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  1. Article ; Online: ANCA positivity and the limited expression of vasculitides.

    Guilpain, Philippe

    Rheumatology (Oxford, England)

    2023  Volume 63, Issue 2, Page(s) 269–270

    MeSH term(s) Humans ; Antibodies, Antineutrophil Cytoplasmic ; Vasculitis ; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/diagnosis
    Chemical Substances Antibodies, Antineutrophil Cytoplasmic
    Language English
    Publishing date 2023-09-11
    Publishing country England
    Document type Journal Article
    ZDB-ID 1464822-2
    ISSN 1462-0332 ; 1462-0324
    ISSN (online) 1462-0332
    ISSN 1462-0324
    DOI 10.1093/rheumatology/kead389
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    Zs.B 240: Show issues Location:
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    Zs.MO 497: Show issues

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  2. Article ; Online: JAK inhibitors in autoinflammatory syndromes? The long road from drug development to daily clinical use.

    Guilpain, Philippe

    Rheumatology (Oxford, England)

    2023  Volume 62, Issue 4, Page(s) 1368–1369

    MeSH term(s) Humans ; Janus Kinase Inhibitors/therapeutic use ; Syndrome ; Arthritis, Juvenile ; Drug Development ; Still's Disease, Adult-Onset
    Chemical Substances Janus Kinase Inhibitors
    Language English
    Publishing date 2023-03-07
    Publishing country England
    Document type Journal Article ; Comment
    ZDB-ID 1464822-2
    ISSN 1462-0332 ; 1462-0324
    ISSN (online) 1462-0332
    ISSN 1462-0324
    DOI 10.1093/rheumatology/keac592
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    Zs.B 240: Show issues Location:
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  3. Article ; Online: Cancer-associated systemic sclerosis in a Canadian cohort: beyond strengths and weaknesses of epidemiological studies.

    Guilpain, Philippe / Mollevi, Caroline

    Rheumatology (Oxford, England)

    2022  Volume 61, Issue 7, Page(s) 2717–2719

    MeSH term(s) Autoantibodies/immunology ; Canada/epidemiology ; Humans ; Neoplasms ; Registries ; Scleroderma, Systemic/complications ; Scleroderma, Systemic/epidemiology ; Scleroderma, Systemic/immunology
    Chemical Substances Autoantibodies
    Language English
    Publishing date 2022-01-05
    Publishing country England
    Document type Editorial ; Comment
    ZDB-ID 1464822-2
    ISSN 1462-0332 ; 1462-0324
    ISSN (online) 1462-0332
    ISSN 1462-0324
    DOI 10.1093/rheumatology/keab924
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  4. Article ; Online: Editorial: Key Players in Systemic Sclerosis: The Immune System and Beyond.

    Guilpain, Philippe / Noël, Danièle / Avouac, Jérôme

    Frontiers in immunology

    2021  Volume 12, Page(s) 770419

    MeSH term(s) Animals ; Cell-Derived Microparticles/immunology ; Cell-Derived Microparticles/metabolism ; Cytokines/immunology ; Cytokines/metabolism ; Humans ; Immune System/cytology ; Immune System/immunology ; Immune System/metabolism ; Immunity, Innate/immunology ; Killer Cells, Natural/immunology ; Killer Cells, Natural/metabolism ; Myofibroblasts/cytology ; Myofibroblasts/immunology ; Scleroderma, Systemic/immunology ; Scleroderma, Systemic/metabolism ; Signal Transduction/immunology ; T-Lymphocytes, Regulatory/immunology
    Chemical Substances Cytokines
    Language English
    Publishing date 2021-09-30
    Publishing country Switzerland
    Document type Editorial ; Introductory Journal Article
    ZDB-ID 2606827-8
    ISSN 1664-3224 ; 1664-3224
    ISSN (online) 1664-3224
    ISSN 1664-3224
    DOI 10.3389/fimmu.2021.770419
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  5. Article ; Online: Risk of severe infection associated with immunoglobulin deficiency under rituximab therapy in immune-mediated inflammatory disease.

    Rempenault, Claire / Lukas, Cédric / Tardivon, Léa / Daien, Claire Immediato / Combe, Bernard / Guilpain, Philippe / Morel, Jacques

    RMD open

    2024  Volume 10, Issue 1

    Abstract: Objectives: We evaluated the risk of severe infection in patients with immune-mediated inflammatory disease (IMID) treated with RTX and with Ig deficiency.: Methods: This was an observational, retrospective single-centre study of patients undergoing ... ...

