Predicting relapse in granulomatosis with polyangiitis: The role of biomarkers

2013 ◽  
Vol 42 (4) ◽  
pp. 716
Author(s):  
P. Hogan ◽  
R. O’connell ◽  
E. Browne ◽  
S. Scollard ◽  
E. Hackett ◽  
...  
Keyword(s):  
Granulomatosis With Polyangiitis

scholarly journals SAT0267 ROLE OF AGGRESSIVE IMMUNOSUPPRESSION ON SUBGLOTTIC STENOSIS IN GRANULOMATOSIS WITH POLYANGIITIS: RETROSPECTIVE ANALYSIS OF A MONOCENTRIC COHORT

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1076.1-1077
Author(s):  
L. Moroni ◽  
L. Giudice ◽  
G. A. Ramirez ◽  
S. Sartorelli ◽  
A. Cariddi ◽  
...  
Keyword(s):  
Medical Treatment ◽  
Clinical Characteristics ◽  
Granulomatosis With Polyangiitis ◽  
Balloon Catheter ◽  
Subglottic Stenosis ◽  
Damage Accrual ◽  
The Mean ◽  
The One

Background:Subglottic stenosis (SGS) is defined as airway narrowing below the vocal cords and is a common and potentially life-threatening manifestation of Granulomatosis with Polyangiitis (GPA), with an estimated prevalence of 16-23% (1). Balloon catheter dilation is effective in GPA-related SGS, but relapses are frequent. Little is known about the role of immunosuppression in this setting.Objectives:to analyse the clinical characteristics of a monocentric GPA cohort, describe phenotype differences among patients with and without SGS and investigate the role of surgical and medical treatments on relapse risk and general outcome.Methods:Biopsy-proven patients with SGS were identified by review of medical charts among a cohort of patients with GPA, classified according to the algorithm of the European Medicine Agency (2). The clinical characteristics of patients with SGS were retrospectively collected over a median follow-up time of 15.9 years and compared to those of patients without SGS.Results:Fourteen patients with SGS-GPA were identified, with a female to male ratio of 1:1 and a prevalence of 29.2% among the cohort. The mean ± SD age at GPA onset was 30.8 ± 14.4 years, with a mean time from GPA diagnosis to SGS onset of 4.7 ± 4.2 years. ANCA were positive in 78.6% (54.0% anti-PR3, 18.1% anti-MPO and 27.9% IFI only). The mean Birmingham Vasculitis Activity Score (BVAS) at onset was 10.0 ± 5.6. The main clinical manifestations associated with SGS were crusty rhinitis (100%), sinusitis (78%), pulmonary disease (72.7%), otitis/mastoiditis (50%), glomerulonephritis (42.9%), orbital pseudotumor (28.6%). Six patients (42.9%) received medical treatment only, other six (42.9%) had one to three balloon dilations and two (14.2%) underwent four or more procedures. Eight patients had no SGS relapse (maximum one dilation) and they all received immunosuppression with rituximab (RTX), cyclophosphamide (CYC) or azathioprine (AZA). All patients who received no immunosuppression, methotrexate (MTX) or mycophenolate (MMF) had at least one relapse. Patients treated with MTX or MMF had a mean relapse-free survival of 13.1 months, which was comparable to the one of patients not receiving medical treatment (40.2 months; p=NS) and shorter than the one of patients receiving CYC or RTX (153.2 months; p=0.032). CYC use also inversely correlated with the number of surgical procedures (r=-0.691, p=0.006). Compared to patients without SGS (31 consecutive patients with at least 4 years of follow-up), patients with SGS-GPA had an earlier disease onset (mean age 30.8 vs 50.4 years; p<0.001), but with lower BVAS (mean 10.0 vs 15.3; p=0.013) and showed a higher prevalence of crusty rhinitis (100% vs 67.7%; p=0.019). No difference was observed in damage accrual over time between the two groups.Conclusion:Subglottic stenosis is highly prevalent in patients with GPA and may define a milder disease subset occurring more frequently in younger patients. MTX and MMF might be insufficient to prevent SGS relapses requiring balloon dilation. Aggressive immunosuppression (CYC or RTX) might have a non-redundant role in this setting and reduce the risk of relapses.References:[1]Quinn KA, et al. Subglottic stenosis and endobronchial disease in granulomatosis with polyangiitis. Rheumatology 2019; 58 (12), 2203-2211.[2]Watts R, et al. Development and validation of a consensus methodology for the classification of the ANCA associated vasculitides and polyarteritis nodosa for epidemiological studies. Ann Rheum Dis 2007; 66: 222-7.Disclosure of Interests:Luca Moroni: None declared, Laura Giudice: None declared, Giuseppe Alvise Ramirez: None declared, Silvia Sartorelli: None declared, adriana cariddi: None declared, Angelo Carretta: None declared, Enrica Bozzolo: None declared, Lorenzo Dagna Grant/research support from: The Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR) received unresctricted research/educational grants from Abbvie, Bristol-Myers Squibb, Celgene, Janssen, Merk Sharp & Dohme, Mundipharma Pharmaceuticals, Novartis, Pfizer, Roche, Sanofi-Genzyme, and SOBI., Consultant of: Prof Lorenzo Dagna received consultation honoraria from Abbvie, Amgen, Biogen, Bristol-Myers Squibb, Celltrion, Novartis, Pfizer, Roche, Sanofi-Genzyme, and SOBI.

