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  1. Article ; Online: Polyarteritis Nodosa: State of the art.

    Puéchal, Xavier

    Joint bone spine

    2021  Volume 89, Issue 4, Page(s) 105320

    Abstract: Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis that preferentially affects medium-sized vessels. The idiopathic form has become rare. Its treatment relies on corticosteroid therapy and is combined with cyclophosphamide infusions for ... ...

    Abstract Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis that preferentially affects medium-sized vessels. The idiopathic form has become rare. Its treatment relies on corticosteroid therapy and is combined with cyclophosphamide infusions for severe forms. Secondary PANs were mainly associated with hepatitis B virus infection; they were treated with plasma exchange and antivirals in combination with short-term corticosteroid therapy. Other secondary forms of PAN are now becoming more common, such as those due to blood disorders. More recently, a monogenic form linked to adenosine deaminase-2 mutations has been identified. It requires treatment with TNF inhibitors to decrease the occurrence of ischemic central nervous system complications, which make it serious. Once remission is obtained, relapses are typically rare during PAN and affect 28% of idiopathic PANs, within an average of 26 months from the diagnosis. The prognosis has improved considerably, with 5- and 10-year survival rates of 83% and 74%.
    MeSH term(s) Adrenal Cortex Hormones ; Cyclophosphamide ; Hepatitis B ; Humans ; Neoplasm Recurrence, Local/complications ; Polyarteritis Nodosa/complications ; Polyarteritis Nodosa/diagnosis ; Polyarteritis Nodosa/therapy
    Chemical Substances Adrenal Cortex Hormones ; Cyclophosphamide (8N3DW7272P)
    Language English
    Publishing date 2021-12-11
    Publishing country France
    Document type Journal Article
    ZDB-ID 2020487-5
    ISSN 1778-7254 ; 1297-319X
    ISSN (online) 1778-7254
    ISSN 1297-319X
    DOI 10.1016/j.jbspin.2021.105320
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  2. Article ; Online: Granulomatosis with polyangiitis (Wegener's).

    Puéchal, Xavier

    Joint bone spine

    2020  Volume 87, Issue 6, Page(s) 572–578

    Abstract: Granulomatosis with polyangiitis (GPA) (Wegener) is a necrotizing vasculitis combining inflammation of the vascular wall and peri- and extravascular granulomatosis. Clinically, GPA is characterized in its full form by ENT signs, lung, and kidney ... ...

    Abstract Granulomatosis with polyangiitis (GPA) (Wegener) is a necrotizing vasculitis combining inflammation of the vascular wall and peri- and extravascular granulomatosis. Clinically, GPA is characterized in its full form by ENT signs, lung, and kidney involvement. One of the key features of GPA is the presence of ANCAs-cytoplasmic in approximately 90% of systemic forms and in 50% of localized forms-directed against proteinase 3 in most cases. PR3-ANCAs are highly specific to GPA and therefore have a high diagnostic value. Treatment of GPA is based on a combination of corticosteroids and cyclophosphamide or rituximab, which allows remission to be achieved in more than 80% of cases. Azathioprine was the most widely used maintenance treatment, but low-dose semi-annual rituximab maintenance infusions further decrease relapses with acceptable safety. Nevertheless, relapses occur in more than 50% of cases. One of the biggest treatment challenges is the occurrence of side effects, the severity and frequency of which are often linked to the prolonged treatment course, which is difficult to avoid.
    MeSH term(s) Antibodies, Antineutrophil Cytoplasmic ; Cyclophosphamide/therapeutic use ; Granulomatosis with Polyangiitis/diagnosis ; Granulomatosis with Polyangiitis/drug therapy ; Granulomatosis with Polyangiitis/epidemiology ; Humans ; Myeloblastin ; Rituximab/therapeutic use
    Chemical Substances Antibodies, Antineutrophil Cytoplasmic ; Rituximab (4F4X42SYQ6) ; Cyclophosphamide (8N3DW7272P) ; Myeloblastin (EC 3.4.21.76)
    Language English
    Publishing date 2020-06-17
    Publishing country France
    Document type Journal Article
    ZDB-ID 2020487-5
    ISSN 1778-7254 ; 1297-319X
    ISSN (online) 1778-7254
    ISSN 1297-319X
    DOI 10.1016/j.jbspin.2020.06.005
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  3. Article ; Online: Targeted immunotherapy strategies in ANCA-associated vasculitis.

    Puéchal, Xavier

    Joint bone spine

    2018  Volume 86, Issue 3, Page(s) 321–326

    Abstract: Targeted immunotherapy is substantially improving the management of ANCA-associated vasculitides (AAV), which include granulomatosis with polyangiitis (GPA, Wegener's granulomatosis), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with ... ...

