Mast Cell Activation Syndrome and Mastocytosis: Initial Treatment Options and Long-Term Management. Authors: Castells M1, Butterfield J2. 1 Division of Rheumatology, Allergy, and Immunology, Brigham and Women's Hospital Mastocytosis Center, Boston, Mass. Electronic address: mcastells@bwh.harvard.edu. 2 Mayo Clinic Program for Mast Cell and Eosinophil Disorders, Mayo Clinic, Rochester, Minn. Abstract: Patients with clonal mast cell activation syndromes (MCAS) including cutaneous and systemic mastocytosis (SM) may present with symptoms of mast cell activation, but in addition can have organ damage from the local effects of tissue infiltration by clonal mast cells. Patients with nonclonal MCAS may have chronic or episodic mast cell activation symptoms with an increase in serum tryptase and/or urinary metabolites of histamine, prostaglandin D2, and leukotrienes. Symptoms of MCAS and SM can be managed by blockade of mediator receptors (H1 and H2 antihistamines, leukotriene r...
Conclusiones:
ResponderEliminar1. La dermatitis atópica es una enfermedad heterogénea.
2. Los cuidados básicos de la piel es la estrategia terapéutica más importante.
3. El advenimiento de terapias biológicas con blancos terapéuticos dirigidos, podría modificar la historia natural de la enfermedad.
4. Recientes estudios han identificado un aumento en el riesgo de desarrollar malignidades de tipo linfoma, con el uso prolongado de inhibidores de la calcineurina.
Artículo recomendado:
ResponderEliminarAtopic dermatitis
Stephan Weidinger1*, Lisa A. Beck2, Thomas Bieber3,4, Kenji Kabashima5
and Alan D. Irvine6,7,8*
Abstract
Atopic dermatitis (AD) is the most common chronic inflammatory skin disease, with a lifetime prevalence of up to 20% and substantial effects on quality of life. AD is characterized by intense itch, recurrent eczematous lesions and a fluctuating course. AD has a strong heritability component and is closely related to and commonly co-occurs with other atopic diseases (such as
asthma and allergic rhinitis). Several pathophysiological mechanisms contribute to AD aetiology and clinical manifestations. Impairment of epidermal barrier function, for example, owing to deficiency in the structural protein filaggrin, can promote inflammation and T cell infiltration.
The immune response in AD is skewed towards T helper 2 cell-mediated pathways and can in turn favour epidermal barrier disruption. Other contributing factors to AD onset include dysbiosis of the skin microbiota (in particular overgrowth of Staphylococcus aureus), systemic immune
responses (including immunoglobulin E (IgE)-mediated sensitization) and neuroinflammation, which is involved in itch. Current treatments for AD include topical moisturizers and anti-inflammatory agents (such as corticosteroids, calcineurin inhibitors and cAMP-specific
3ʹ,5ʹ-cyclic phosphodiesterase 4 (PDE4) inhibitors), phototherapy and systemic immunosuppressants. Translational research has fostered the development of targeted small molecules and biologic therapies, especially for moderate-to-severe disease.
Texto completo:
https://doi.org/10.1038/s41572-018-0001-z