    Abstract Objectives: We evaluated the risk of severe infection in patients with immune-mediated inflammatory disease (IMID) treated with RTX and with Ig deficiency.
    Methods: This was an observational, retrospective single-centre study of patients undergoing treatment with at least one rituximab (RTX) infusion for an IMID until 31 May 2020. Patients were followed up for at least 12 months after the last infusion or until severe infection or death. Ig deficiency was classified as prevalent (before RTX) or acquired (normal Ig assay results before RTX but Ig deficiency during a follow-up).
    Results: Of 311 patients, 10.6% had prevalent and 19.6% acquired Ig deficiency. Prevalent Ig deficiency was related to concomitant treatment with glucocorticoids (GCs), in particular with a high daily dose at baseline; and acquired Ig deficiency to cumulative dose of RTX, mean Disease Activity Score in 28 joints (DAS28), immunosuppressor or GCs therapy at baseline, diabetes mellitus and obesity. Overall, 14.5% of patients had a severe infection during follow-up, which was numerically but not statistically more frequent in patients with prevalent Ig deficiency than normal Ig level. On multivariate analysis, risk of severe infection was associated with chronic pulmonary disease, GCs dose and mean DAS28-C reactive protein. In a time-dependent analysis, risk of severe infection was not associated with Ig deficiency, either acquired or prevalent (adjusted HR 1.04 (95% CI 0.5 to 2.3), p=0.92).
    Conclusion: Risk of severe infection was not associated with RTX-induced Ig deficiency in patients with an IMID. RTX management should be discussed according to an individual assessment of the infectious risk, especially in patients with GC therapy or chronic lung disease.
    MeSH term(s) Humans ; Rituximab/adverse effects ; Retrospective Studies ; Treatment Outcome
    Chemical Substances Rituximab (4F4X42SYQ6)
    Language English
    Publishing date 2024-01-30
    Publishing country England
    Document type Observational Study ; Journal Article
    ZDB-ID 2812592-7
    ISSN 2056-5933 ; 2056-5933
    ISSN (online) 2056-5933
    ISSN 2056-5933
    DOI 10.1136/rmdopen-2023-003415
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  6. Article ; Online: Subcutaneous tissue involvement in idiopathic inflammatory myopathies: Systematic literature review including three new cases and hypothetical mechanisms.

    Suzon, Benoit / Goulabchand, Radjiv / Louis-Sidney, Fabienne / Maria, Alexandre / Najjari, Redwann / Chauvet, Elodie / Le Quellec, Alain / Bessis, Didier / Guilpain, Philippe

    Autoimmunity reviews

    2023  Volume 22, Issue 4, Page(s) 103284

    Abstract: Introduction: Involvement of subcutaneous tissue in idiopathic inflammatory myopathies (IIM) is poorly known.: Methods: We conducted a systematic review of the literature regarding panniculitis and lipodystrophy/lipoatrophy in juvenile and adult IIM ... ...

    Abstract Introduction: Involvement of subcutaneous tissue in idiopathic inflammatory myopathies (IIM) is poorly known.
    Methods: We conducted a systematic review of the literature regarding panniculitis and lipodystrophy/lipoatrophy in juvenile and adult IIM via PubMed/Medline, Embase and Scopus databases. Three local observations are included in this review. Epidemiological, clinical, paraclinical and therapeutic data were collected.
    Results: Panniculitis appears to be more common in adults than in juveniles. It was mainly localised in the upper and lower limbs. Panniculitis improved in most cases with steroids and panniculitis and myositis had a similar course in 83.3% and 72.2% of cases in juveniles and adults, respectively. Lipodystrophy appeared to be more frequent in juveniles and was only observed in dermatomyositis in both juveniles and adults. Lipodystrophy was mainly partial in juveniles and adults. The median time from myositis to the diagnosis of lipodystrophy was 6 years [0-35] and 2.5 years [0-10] in juveniles and adults, respectively. Lipodystrophy was associated with anti-TIF1 gamma auto-antibody positivity, a polycyclic/chronic course of myositis and the occurrence of calcinosis and might be an indicator of poor disease control.
    Conclusion: Adipose tissue involvement, particularly lipodystrophy, occurs almost exclusively in dermatomyositis. The insidious onset and lack of awareness of the diagnosis may underestimate its prevalence. Larger studies are needed to identify possible risk factors in these patients, to better potential underlying pathophysiological process, in order to discuss potential therapeutic targets.
    MeSH term(s) Adult ; Humans ; Dermatomyositis/complications ; Subcutaneous Tissue ; Autoantibodies ; Myositis ; Panniculitis/complications ; Lipodystrophy/complications
    Chemical Substances Autoantibodies
    Language English
    Publishing date 2023-02-01
    Publishing country Netherlands
    Document type Systematic Review ; Case Reports ; Journal Article ; Review
    ZDB-ID 2144145-5
    ISSN 1873-0183 ; 1568-9972
    ISSN (online) 1873-0183
    ISSN 1568-9972
    DOI 10.1016/j.autrev.2023.103284
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    Zs.A 5913: Show issues Location:
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  7. Article ; Online: Long-term home parenteral nutrition in systemic sclerosis-related intestinal failure is feasible but unveils occult cardiac disease.

