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Laren Tan - One of the best experts on this subject based on the ideXlab platform.
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A rare case report of Polyangiitis overlap syndrome: Granulomatosis with Polyangiitis and eosinophilic Granulomatosis with Polyangiitis.
BMC pulmonary medicine, 2018Co-Authors: Michele V. Quan, Stephen K. Frankel, Mehrnaz Maleki-fischbach, Laren TanAbstract:Granulomatosis with Polyangiitis (GPA) is a systemic ANCA-associated vasculitis characterized by necrotizing granulomatous inflammation and a predilection for the upper and lower respiratory tract. Eosinophilic Granulomatosis with Polyangiitis (EGPA) is also a systemic ANCA-associated vasculitis, but EGPA is characterized by eosinophilic as well as granulomatous inflammation and is more commonly associated with asthma and eosinophilia. Polyangiitis overlap syndrome is defined as systemic vasculitis that does not fit precisely into a single category of classical vasculitis classification and/or overlaps with more than one category. Several Polyangiitis overlap syndromes have been identified, however, there are very few case reports of an overlap syndrome involving both GPA and EGPA in the medical literature. We conducted a PUBMED literature review using key words ‘Granulomatosis with Polyangiitis,’ ‘Wegener’s,’ ‘GPA,’ ‘eosinophilic Granulomatosis with Polyangiitis,’ ‘Churg-Strauss,’ ‘EGPA,’ ‘overlap syndrome,’ ‘Wegener’s with eosinophilia,’ and ‘GPA with eosinophilia’ in English only journals from 1986 to 2017. Relevant case reports and review articles of overlap syndromes of GPA and EGPA were identified. We aim to report a unique case of GPA and EGPA overlap syndrome and review the cases that have been previously described. Between 1986 and 2017, we identified 15 cases that represent an overlap syndrome with compelling features of both GPA and EGPA. Patients ranged in age between 21 and 78. Of those whose gender was identified, 80 % of the patients were female. All cases described involved the lungs, 60 % reported sinus involvement, and more than 50 % displayed renal involvement. An overwhelming majority of patients were positive for c-ANCA and demonstrated eosinophilia (peripheral blood or tissue eosinophilia). A preponderance of the cases described were treated with systemic corticosteroids combined with an immunosuppressive/cytotoxic agents. To our knowledge, there have been very few cases reported of an overlap syndrome of GPA and EGPA. Identification of patients with a Polyangiitis overlap syndrome of GPA and EGPA is imperative as prognosis, longitudinal management and treatment modalities may differ between these entities.
Loïc Guillevin - One of the best experts on this subject based on the ideXlab platform.
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Treatment of Eosinophilic Granulomatosis with Polyangiitis: A Review
Drugs, 2018Co-Authors: Loïc Raffray, Loïc GuillevinAbstract:Eosinophilic Granulomatosis with Polyangiitis (formerly Churg–Strauss syndrome) is a rare type of anti-neutrophil cytoplasm antibody-associated vasculitis. Nevertheless, eosinophilic Granulomatosis with Polyangiitis stands apart because it has features of vasculitis and eosinophilic disorders that require targeted therapies somewhat different from those used for other anti-neutrophil cytoplasm antibody-associated vasculitides. Considerable advances have been made in understanding the underlying pathophysiology of eosinophilic Granulomatosis with Polyangiitis that have highlighted the key role of eosinophils and opened new therapeutic opportunities. Its conventional treatment relies mainly on agents that decrease inflammation: corticosteroids and immunosuppressant adjunction for severe manifestations. New therapeutic approaches are needed for refractory disease, relapses and issues associated with corticosteroid dependence, especially for asthma manifestations. Drugs under evaluation mostly target eosinophils and B cells. Results of low-evidence-based trials suggested possible efficacies of biologicals: B-cell-blocking rituximab and anti-immunoglobulin E omalizumab. Recently, the first large-scale randomised controlled trial on eosinophilic Granulomatosis with Polyangiitis proved the efficacy of anti-interleukin-5 mepolizumab. That finding opens a new era in eosinophilic Granulomatosis with Polyangiitis management, with mepolizumab approval but also in future drug evaluations and trial designs for eosinophilic Granulomatosis with Polyangiitis. Additional studies are needed to determine which patients would benefit most from targeted therapies and achieve personalised treatment for patients with eosinophilic Granulomatosis with Polyangiitis. Herein, we review eosinophilic Granulomatosis with Polyangiitis characteristics and provide an overview of established and novel pharmacological agents.