    Abstract Targeted immunotherapy is substantially improving the management of ANCA-associated vasculitides (AAV), which include granulomatosis with polyangiitis (GPA, Wegener's granulomatosis), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss syndrome). This article reviews the current role for targeted immunotherapy in AAV, its validated indications, and avenues for further development. Rituximab is a validated induction treatment for GPA and severe MPA. Rituximab in these indications is not less effective than cyclophosphamide and is particularly useful in patients with refractory or relapsing disease, women of childbearing potential, and patients previously treated with cyclophosphamide. Rituximab is more effective than cyclophosphamide for treating relapses. For remission maintenance therapy, which is indispensable, rituximab has been proven superior over conventional immunosuppressive treatment. Rituximab is licensed in the USA and in Europe for the induction treatment of severe forms of GPA and MPA. An extension study for remission maintenance therapy is ongoing. In EGPA, although maintenance treatment with the interleukin-5 antagonist mepolizumab is effective in decreasing glucocorticoid requirements and in alleviating asthma and sinonasal symptoms, its efficacy on the vasculitis remains somewhat unclear. Mepolizumab is licensed for use in EGPA, and rituximab is also being evaluated as an induction and maintenance agent. Immunoglobulins can be helpful as an adjuvant treatment for active AAV with severe immunedepression, notably when infections occur. Plasma exchange is indicated in AAV with advanced renal dysfunction and, perhaps, in the event of alveolar hemorrhage, a possibility that will be assessed in 2018 in a large international study.
    MeSH term(s) Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/diagnosis ; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/drug therapy ; Antibodies, Monoclonal, Humanized/administration & dosage ; Biological Products/therapeutic use ; Cyclophosphamide/administration & dosage ; Female ; France ; Humans ; Immunosuppressive Agents/administration & dosage ; Immunotherapy/methods ; Male ; Molecular Targeted Therapy/methods ; Plasma Exchange ; Remission Induction ; Risk Assessment ; Rituximab/administration & dosage ; Treatment Outcome
    Chemical Substances Antibodies, Monoclonal, Humanized ; Biological Products ; Immunosuppressive Agents ; Rituximab (4F4X42SYQ6) ; Cyclophosphamide (8N3DW7272P) ; mepolizumab (90Z2UF0E52)
    Language English
    Publishing date 2018-09-07
    Publishing country France
    Document type Journal Article ; Review
    ZDB-ID 2020487-5
    ISSN 1778-7254 ; 1297-319X
    ISSN (online) 1778-7254
    ISSN 1297-319X
    DOI 10.1016/j.jbspin.2018.09.002
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  4. Article ; Online: How best to manage relapse and remission in ANCA-associated vasculitis.

    Puéchal, Xavier / Guillevin, Loïc

    Expert review of clinical immunology

    2022  Volume 18, Issue 11, Page(s) 1135–1143

    Abstract: Introduction: A two-stage therapeutic approach is now applied as standard-of-care to treat antineutrophil cytoplasm antibody (ANCA)-associated vasculitides (AAVs): first, glucocorticoids (GCs) combined with cyclophosphamide (CYC) or rituximab (RTX) to ... ...

    Abstract Introduction: A two-stage therapeutic approach is now applied as standard-of-care to treat antineutrophil cytoplasm antibody (ANCA)-associated vasculitides (AAVs): first, glucocorticoids (GCs) combined with cyclophosphamide (CYC) or rituximab (RTX) to induce remission, and then relapse prevention with remission-maintenance therapy. Nonetheless, a substantial risk of relapse persists.
    Areas covered: The authors provide an overview of the current state of AAV remission-induction after relapse and maintenance therapies, and discuss new strategies recommended to prevent and treat relapses, focusing on granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA).
    Expert opinion: For remission-induction after GPA or MPA relapse with organ-threatening manifestations, reintroduction or intensification of GCs in combination with CYC or RTX cycle is recommended; we prefer RTX in light of its superior responses obtained in patients with relapsing disease. Rapid tapering of GCs has been shown not to alter AAV evolution while decreasing the risk of serious infections. In contrast, for non-severe, active MPA, we recommend GCs alone as first-line therapy. For patients whose MPA remains uncontrolled by GCs alone, immunosuppressant adjunction can be a GC-sparing option or to counter GC intolerance. Once remission is achieved, we recommend prolonged maintenance therapy with preemptive low-dose (500 mg) RTX infusion biannually.
    MeSH term(s) Humans ; Rituximab/therapeutic use ; Antibodies, Antineutrophil Cytoplasmic ; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/drug therapy ; Microscopic Polyangiitis/drug therapy ; Remission Induction ; Cyclophosphamide/therapeutic use ; Immunosuppressive Agents/therapeutic use ; Recurrence ; Glucocorticoids/therapeutic use ; Granulomatosis with Polyangiitis/drug therapy ; Treatment Outcome
    Chemical Substances Rituximab (4F4X42SYQ6) ; Antibodies, Antineutrophil Cytoplasmic ; Cyclophosphamide (8N3DW7272P) ; Immunosuppressive Agents ; Glucocorticoids
    Language English
    Publishing date 2022-09-14
    Publishing country England
    Document type Journal Article
    ZDB-ID 2274260-8
    ISSN 1744-8409 ; 1744-666X
    ISSN (online) 1744-8409
    ISSN 1744-666X
    DOI 10.1080/1744666X.2022.2122954
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  5. Article ; Online: Finger ischaemia, enlarged spleen and lung nodules.