P0280ENVIRONMENTAL AND OCCUPATIONAL EXPOSURES IN EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS. A CASE-CONTROL STUDY

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Federica Maritati ◽  
Francesco Peyronel ◽  
Paride Fenaroli ◽  
Giuseppe Daniele Benigno ◽  
Alessandra Palmisano ◽  
...  
Keyword(s):  
Risk Factors ◽  
Organic Solvents ◽  
Granulomatosis With Polyangiitis ◽  
Geographical Origin ◽  
Case Control ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Occupational Risk Factors ◽  
Chemical Agents ◽  
Eosinophilic Granulomatosis

Abstract Background and Aims Eosinophilic granulomatosis with polyangiitis (EGPA, formerly Churg-Strauss Syndrome) is a rare systemic disease, included in the group of antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV), characterized by adult-onset asthma, blood and tissue eosinophilia with organ involvement, and small-vessel vasculitis. The pathogenesis of the disease is poorly understood, but it is likely that immunogenetic factors play a crucial role in its development. However, it has been suspected that the disease may be triggered by exogenous factors including environmental agents, infections, vaccinations, and drugs. Data about the association between individual, environmental and occupational risk factors and the development of EGPA are scarce. In this study we aimed to investigate the role of occupational agents (such as silica, farming, organic solvents and chemical agents) as well as individual agents (such as smoking habits) and their interactions, as potential risk factors for EGPA. Method The study has a case-control design. We enrolled 111 patients with EGPA and 333 healthy controls, matched for age, sex and geographical origin. Occupational history was obtained using a structured questionnaire administered by blinded specialists in occupational medicine. The exposures to non-occupational risk factors potentially predisposing to EGPA were assessed through the interview and through the examination of medical records. All exposures were considered until the time of EGPA diagnosis. Results At a multivariate logistic regression analysis, EGPA was associated with silica exposure (OR 2.26 [95% CI 1.10-4.62], p=0.026), farming (OR 2.10 [95% CI 1.19-3.73], p=0.011) and with the exposure to organic solvents (OR 2.20 [95% CI 1.14-4.2], p=0.018). There was a positive relationship between the duration of exposure to silica, chemical agents and the risk of developing EGPA (Figure 1). A multiplicative effect on risk was found for the co-exposure of silica and farming (OR 7.49 [95% CI 2.77-20.25], p&lt;0.0001) (Figure 2). The exposure to tobacco smoke appeared protective against EGPA (OR 0.49 [95% CI 0.29-0.70], p&lt;0.0001). Conclusion This is, to our knowledge, the first study investigating the role of environmental, occupational and individual risk factors in the development of EGPA, through the comparison of a group of EGPA patients with a group of healthy controls, matched for age, gender and geographical origin. Our results demonstrate that occupational factors may significantly contribute to the development of the disease. In particular, we found a significative association between exposure to silica, farming, organic solvents and EGPA. In addition, our findings reveal that tobacco smoking has a protective role against the disease. Further studies are necessary to investigate the biological effects of such risk factors on disease pathogenesis.