    Suzon, Benoit / Rivière, Sophie / Schiffmann, Auelie / Rivet, Valérian / Flori, Nicolas / Guilpain, Philippe / Maria, Alexandre Thibault Jacques

    Nutrition (Burbank, Los Angeles County, Calif.)

    2023  Volume 110, Page(s) 112009

    Abstract: Objective: The aim of this study was to compare safety and efficacy of long-term home parenteral nutrition between patients with systemic sclerosis and intestinal failure (IF) and controls with IF from another etiology.: Methods: A retrospective ... ...

    Abstract Objective: The aim of this study was to compare safety and efficacy of long-term home parenteral nutrition between patients with systemic sclerosis and intestinal failure (IF) and controls with IF from another etiology.
    Methods: A retrospective study was conducted in a referral center for systemic sclerosis (SSc) in Montpellier, France. Patients followed between 1985 and 2020 with SSc-related IF were included and compared with control patients with IF from another etiology. The patients included had to be treated for ≥4 wk by home parenteral nutrition (HPN). Primary outcome was occurrence of HPN-related complications. Secondary outcomes included duration of parenteral nutrition, body mass index at 12 mo, and survival.
    Results: Cumulative duration of HPN was 23 397 catheter days. HPN resulted in body mass index increase in both groups. There was no statistical difference regarding catheter-related bloodstream infections and thrombosis between the groups, despite use of immunosuppressive drugs and autologous hematopoietic stem cell transplantation in patients with SSc. However, the patients with SSc had significantly more HPN-related cardiac overload than the controls (P < 0.0001). Overloads occurred in SSc patients with and without cardiac disease, arguing for comprehensive hemodynamic screening in this condition.
    Conclusion: Long-term HPN in SSc-related IF is feasible but unveils occult cardiac disease.
    MeSH term(s) Humans ; Retrospective Studies ; Intestinal Failure ; Parenteral Nutrition, Home/adverse effects ; Scleroderma, Systemic/complications ; Scleroderma, Systemic/therapy ; Catheter-Related Infections/epidemiology ; Catheter-Related Infections/etiology ; Heart Diseases/etiology ; Heart Diseases/therapy ; Intestinal Diseases/etiology ; Intestinal Diseases/therapy
    Language English
    Publishing date 2023-02-23
    Publishing country United States
    Document type Journal Article
    ZDB-ID 639259-3
    ISSN 1873-1244 ; 0899-9007
    ISSN (online) 1873-1244
    ISSN 0899-9007
    DOI 10.1016/j.nut.2023.112009
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  8. Article ; Online: About the complexity of adult onset Still's disease… and advances still required for its management.

    Guilpain, Philippe / Le Quellec, Alain

    BMC medicine

    2017  Volume 15, Issue 1, Page(s) 5

    Abstract: Adult onset Still's disease (AOSD) is a rare inflammatory disorder that remains poorly understood. Its pathophysiology is yet to be completely elucidated, but is known to consist mainly on a cytokine cascade, responsible for the systemic manifestations. ... ...

