Publications by authors named "Cathebras P"

Article Synopsis
  • MGUS-associated angioedema due to acquired C1 inhibitor deficiency (AAE-C1-INH) hasn't been specifically characterized before, prompting this study to explore its biological and clinical features over 30 years in France.
  • In a study of 41 patients, most had anti-C1INH antibodies, and treatments included acute management and long-term prophylaxis, with a significant number developing malignant blood disorders like lymphoma or myeloma.
  • The study found a link between the remission of angioedema and the underlying hematological malignancy, emphasizing the need for regular hematological evaluations in patients with MGUS-AE-C1-INH.
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  • The study examines the blood-related characteristics and overall prognosis of 127 patients with telomere biology disorders (TBD) who were diagnosed after age 15, highlighting a lack of data on this topic.
  • At diagnosis, significant haematological issues were present in nearly 76% of patients, with bone marrow failure (BMF) being the most common, affecting 46.5% of the cases, while some patients also developed additional complications over time.
  • The findings suggest that BMF patients tend to be younger and have a better survival rate compared to those with higher-risk blood cancers, indicating TBD as a complex multi-organ disease needing further research on its evolutionary nature and outcomes.
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Introduction: Severe acute respiratory syndrome coronavirus 2 is a viral respiratory infection that can cause systemic disorders and lead to death, particularly in older people. Proton pump inhibitors (PPIs) increase the risk of enteric and lung infections. Considering the broad use of PPIs in older people, the potential role of PPIs in COVID-19 could be of dramatic significance.

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  • Health anxiety (HA) affects around 20% of patients in secondary care, leading to decreased well-being, increased healthcare costs, and various health complications.
  • The rise in anxiety related to health issues is linked to factors such as increased medicalization, media focus on health, and the rise of "cyberchondria" due to internet use, with the COVID-19 pandemic heightening overall psychological distress.
  • Effective diagnosis of HA should be integral to medical consultations, and cognitive behavioral therapy (CBT) is a key treatment; however, there is a shortage of affordable, trained therapists, especially in France.
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  • This study evaluated the effectiveness and safety of the IL-5 receptor inhibitor benralizumab for treating patients with eosinophilic granulomatosis with polyangiitis (EGPA) across 28 European centers.
  • Out of 121 patients treated, complete responses increased from 12.4% at 3 months to 46.4% at 12 months, while partial responses decreased over the same period.
  • Notable improvements were seen in disease activity, as measured by the Birmingham Vasculitis Activity Score (BVAS), and a reduction in various disease manifestations, alongside better lung function.
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Background: Benralizumab is effective in the treatment of eosinophilic asthma and is being investigated for the treatment of other eosinophil-associated diseases. Reports on the use of benralizumab for the treatment of eosinophilic granulomatosis with polyangiitis (EGPA) are limited to case reports and small case series.

Methods: We conducted a multicentre, retrospective study including EGPA patients treated with off-label benralizumab.

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Background: Activated phosphoinositide-3-kinase δ syndrome (APDS) is an inborn error of immunity (IEI) with infection susceptibility and immune dysregulation, clinically overlapping with other conditions. Management depends on disease evolution, but predictors of severe disease are lacking.

Objectives: This study sought to report the extended spectrum of disease manifestations in APDS1 versus APDS2; compare these to CTLA4 deficiency, NFKB1 deficiency, and STAT3 gain-of-function (GOF) disease; and identify predictors of severity in APDS.

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Chronic fatigue is a frequent complaint, expressed at all levels of the healthcare system. It is perceived as disabling in a high proportion of cases, and internists are frequently called upon to find "the" cause. The etiological diagnostic approach of an unexplained state of fatigue relies on the careful search for more specific clues by questioning and clinical examination.

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Aims: This study aimed to report the association of focal myositis (FM) and Behçet's disease (BD) and to analyse the main characteristics of such an association.

Methods: This is a retrospective multicentre study of patients with BD and FM (BD + FM+ group) and those without FM (BD - FM+ group). Clinical, laboratory, radiological, pathological, treatment and outcome data were analysed.

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  • Population differences significantly impact disease severity and treatment effects in biomedical research, yet there is a lack of rigorous practices in labeling subpopulations, especially for complex diseases like cancer.
  • Current classification terms like "Black" and "Caucasian" are overly broad and outdated, leading to inconsistencies in clinical research and potentially obscuring other critical factors influencing health.
  • To improve biomedical practices, a collaborative effort among various disciplines is needed to create clearer, more scientifically accurate labeling and to address the complexities of human diversity in medical research.
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Objective: While myocardial impairment is a predictor of poor prognosis in antineutrophil cytoplasm antibody (ANCA)-associated vasculitides (AAV), little is known about valvular involvement. This study aims at describing the clinical presentation, management, and outcome of endocarditis associated with AAV.