    Puéchal, Xavier / Delaval, Laure / Blanche, Philippe

    Annals of the rheumatic diseases

    2023  Volume 82, Issue 12, Page(s) 1635–1636

    MeSH term(s) Humans ; Splenomegaly ; Granulomatosis with Polyangiitis ; Lung Diseases ; Lung/diagnostic imaging ; Ischemia ; Antibodies, Antineutrophil Cytoplasmic ; Churg-Strauss Syndrome
    Chemical Substances Antibodies, Antineutrophil Cytoplasmic
    Language English
    Publishing date 2023-04-28
    Publishing country England
    Document type Journal Article
    ZDB-ID 7090-7
    ISSN 1468-2060 ; 0003-4967
    ISSN (online) 1468-2060
    ISSN 0003-4967
    DOI 10.1136/ard-2023-224161
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  6. Article ; Online: Whipple's arthritis.

    Puéchal, Xavier

    Joint bone spine

    2016  Volume 83, Issue 6, Page(s) 631–635

    Abstract: Whipple's disease is a chronic systemic infection that is due to the bacterial agent Tropheryma whipplei and can be cured by appropriate antibiotic therapy. The typical patient is a middle-aged man. Rheumatologists are in a prime position to handle ... ...

    Abstract Whipple's disease is a chronic systemic infection that is due to the bacterial agent Tropheryma whipplei and can be cured by appropriate antibiotic therapy. The typical patient is a middle-aged man. Rheumatologists are in a prime position to handle Whipple's disease. The classical presentation combines weight loss and diarrhea, preceded in three-quarters of patients by a distinctive pattern of joint manifestations that run an intermittent course, at least initially. The mean time from joint symptom onset to the diagnosis of Whipple's disease is 6 years. Either oligoarthritis or chronic polyarthritis with negative tests for rheumatoid factors (RFs) develops. If the diagnosis is missed, progression to chronic septic destructive polyarthritis may occur. Spondyloarthritis has also been reported, as well as a few cases of diskitis or, even more rarely, of hypertrophic osteoarthropathy. In most patients with the classical form of Whipple's disease, periodic acid-Schiff (PAS) staining of duodenal and jejunal biopsies shows macrophagic inclusions that contain bacteria. However, the involvement of the bowel may be undetectable clinically or, less often, histologically, and even PCR testing of bowel biopsies may be negative. Therefore, when nothing points to bowel disease, rheumatologists should consider T. whipplei infection in middle-aged men with unexplained intermittent oligoarthritis. PCR testing allows the detection of T. whipplei genetic material in joint fluid, saliva, and feces. This test is now a first-line diagnostic investigation, although T. whipplei is a rare cause of unexplained RF-negative oligoarthritis or polyarthritis in males. PCR testing can provide an early diagnosis before the development of severe systemic complications, which are still fatal in some cases.
    MeSH term(s) Adult ; Anti-Bacterial Agents/therapeutic use ; Arthritis/drug therapy ; Arthritis/etiology ; Arthritis/physiopathology ; Biopsy, Needle ; Diagnosis, Differential ; Follow-Up Studies ; Humans ; Immunohistochemistry ; Male ; Middle Aged ; Risk Assessment ; Treatment Outcome ; Tropheryma/isolation & purification ; Whipple Disease/complications ; Whipple Disease/diagnosis ; Whipple Disease/drug therapy
    Chemical Substances Anti-Bacterial Agents
    Language English
    Publishing date 2016-08-05
    Publishing country France
    Document type Journal Article ; Review
    ZDB-ID 2020487-5
    ISSN 1778-7254 ; 1297-319X
    ISSN (online) 1778-7254
    ISSN 1297-319X
    DOI 10.1016/j.jbspin.2016.07.001
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  7. Article ; Online: Therapeutic immunomodulation in eosinophilic granulomatosis with polyangiitis (Churg-Strauss).

