ABSTRACT
A urticária aguda é uma causa frequente de consulta com alergistas, caracterizada por urticas e/ou angioedema. Embora autolimitada e benigna, pode causar desconforto significativo e raramente representar uma doença sistêmica grave ou reação alérgica com risco de vida. Nesta revisão, elaborada pelo Departamento Científico de Urticária da Associação Brasileira de Alergia e Imunologia, foram abordadas as principais questões referentes ao tema para auxiliar o médico especialista e generalista.
Acute urticaria is a frequent cause of consultations with allergists, being characterized by wheals and/or angioedema. Although self-limited and benign, it may cause significant discomfort and uncommonly represent a serious systemic disease or life-threatening allergic reaction. In this review prepared by the Urticaria Scientific Department of the Brazilian Association of Allergy and Immunology, the main questions about this topic are addressed to help specialists and general practitioners.
Subject(s)
Humans , Urticaria , Epinephrine , Milk Hypersensitivity , Egg Hypersensitivity , Drug Hypersensitivity , Shellfish Hypersensitivity , Nut and Peanut Hypersensitivity , Histamine H1 Antagonists , Anaphylaxis , Spider Bites , Physicians , Societies, Medical , Therapeutics , Anti-Inflammatory Agents, Non-Steroidal , Sweet Syndrome , Dermatitis, Allergic Contact , Adrenal Cortex Hormones , Hypereosinophilic Syndrome , Schnitzler Syndrome , Mastocytosis, Cutaneous , Diagnosis , Allergy and Immunology , Erythema , Angioedemas, Hereditary , Food Hypersensitivity , Allergists , Hypersensitivity , AngioedemaABSTRACT
El síndrome de Schnitzler es un raro trastorno caracterizado por rash urticariano crónico y gammapatía monoclonal, acompañado de fiebre intermitente, artralgias o artritis, dolor óseo y linfadenopatías, en el cual la interleuquina 1 (IL-1) tiene un papel preponderante. Se presenta el caso de un varón de 48 años que reúne criterios para síndrome de Schnitzler y que luego de fallar a múltiples tratamientos presenta respuesta exitosa a canakinumab
Schnitzler syndrome is a rare disorder characterized by chronic urticarial rash and monoclonal gammopathy, accompanied by intermittent fever, arthralgia or arthritis, bone pain and lymphadenopathy, in which interleukin 1 (IL-1) has a preponderant role. The case of a 48-year-old male who meets criteria for Schnitzler syndrome and who after failing too many treatments presents a successful response to canakinumab is presented here
Subject(s)
Therapeutics , Schnitzler SyndromeABSTRACT
Chronic urticaria may often be associated with interleukin (IL)-1-mediated autoinflammatory disease, which should be suspected if systemic inflammation signs are present. Here, we report a case of Schnitzler's syndrome without monoclonal gammopathy treated successfully with the IL-1 receptor antagonist anakinra. A 69-year-old man suffered from a pruritic urticarial rash for 12 years. It became aggravated episodically and was accompanied by high fever, arthralgia, leukocytosis, and an elevated C-reactive protein and erythrocyte sedimentation rate. The episodes each lasted for over one week. Neutrophilic and eosinophilic inflammation was found on skin biopsy. However, serum and urine electrophoresis showed no evidence of monoclonal gammopathy. The cutaneous lesions were unresponsive to various kinds of anti-histamines, systemic glucocorticoids, colchicine, cyclosporine, dapsone, and methotrexate, which were administered over a span of 3 years immediately preceding successful treatment. A dramatic response, however, was observed after a daily administration of anakinra. This observation suggests that the correct diagnosis of this case is Schnitzler's syndrome without monoclonal gammopathy. For an adult patient with refractory chronic urticaria and systemic inflammation, Schnitzler's syndrome could be considered as a possible differential diagnosis. Although the typical form of Schnitzler's syndrome exhibits the presence of monoclonal gammopathy as a diagnostic criterion, monoclonal gammopathy may be absent in an atypical form. In such a situation, an IL-1 antagonist should be effective for the management of chronic urticaria.
Subject(s)
Aged , Humans , Male , Blood Sedimentation , C-Reactive Protein/metabolism , Chronic Disease , Interleukin 1 Receptor Antagonist Protein/therapeutic use , Leukocytes/metabolism , Paraproteinemias/complications , Schnitzler Syndrome/blood , Schnitzler Syndrome/drug therapy , Urticaria/complicationsABSTRACT
As doenças autoinflamatórias sistêmicas são um grupo de doenças raras recentemente descritas, mas que vêm ganhando espaço no cenário clínico. Caracterizam-se por alterações da imunidade inata, portanto sem a presença de linfócito T autorreator ou autoanticorpo, e que respondem ao bloqueio de uma única citocina. Esta revisão tem como objetivo analisar a base imunofisiológica destas doenças e descrever brevemente cada uma delas com suas características clínicas mais importantes.
Systemic autoinflammatory diseases are a group of rare diseases only recently described but rapidly growing in importance in the clinical setting. They are characterized by innate immunity impairment, i.e., absence of autoreactive T lymphocytes or autoantibodies, and respond to individual cytokine blockade. The objective of this review was to analyze the immunophysiological basis of these diseases and to briefly describe each of them along with their most relevant clinical characteristics.