    Abstract Adult onset Still's disease (AOSD) is a rare inflammatory disorder that remains poorly understood. Its pathophysiology is yet to be completely elucidated, but is known to consist mainly on a cytokine cascade, responsible for the systemic manifestations. AOSD diagnosis is usually difficult and delayed, with physicians having to rule out several other conditions, including cancer or infectious diseases. Prognosis is heterogeneous and difficult to establish, ranging from benign outcome to chronic destructive polyarthritis and/or life-threatening events. In addition, treatment remains to be codified, especially considering the development of new drugs. In this commentary, we attempt to elucidate the complexity of AOSD and to highlight the need of working on prognostic tools for this disorder. We also discuss the numerous advances that would be useful for patients in the daily management of this disease.Please see related article: http://bmcmedicine.biomedcentral.com/articles/ 10.1186/s12916-016-0738-8 .
    MeSH term(s) Adaptive Immunity ; Adult ; Biological Therapy/methods ; Cytokines/therapeutic use ; Humans ; Prognosis ; Still's Disease, Adult-Onset/diagnosis ; Still's Disease, Adult-Onset/therapy
    Chemical Substances Cytokines
    Language English
    Publishing date 2017-01-06
    Publishing country England
    Document type Editorial
    ISSN 1741-7015
    ISSN (online) 1741-7015
    DOI 10.1186/s12916-016-0769-1
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  9. Article ; Online: Challenges of autoimmune rheumatic disease treatment during the COVID-19 pandemic: A review.

    Grange, Lucile / Guilpain, Philippe / Truchetet, Marie-Elise / Cracowski, Jean-Luc

    Therapie

    2020  Volume 75, Issue 4, Page(s) 335–342

    Abstract: Since December 2019, the COVID-19 pandemic has become a major public health problem. To date, there is no evidence of a higher incidence of COVID in patients with autoimmune rheumatic diseases and we support the approach of maintaining chronic ... ...

    Abstract Since December 2019, the COVID-19 pandemic has become a major public health problem. To date, there is no evidence of a higher incidence of COVID in patients with autoimmune rheumatic diseases and we support the approach of maintaining chronic rheumatological treatments. However, once infected there is a small but significant increased risk of mortality. Among the different treatments, NSAIDs are associated with higher rates of complications, but data for other drugs are conflicting or incomplete. The use of certain drugs for autoimmune inflammatory rheumatisms appears to be a potentially interesting options for the treatment. The rationale for their use is based on the immune system runaway and the secretion of pro-inflammatory cytokines (Il1, IL6, TNFα) in severe forms of the disease. Notably, patients on chloroquine or hydroxychloroquine as a treatment for their autoimmune rheumatic disease are not protected from COVID-19.
    MeSH term(s) Anti-Inflammatory Agents, Non-Steroidal/administration & dosage ; Antirheumatic Agents/administration & dosage ; Autoimmune Diseases/drug therapy ; Autoimmune Diseases/epidemiology ; COVID-19 ; Chloroquine/administration & dosage ; Coronavirus Infections/epidemiology ; Coronavirus Infections/mortality ; Humans ; Hydroxychloroquine/administration & dosage ; Incidence ; Pandemics ; Pneumonia, Viral/epidemiology ; Pneumonia, Viral/mortality ; Rheumatic Diseases/drug therapy ; Rheumatic Diseases/epidemiology
    Chemical Substances Anti-Inflammatory Agents, Non-Steroidal ; Antirheumatic Agents ; Hydroxychloroquine (4QWG6N8QKH) ; Chloroquine (886U3H6UFF)
    Keywords covid19
    Language English
    Publishing date 2020-06-27
    Publishing country France
    Document type Journal Article ; Review
    ZDB-ID 603474-3
    ISSN 1958-5578 ; 0040-5957
    ISSN (online) 1958-5578
    ISSN 0040-5957
    DOI 10.1016/j.therap.2020.06.013
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    Ue I Zs.272: Show issues Location:
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  10. Article ; Online: Therapeutic innovation in adult-onset Still's disease (and other rare inflammatory disorders): how to secure evidence-based medicine?

    Guilpain, Philippe / Le Quellec, Alain / Maria, Alexandre Thibault Jacques

    Annals of the rheumatic diseases

    2018  Volume 77, Issue 12, Page(s) 1699–1701

    MeSH term(s) Adult ; Evidence-Based Medicine ; Humans ; Rare Diseases ; Still's Disease, Adult-Onset
    Language English
    Publishing date 2018-06-02
    Publishing country England
    Document type Editorial ; Comment
    ZDB-ID 7090-7
    ISSN 1468-2060 ; 0003-4967
    ISSN (online) 1468-2060
    ISSN 0003-4967
    DOI 10.1136/annrheumdis-2018-213106
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