作为不完整的施尼茨勒综合征的长期症状,耐治疗性疹
Viktoria Puxkandl1, Antonia Currie1, Wolfram Hoetzenecker2
1Department of Dermatology, Comprehensive Allergy Center, Kepler University Hospital Linz & Johannes Kepler University, Krankenhausstrasse 9, 4020, Linz, Austria.
概括
施尼茨勒综合征是一种罕见的自身免疫性疾病,可以出现持续的疹和延迟的症状. 干白素-1 (IL-1) 抗剂,如卡纳基努马布,为这种情况提供有效的治疗方法.
科学领域:
- 类风湿病学 类风湿病学
- 免疫学 免疫学 免疫学
- 皮肤病学 皮肤病学
背景情况:
- 施尼茨勒综合征是一种罕见的自身免疫性疾病,其特征是慢性疹,IgM-糖性病,发烧和关节疼痛.
- 目前,对于施尼茨勒综合征没有批准的特定治疗方法,但IL-1抗剂在诱导缓解方面表现有前途.
研究的目的:
- 报告在长期耐治疗性疹症后诊断出的施尼茨勒综合征病例.
- 突出IL-1抗剂在控制施尼茨勒综合征症状中的有效性.
主要方法:
- 一名患有抗胰岛素药物和奥马利祖马布耐药的慢性疹患者在几年内被监测.
- 施尼茨勒综合征的诊断标准是在关节疼痛,发烧和IgM糖性病变发生后得到满足的.
- 治疗是用anakinra开始的,其次是canakinumab,因为不良反应.
主要成果:
- 最初的阿纳金拉治疗导致症状改善,但引起了注射部位反应,需要停止治疗.
- 随后使用长效IL-1抗剂卡纳基努马布进行治疗,导致施尼茨勒综合征症状完全缓解.
- 该案例说明了延迟诊断施尼茨勒综合征的潜力.
结论:
- 患有持续性疹不响应标准治疗的患者需要重新评估诸如施尼茨勒综合征之类的疾病,即使是几年后.
- 在施尼茨勒综合征中,甘马病和其他特征性症状的发展可能会被延迟.
- IL-1抗剂代表了施尼茨勒综合征的可行的治疗选择,更长效的药物可能提供更好的耐受性.
相关概念视频
Allergic Reactions
27.8K
Overview
27.8K
Hypersensitivities
747
Hypersensitivity, also known as a hypersensitivity reaction or allergic reaction, is a condition where the body's immune system reacts abnormally to a foreign substance. Such substances, that cause hypersensitivity are referred to as an allergen, could be something typically harmless to most people, like pollen or certain foods.
Types of Hypersensitivities
Hypersensitivity reactions are categorized into four types: Type 1, Type 2, Type 3, and Type 4. Each type has a distinct mechanism...
Types of Hypersensitivities
Hypersensitivity reactions are categorized into four types: Type 1, Type 2, Type 3, and Type 4. Each type has a distinct mechanism...
747
Desensitization and Tachyphylaxis
1.8K
Tachyphylaxis is described as a rapid decrease in response to a drug after repeated or continuous administration of the same drug dose. It is a phenomenon where the body becomes less responsive to a particular substance or intervention over time, requiring higher doses or stronger interventions to achieve the same effect. It results from adaptive changes in the body's receptors, signaling pathways, or physiological processes that occur in response to prolonged exposure to a stimulus.
1.8K
Rheumatic Heart Disease I: Introduction
17
Rheumatic heart disease or RHD is a chronic condition that results from rheumatic fever, causing permanent damage to the heart valves.Etiology and Risk FactorsIt primarily arises from rheumatic fever, an inflammatory disease that can develop after untreated or inadequately treated group A streptococcal (GAS) pharyngitis. Streptococcus spreads through direct contact with oral or respiratory secretions. While the bacteria are the causative agents, factors like malnutrition, overcrowding, poor...
17
Chronic Bowel Disorders: Introduction
486
Chronic bowel diseases are a group of long-term conditions affecting the digestive tract, characterized by inflammation and damage to the gut lining. These conditions primarily include irritable bowel syndrome and inflammatory bowel disease.
Irritable Bowel Syndrome (IBS) is a common disorder affecting the gastrointestinal tract. The distinctive feature is recurrent abdominal pain associated with altered bowel movements, manifesting as constipation, diarrhea, or fluctuating between both. The...
Irritable Bowel Syndrome (IBS) is a common disorder affecting the gastrointestinal tract. The distinctive feature is recurrent abdominal pain associated with altered bowel movements, manifesting as constipation, diarrhea, or fluctuating between both. The...
486
Chronic Kidney Disease II: Clinical Manifestations
32
Chronic Kidney Disease (CKD) progressively impairs multiple body systems due to the accumulation of uremic toxins, which disrupt cellular functions across various organs.Neurologic symptomsNeurologic symptoms often arise early in CKD, as uremic toxin buildup drives changes in cognitive and motor functions. Patients frequently experience fatigue, headache, confusion, difficulty concentrating, and, in severe cases, seizures. Peripheral neuropathy commonly manifests as burning sensations in the...
32