scholarly journals Current therapy of granulomatosis with polyangiitis and microscopic polyangiitis: the role of rituximab

2014 ◽  
Vol 28 (1) ◽  
pp. 17-27 ◽  
Author(s):  
Duvuru Geetha ◽  
Cees Kallenberg ◽  
John H. Stone ◽  
Alan D. Salama ◽  
Gerald B. Appel ◽  
...  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Microscopic Polyangiitis ◽  
Current Therapy

The role of intratracheal dilation – Injection technique in treatment of granulomatosis with polyangiitis (GPA) patients with subglottic stenosis

2016 ◽  
Author(s):  
Justyna Fijolek ◽  
Elzbieta Wiatr ◽  
Dariusz Gawryluk ◽  
Magdalena Maria Martusewicz-Boros ◽  
Tadeusz Maria Orlowski ◽  
...  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Subglottic Stenosis ◽  
Injection Technique

scholarly journals Subglottic Stenosis in Granulomatosis With Polyangiitis: The Role of Laryngotracheal Resection

2018 ◽  
Vol 105 (1) ◽  
pp. 249-253 ◽  
Author(s):  
Christina L. Costantino ◽  
John L. Niles ◽  
Cameron D. Wright ◽  
Douglas J. Mathisen ◽  
Ashok Muniappan
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Subglottic Stenosis ◽  
Laryngotracheal Resection

scholarly journals ANCA-Associated Vasculitides and Hematologic Malignancies: Lessons from the Past and Future Perspectives

2019 ◽  
Vol 2019 ◽  
pp. 1-9 ◽  
Author(s):  
Marco Folci ◽  
Giacomo Ramponi ◽  
Dana Shiffer ◽  
Aurora Zumbo ◽  
Michele Agosti ◽  
...  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Hematologic Malignancy ◽  
Hematologic Malignancies ◽  
Future Perspectives ◽  
The Past ◽  
Anca Associated Vasculitis ◽  
Uncommon Presentation ◽  
Immunotherapy Target ◽  
And Function

The purpose of this paper is to collect and summarize all evidences relating to an association between ANCA-associated vasculitides (AAVs) and hematologic malignancies, in the form of either a paraneoplastic vasculitis or leukemias and lymphomas developing on a preexisting vasculitis. Additionally, the role of cyclophosphamide in vasculitis treatment has been assessed and compared to rituximab. Paraneoplastic AAV seems to be an uncommon presentation of hemopathies. Hematologic malignancy risk in AAV is more likely to be increased by cyclophosphamide, although not yet definitely proven. Furthermore, the pathogenesis of ANCA-associated vasculitis has been reviewed with particular emphasis on the role of proteinase 3 (PR3) in fuelling granulomatosis with polyangiitis (GPA) inflammation. PR3 is a bactericidal protein expressed by neutrophilic granules and on their plasma membrane. Derangements in its expression and function have been linked to leukemias and GPA alike. PR3-derived PR1 peptide is being studied as an immunotherapy target in leukemia and multiple myeloma. This study is aimed at bringing together various evidences from the field of immunological and hematological research, at exposing contradictions, and at revealing novel insights on the association between ANCA-associated vasculitis and hematologic malignancies.

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