Is blood vitamin B6 a marker of interest for individualized psoriasis disease care management in cardiovascular disease prevention?

Psoriasis disease (PsD) is an immune-mediated inflammatory disease (IMID) associated with comorbidities, as cardiovascular diseases (CVD). Several factors have been identified as trigger for CVD in PsD. As for other IMID, we propose to investigate the role of vitamin B6 (B6) in hyperhomocystinemia and cardiovascular risk management in order to individualize PsD care psoriasis.

Patient-Reported Outcomes and Provider Perceptions of Systemic Mastocytosis: Results From the PRISM Study.

Background: Systemic mastocytosis (SM) is a clonal mast cell disease primarily driven by the KIT D816V mutation and often characterised by unpredictable and debilitating symptoms. The Perceptions Realities and Insights on Systemic Mastocytosis (PRISM) survey queried patient and provider perceptions of SM in Europe.

Methods: PRISM (funded by Blueprint Medicines Corporation) was composed of two independent surveys: a 119-item patient survey on diagnosis, symptom burden, quality of life (QoL) and work impact; and a 103-item healthcare provider (HCP) survey on approaches to SM diagnosis and management.

Management of Bone Health in Adult Mastocytosis.

Purpose Of Review: The present review will examine bone disease in mastocytosis, analyze the existing literature on its management, and propose a strategy for osteoporosis treatment in systemic mastocytosis. This strategy is based on both the available scientific evidence and the experience gained at our expert center (CEREMAST).

Recent Findings: Systemic mastocytosis is a rare disorder, primarily affecting the bone and leading to osteoporosis, bone pain, and bone structural abnormalities.

Generalized pustular psoriasis: a multicentric study on patient characteristics and clinical burden.

The objective of this study was to assess the demographic characteristics and impact on quality of life (QoL) of patients with PPG in France through a multicentre study. The results of the study are as follows: The PRO [PUSH-D, PHQ-9 et GAD-7] revealed that more than half of the patients exhibited a significant impact on their quality of life. High scores for fatigue, stress, skin and joint pain were reported, with 65% of patients at risk of mild to severe depression.

Lifetime Disability-Adjusted Life-Year Assessment of Indolent Systemic Mastocytosis.

Background: Indolent systemic mastocytosis (ISM) is a rare disease associated with numerous and diverse symptoms that significantly affect patients' overall health and psychological, emotional, and professional well-being, ultimately affecting the quality of life.

Objective: To estimate the disability-adjusted life-years (DALY) of ISM to assess the burden for patients and society.

Methods: We used prospective and retrospective data on symptoms and quality of life from a population with ISM recruited at the French expert center CEREMAST, to estimate disability weight allowing DALY calculation.

Histological characterization of liver involvement in systemic mastocytosis.

Background And Aims: Systemic mastocytosis (SM) is characterized by the accumulation of atypical mast cells (MCs) in organs. Liver histology of SM has been marginally described and accurate histological classification is critical, given the consequences of aggressive SM diagnosis. We aimed to describe the histological features associated with liver SM using updated tools.

Avapritinib versus Placebo in Indolent Systemic Mastocytosis.

BACKGROUND: Indolent systemic mastocytosis (ISM) is a clonal mast-cell disease driven by the KIT D816V mutation. We assessed the efficacy and safety of avapritinib versus placebo, both with best supportive care, in patients with ISM. METHODS: We randomized patients with moderate to severe ISM (total symptom score [TSS] of ≥28; scores range from 0 to 110, with higher numbers indicating more severe symptoms) two to one to avapritinib 25 mg once daily (n=141) or placebo (n=71).

Pathophysiologic implications of elevated prevalence of hereditary alpha-tryptasemia in all mastocytosis subtypes.

Background: Mastocytosis and monoclonal mast cell (MC) activation syndrome (MMAS) are heterogeneous conditions characterized by the accumulation of atypical MCs. Despite the recurrent involvement of KIT mutations, the pathophysiologic origin of mastocytosis and MMAS is unclear. Although hereditary α-tryptasemia (HαT, related to TPSAB1 gene duplication) is abnormally frequent in these diseases, it is not known whether the association is coincidental or causal.

3D deconvolution of human skin immune architecture with Multiplex Annotated Tissue Imaging System.

Routine clinical assays, such as conventional immunohistochemistry, often fail to resolve the regional heterogeneity of complex inflammatory skin conditions. We introduce MANTIS (Multiplex Annotated Tissue Imaging System), a flexible analytic pipeline compatible with routine practice, specifically designed for spatially resolved immune phenotyping of the skin in experimental or clinical samples. On the basis of phenotype attribution matrices coupled to α-shape algorithms, MANTIS projects a representative digital immune landscape while enabling automated detection of major inflammatory clusters and concomitant single-cell data quantification of biomarkers.

Effective Anti-SARS-CoV-2 Immune Response in Patients With Clonal Mast Cell Disorders.

Background: Mast cells are key players in innate immunity and the T2 adaptive immune response. The latter counterbalances the T1 response, which is critical for antiviral immunity. Clonal mast cell activation disorders (cMCADs, such as mastocytosis and clonal mast cell activation syndrome) are characterized by abnormal mast cell accumulation and/or activation.