    Puéchal, Xavier

    Joint bone spine

    2015  Volume 83, Issue 1, Page(s) 7–10

    MeSH term(s) Churg-Strauss Syndrome/drug therapy ; Granulomatosis with Polyangiitis ; Humans ; Immunomodulation
    Language English
    Publishing date 2015-11-27
    Publishing country France
    Document type Editorial ; Comment
    ZDB-ID 2020487-5
    ISSN 1778-7254 ; 1297-319X
    ISSN (online) 1778-7254
    ISSN 1297-319X
    DOI 10.1016/j.jbspin.2015.05.007
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  8. Article: Reply to the comment on: Microscopic polyangiitis: clinical characteristics and long-term outcomes of 378 patients from the French Vasculitis Study Group Registry by Nguyen et al.

    Nguyen, Yann / Puéchal, Xavier

    Clinical and experimental rheumatology

    2020  Volume 39 Suppl 128, Issue 1, Page(s) 9–10

    MeSH term(s) Antibodies, Antineutrophil Cytoplasmic ; Humans ; Microscopic Polyangiitis/diagnosis ; Microscopic Polyangiitis/drug therapy ; Microscopic Polyangiitis/epidemiology ; Registries
    Chemical Substances Antibodies, Antineutrophil Cytoplasmic
    Language English
    Publishing date 2020-09-29
    Publishing country Italy
    Document type Letter ; Comment
    ZDB-ID 605886-3
    ISSN 1593-098X ; 0392-856X
    ISSN (online) 1593-098X
    ISSN 0392-856X
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  9. Article ; Online: L12. Rheumatoid arthritis vasculitis.

    Puéchal, Xavier

    Presse medicale (Paris, France : 1983)

    2013  Volume 42, Issue 4 Pt 2, Page(s) 527–530

    MeSH term(s) Adrenal Cortex Hormones/therapeutic use ; Antibodies, Antineutrophil Cytoplasmic/blood ; Antibodies, Monoclonal, Murine-Derived/therapeutic use ; Biological Products/therapeutic use ; Blood Sedimentation ; Cohort Studies ; Cross-Sectional Studies ; Diagnosis, Differential ; Female ; Gastrointestinal Diseases/diagnosis ; Gastrointestinal Diseases/epidemiology ; Humans ; Immunosuppressive Agents/therapeutic use ; Incidence ; Male ; Mononeuropathies/diagnosis ; Mononeuropathies/epidemiology ; Prognosis ; Rheumatoid Vasculitis/diagnosis ; Rheumatoid Vasculitis/drug therapy ; Rheumatoid Vasculitis/epidemiology ; Rituximab ; Scleritis/diagnosis ; Scleritis/epidemiology ; Sex Factors ; Tumor Necrosis Factor-alpha/antagonists & inhibitors
    Chemical Substances Adrenal Cortex Hormones ; Antibodies, Antineutrophil Cytoplasmic ; Antibodies, Monoclonal, Murine-Derived ; Biological Products ; Immunosuppressive Agents ; Tumor Necrosis Factor-alpha ; Rituximab (4F4X42SYQ6)
    Language English
    Publishing date 2013-04
    Publishing country France
    Document type Journal Article ; Review
    ZDB-ID 120943-7
    ISSN 2213-0276 ; 0032-7867 ; 0755-4982 ; 0301-1518
    ISSN (online) 2213-0276
    ISSN 0032-7867 ; 0755-4982 ; 0301-1518
    DOI 10.1016/j.lpm.2013.01.031
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  10. Article ; Online: Mepolizumab in patients with eosinophilic granulomatosis with polyangiitis in remission: What is the right dose?

    Akdime, Florie / Puéchal, Xavier / Roche, Nicolas

    The journal of allergy and clinical immunology. In practice

    2021  Volume 9, Issue 7, Page(s) 2942–2943

    MeSH term(s) Antibodies, Monoclonal, Humanized/therapeutic use ; Churg-Strauss Syndrome/diagnosis ; Churg-Strauss Syndrome/drug therapy ; Granulomatosis with Polyangiitis/diagnosis ; Granulomatosis with Polyangiitis/drug therapy ; Humans ; Interleukin-5
    Chemical Substances Antibodies, Monoclonal, Humanized ; Interleukin-5 ; mepolizumab (90Z2UF0E52)
    Language English
    Publishing date 2021-07-10
    Publishing country United States
    Document type Letter ; Comment
    ZDB-ID 2843237-X
    ISSN 2213-2201 ; 2213-2198
    ISSN (online) 2213-2201
    ISSN 2213-2198
    DOI 10.1016/j.jaip.2021.03.061
    Database MEDical Literature Analysis and Retrieval System OnLINE

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