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Vasculitis: Mepolizumab for eosinophilic Granulomatosis with Polyangiitis
Nature reviews. Rheumatology, 2017Co-Authors: Loïc GuillevinAbstract:Targeting IL-5 could improve outcomes and have steroid-sparing effects in the management of eosinophilic Granulomatosis with Polyangiitis. However, some questions remain about how this approach affects specific aspects of the disease.
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Treatment of Granulomatosis with Polyangiitis (Wegener’s)
Expert review of clinical immunology, 2015Co-Authors: Christian Pagnoux, Loïc GuillevinAbstract:Granulomatosis with Polyangiitis is a systemic necrotizing vasculitis characterized by granulomatous inflammation of small vessels and is associated with autoantibodies to neutrophil cytoplasmic proteases, mainly proteinase 3. Potentially lethal if not promptly diagnosed and treated, most patients with Granulomatosis with Polyangiitis can achieve remission with the current treatment modalities, with fewer side effects compared to three decades ago. However, the risk of relapse remains high, necessitating prolonged maintenance immunosuppressive therapy whose optimal duration remains undetermined. We review herein the treatment modalities for Granulomatosis with Polyangiitis and how they have evolved over the past decades. The findings of the most important and recently completed therapeutic studies, including on rituximab for maintenance, are summarized, before describing the main ongoing studies aimed at further optimizing treatment strategies.
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Imaging Features of Granulomatosis with Polyangiitis (Wegener’s)
Systemic Vasculitis, 2011Co-Authors: Stéphane Silvera, Loïc Guillevin, Estelle Palangié, Lotfi Hendaoui, Paul LegmannAbstract:Granulomatosis with Polyangiitis or Wegener’s Granulomatosis is a small-vessel vasculitis characterized by necrotizing granulomatous inflammation with involvement of: (1) Upper airways (sinusitis, otitis, ulcerations, bone deformities, subglotic or bronchial stenosis), (2) Lower respiratory tract (cough, chest pain, dyspnea, and hemoptysis), (3) Glomerulonephritis (hematuria, red blood cell casts, proteinuria, and azotemia). When Granulomatosis with Polyangiitis (Wegener’s) is suspected, the search to locate involvement of the upper respiratory tract, lungs and airways, CNS, or kidneys should include: (a) A clinical ORL exam and a sinonasal CT or MRI, (b) A high resolution chest CT, (c) An electrocardiogram (ECG), (d) Research of proteinuria and urinary sediment, (e) An ophthalmological exam, (f) Titers of c-ANCA and PR3 (antiprotease 3).
Michele V. Quan - One of the best experts on this subject based on the ideXlab platform.
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A rare case report of Polyangiitis overlap syndrome: Granulomatosis with Polyangiitis and eosinophilic Granulomatosis with Polyangiitis.
BMC pulmonary medicine, 2018Co-Authors: Michele V. Quan, Stephen K. Frankel, Mehrnaz Maleki-fischbach, Laren TanAbstract:Granulomatosis with Polyangiitis (GPA) is a systemic ANCA-associated vasculitis characterized by necrotizing granulomatous inflammation and a predilection for the upper and lower respiratory tract. Eosinophilic Granulomatosis with Polyangiitis (EGPA) is also a systemic ANCA-associated vasculitis, but EGPA is characterized by eosinophilic as well as granulomatous inflammation and is more commonly associated with asthma and eosinophilia. Polyangiitis overlap syndrome is defined as systemic vasculitis that does not fit precisely into a single category of classical vasculitis classification and/or overlaps with more than one category. Several Polyangiitis overlap syndromes have been identified, however, there are very few case reports of an overlap syndrome involving both GPA and EGPA in the medical literature. We conducted a PUBMED literature review using key words ‘Granulomatosis with Polyangiitis,’ ‘Wegener’s,’ ‘GPA,’ ‘eosinophilic Granulomatosis with Polyangiitis,’ ‘Churg-Strauss,’ ‘EGPA,’ ‘overlap syndrome,’ ‘Wegener’s with eosinophilia,’ and ‘GPA with eosinophilia’ in English only journals from 1986 to 2017. Relevant case reports and review articles of overlap syndromes of GPA and EGPA were identified. We aim to report a unique case of GPA and EGPA overlap syndrome and review the cases that have been previously described. Between 1986 and 2017, we identified 15 cases that represent an overlap syndrome with compelling features of both GPA and EGPA. Patients ranged in age between 21 and 78. Of those whose gender was identified, 80 % of the patients were female. All cases described involved the lungs, 60 % reported sinus involvement, and more than 50 % displayed renal involvement. An overwhelming majority of patients were positive for c-ANCA and demonstrated eosinophilia (peripheral blood or tissue eosinophilia). A preponderance of the cases described were treated with systemic corticosteroids combined with an immunosuppressive/cytotoxic agents. To our knowledge, there have been very few cases reported of an overlap syndrome of GPA and EGPA. Identification of patients with a Polyangiitis overlap syndrome of GPA and EGPA is imperative as prognosis, longitudinal management and treatment modalities may differ between these entities.
