treatment. Patients were classified based on AAV disease subtypes: microscopic polyangiitis, granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis (EGPA). Results Results revealed significant reductions in sVEGF-A and sVEGF-A165b concentrations in patients with microscopic polyangiitis and EGPA, respectively. However, despite the comparable concentrations of sVEGF-A and sVEGF-A165b during the 6 months of treatment in granulomatosis with polyangiitis patients, correlation analysis revealed that the differences in log2-transformed concentrations of sVEGF-A and sVEGF-A165b were inversely correlated with time to remission in granulomatosis with polyangiitis patients. Conclusion These results suggest that sVEGF-A and -A165b can serve as potential markers of time to remission in patients with granulomatosis with polyangiitis.

Rituximab Maintenance Therapy for Granulomatosis with Polyangiitis and Microscopic Polyangiitis

2011 ◽  
Vol 39 (1) ◽  
pp. 125-130 ◽  
Author(s):  
CLAIRE ROUBAUD-BAUDRON ◽  
CHRISTIAN PAGNOUX ◽  
NADINE MÉAUX-RUAULT ◽  
ANNE GRASLAND ◽  
ABDELKADER ZOULIM ◽  
...  
Keyword(s):  
Maintenance Therapy ◽  
Granulomatosis With Polyangiitis ◽  
Microscopic Polyangiitis ◽  
Controlled Trial ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Maintenance Infusion ◽  
Randomized Controlled ◽  
Rituximab Maintenance ◽  
Induction Regimen ◽  
Antibody Levels

Objective.To evaluate the efficacy compared to the relapse risk and tolerance of systematic rituximab (RTX) infusions as maintenance therapy for patients with granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA), who entered remission taking conventional immunosuppressants or RTX.Methods.A retrospective study of the main clinical characteristics, outcomes, and RTX tolerance of patients who had received ≥ 2 RTX maintenance infusions in our center, regardless of induction regimen, between 2003 and 2010.Results.We identified 28 patients [4 MPA and 24 GPA; median age 55.5 yrs (range 18–78); 17 (60%) males] who received a median of 4 (range 2–10) RTX maintenance infusions, with median followup of 38 months (range 21–97) since diagnosis or last flare. None experienced a RTX infusion-related adverse event; 15 patients (among the 21 with available data) had hypogammaglobulinemia (predominantly IgM) prior to their last RTX maintenance infusion; 3 had infectious events (1 cutaneous abscess, 1 otitis, 1 fatal H1N1 flu). Two patients suffered pulmonary relapses shortly before a planned RTX maintenance infusion (both had increased antineutrophil cytoplasmic antibody levels and 1 had CD19+ lymphocyte reconstitution).Conclusion.Rituximab maintenance therapy was well tolerated but did not completely prevent relapses and persistent “grumbling” disease. These preliminary results remain to be confirmed by a randomized controlled trial currently in progress.

Antineutrophil Cytoplasmic Antibody–Positive Vasculitis

Asthma ◽  
2014 ◽  
pp. 12-20
Author(s):  
Lanny J. Rosenwasser ◽  
Dennis K. Ledford
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Microscopic Polyangiitis ◽  
Upper Airway ◽  
Airway Disease ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Anca Associated Vasculitis ◽  
Granulomatous Polyangiitis ◽  
Strauss Syndrome ◽  
Eosinophilic Granulomatosis

The antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis syndromes— eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome or allergic angiitis with granulomatosis), granulomatous polyangiitis (Wegener’s granulomatosis), and microscopic polyangiitis—are serious comorbidities of asthma or upper respiratory disease, or the symptoms of the vasculitis may resemble asthma or associated upper airway disease. ANCA-associated vasculitis is potentially fatal but is responsive to a variety of treatments, if the vasculitis is recognized before serious organ dysfunction. The role of ANCA in these conditions has not been defined because eosinophilic granulomatosis with polyangiitis, granulomatous polyangiitis, or microscopic polyangiitis may occur without ANCA, and the titer of ANCA does not predict clinical course. Clinical suspicion and consideration of tissue biopsy are important for recognition before irreversible complications occur. Corticosteroid therapy for asthma or suspected asthma may modify the presentations of ANCA-positive vasculitis.

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