Methods: We conducted a multicenter retrospective study in centers affiliated with the French Vasculitis Study Group.

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Introduction: Neurologic and muscular damage associated with acute hepatitis due to hepatitis E virus (HEV) are rare and may be underdiagnosed.

Case Report: We report the case of a 56-year-old man, presenting with flaccid tetraparesis secondary to an acute rhabdomyolysis induced by acute E virus hepatitis. He fully recovered after one month under supportive treatment.

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Objectives: To describe the characteristics, treatment and outcome of patients with immune thrombocytopenia with clinical significance (ITPCS) associated with SLE.

Methods: This retrospective multicentre study included SLE patients who experienced ≥1 ITPCS (defined as ITP with attributable bleeding disorders and/or a platelet count <30×109/l). Other causes of secondary thrombocytopenia were excluded.

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Sjögren's syndrome (SjS) is a frequent systemic autoimmune disease responsible for a major decrease in patients' quality of life, potentially leading to life-threatening conditions while facing an unmet therapeutic need. Hence, we assessed the immunogenicity, efficacy, and tolerance of IFN-Kinoid (IFN-K), an anti-IFNα vaccination strategy, in a well-known mouse model of systemic autoimmunity with SjS-like features: MRL/MpJ-Faslpr/lpr (MRL/lpr) mice. Two cohorts (with ISA51 or SWE01 as adjuvants) of 26 female MRL/lpr were divided in parallel groups, "controls" (not treated, PBS and Keyhole Limpet Hemocyanin [KLH] groups) or "IFN-K" and followed up for 122 days.

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Unlabelled: Type 1 Gaucher disease (GD) is a rare autosomal recessive lysosomal storage disorder caused by deficient activity of beta-glucocerebrosidase, leading to accumulation of its substrate (glucosylceramide) in macrophages of the reticuloendothelial system, which are then referred to as Gaucher cells. The most frequent symptoms are asthenia, spleen and liver enlargement, bone abnormalities and cytopenia due to bone marrow infiltration. Lung involvement in GD is a rare finding, and it is unclear whether it may regress under enzyme replacement therapy (ERT) or substrate reduction therapy (SRT).

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In this work, we address the issue of prolonged symptoms following an infection by SARS-CoV-2, labeled "long COVID". This clinically unspecific syndrome must be put in perspective with the post-infectious syndromes known for a long time but ultimately poorly understood and little studied, qualified, for lack of convincing arguments for a unambiguous pathophysiology and better terms, as functional somatic syndromes. The clinical implications for clinical care ("holistic" work-up and care of patients), for research (need for truly "bio-psycho-social" investigations), and the social implications of "long COVID" (social construction of the syndrome through the experiences of patients exposed on social networks, inequalities in the face of the disease and its socioeconomic consequences) are considered.

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Fibromyalgia (FM) is characterized by multiple symptoms including pain, fatigue, and sleep disorders, altering patient's quality of life. In the absence of effective pharmacological therapy, the last European guidelines recommend a multidisciplinary management based on exercise and education. Thus, our main objective was to measure the effectiveness of a healthcare organization offering a specific program of adapted physical activity combined with a therapeutic education program for FM patients.

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This paper argues that "functional," "medically unexplained," or "somatoform" symptoms and disorders necessarily require a patient-centered approach from the clinicians. In the first part, I address the multiple causes of the patients' suffering and I analyze the unease of the doctors faced with these disorders. I emphasize the iatrogenic role of medical investigations and the frequent failure in attempting to reassure the patients.

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Introduction: Periodic spontaneous hypothermia syndrome (PSHS), also known as hypersudation-hypothermia syndrome, is a rare condition characterised by the occurrence of paroxysmal attacks of spontaneous hypothermia occurring without age limit. Few studies have attempted to synthesise the available data on PSHS. The objective of this study was to summarize the literature on this condition.

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Introduction: Immune monitoring of monoclonal antibodies is a helpful tool in optimizing the management of patients treated with TNF blockers, especially in gastroenterology. In contrast, studies evaluating the interest of such monitoring are lacking for other monoclonal antibodies used in autoimmune diseases, including rituximab despite its widespread use in the field for almost 15 years. Hence, we conducted a study whose goal was to describe the clinical and biological characteristics of all patients who had a rituximab immune monitoring.

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