Department of Otorhinolaryngology, Head and Neck Surgery, Division for Surgery and Clinical Neuroscience, Oslo University Hospital-Rikshospitalet, Oslo, Norway.
Clin Exp Allergy. 2013 Apr;43(4):406-12. doi: 10.1111/cea.12053.
Systemic mastocytosis (SM) is a clonal proliferative disorder of mast cells (MC) that causes pathological accumulation of mast cells in various tissues, which results in clinical symptoms (e.g. diarrhoea, urticaria) due to MC mediator release. Previous studies have shown that up to fifty percent of rhinitis symptoms in SM patients are non-allergic and it has been assumed that these nasal complaints in SM patients are due an increased nasal mast cell burden. Nevertheless, to date there are no data supporting this hypothesis.
The study aims to investigate if the presence of allergy-suggesting nasal complaints in non-allergic SM patients is correlated with objective measure of nasal mast cell burden.
Eleven adult patients with systemic mastocytosis underwent a comprehensive rhinologic work-up. All patients fulfilled the clinical ARIA criteria for rhinitis. The allergologic work-up included serological allergy testing, determination of tryptase levels (serum and nasal secretion), skin prick testing, and nasal provocation testing.
Ten out of eleven SM patients with clinical persistent allergic rhinitis were found to be non-allergic. In these patients, the most predominant symptoms were rhinorrhea, sneezing, and itching. All three symptoms were strongly correlated with the nasal tryptase level but not to the level of serum tryptase.
Non-allergic persistent nasal complaints in systemic mastocytosis were significantly correlated with elevated nasal tryptase level as a measure of local MC burden. Furthermore, elevated nasal tryptase correlated with persistent rhinorrhea, sneezing, and itching as predominant symptoms, which seems to characterize a non-allergic mastocytosis-associated rhinitis (NAMAR) in systemic mastocytosis.
系统性肥大细胞增多症（SM）是一种肥大细胞（MC）克隆性增殖性疾病，导致 MC 在各种组织中的病理性堆积，从而由于 MC 介质释放而导致临床症状（例如腹泻、荨麻疹）。先前的研究表明，SM 患者的 50%的鼻炎症状是非过敏性的，并且假定 SM 患者的这些鼻部投诉是由于鼻肥大细胞负担增加所致。然而，迄今为止，尚无数据支持这一假设。
本研究旨在调查非过敏性 SM 患者存在提示过敏的鼻部投诉是否与鼻肥大细胞负担的客观测量相关。
11 名成年系统性肥大细胞增多症患者接受了全面的鼻科检查。所有患者均符合 ARIA 标准的过敏性鼻炎临床标准。过敏学检查包括血清学过敏测试、测定血清和鼻分泌物中的类胰蛋白酶水平、皮肤点刺试验和鼻激发试验。
11 名 SM 患者中有 10 名被发现患有临床持续性过敏性鼻炎，他们均为非过敏性。在这些患者中，最主要的症状是流涕、打喷嚏和瘙痒。这三种症状均与鼻类胰蛋白酶水平强烈相关，但与血清类胰蛋白酶水平无关。
系统性肥大细胞增多症中的非过敏性持续性鼻部投诉与作为局部 MC 负担的测量指标的升高的鼻类胰蛋白酶水平显著相关。此外，升高的鼻类胰蛋白酶与持续性流涕、打喷嚏和瘙痒相关，这似乎是系统性肥大细胞增多症中非过敏性肥大细胞相关性鼻炎（NAMAR）的特征。