Oskar Ragnarsson - One of the best experts on this subject based on the ideXlab platform.
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Pituitary dysfunction in Granulomatosis with Polyangiitis.
Pituitary, 2017Co-Authors: Daniela Esposito, Penelope Trimpou, Dario Giugliano, Mats Dehlin, Oskar RagnarssonAbstract:Purpose Granulomatosis with Polyangiitis (GPA) is a multisystem disease, characterized by necrotizing small-vessel vasculitis, which mainly affects the respiratory tract and the kidneys. Pituitary involvement in GPA is rare, present in about 1% of all cases of GPA. To date, only case reports or small case series have been published. Herein we report clinical features, imaging findings, treatment and outcomes in three patients with GPA-related pituitary dysfunction (PD).
Mehrnaz Maleki-fischbach - One of the best experts on this subject based on the ideXlab platform.
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A rare case report of Polyangiitis overlap syndrome: Granulomatosis with Polyangiitis and eosinophilic Granulomatosis with Polyangiitis.
BMC pulmonary medicine, 2018Co-Authors: Michele V. Quan, Stephen K. Frankel, Mehrnaz Maleki-fischbach, Laren TanAbstract:Granulomatosis with Polyangiitis (GPA) is a systemic ANCA-associated vasculitis characterized by necrotizing granulomatous inflammation and a predilection for the upper and lower respiratory tract. Eosinophilic Granulomatosis with Polyangiitis (EGPA) is also a systemic ANCA-associated vasculitis, but EGPA is characterized by eosinophilic as well as granulomatous inflammation and is more commonly associated with asthma and eosinophilia. Polyangiitis overlap syndrome is defined as systemic vasculitis that does not fit precisely into a single category of classical vasculitis classification and/or overlaps with more than one category. Several Polyangiitis overlap syndromes have been identified, however, there are very few case reports of an overlap syndrome involving both GPA and EGPA in the medical literature. We conducted a PUBMED literature review using key words ‘Granulomatosis with Polyangiitis,’ ‘Wegener’s,’ ‘GPA,’ ‘eosinophilic Granulomatosis with Polyangiitis,’ ‘Churg-Strauss,’ ‘EGPA,’ ‘overlap syndrome,’ ‘Wegener’s with eosinophilia,’ and ‘GPA with eosinophilia’ in English only journals from 1986 to 2017. Relevant case reports and review articles of overlap syndromes of GPA and EGPA were identified. We aim to report a unique case of GPA and EGPA overlap syndrome and review the cases that have been previously described. Between 1986 and 2017, we identified 15 cases that represent an overlap syndrome with compelling features of both GPA and EGPA. Patients ranged in age between 21 and 78. Of those whose gender was identified, 80 % of the patients were female. All cases described involved the lungs, 60 % reported sinus involvement, and more than 50 % displayed renal involvement. An overwhelming majority of patients were positive for c-ANCA and demonstrated eosinophilia (peripheral blood or tissue eosinophilia). A preponderance of the cases described were treated with systemic corticosteroids combined with an immunosuppressive/cytotoxic agents. To our knowledge, there have been very few cases reported of an overlap syndrome of GPA and EGPA. Identification of patients with a Polyangiitis overlap syndrome of GPA and EGPA is imperative as prognosis, longitudinal management and treatment modalities may differ between these entities.
