IgG4-related disease in pulmonary arterial hypertension on long-term epoprostenol treatment
IgG4-related disease in pulmonary arterial hypertension on long-term epoprostenol treatment
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长期依前列醇治疗肺动脉高压中的 IgG4 相关疾病
DOI：
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发表时间：
2014
影响因子：
24.3
通讯作者：
M. Kuwana
中科院分区：
文献类型：
--
作者：
Y. Shirai;Yuichi Tamura;H. Yasuoka;T. Satoh;M. Kuwana
致编辑：
IgG 4相关疾病（IgG 4-RD）是最近描述的一种全身性纤维炎性疾病，其特征为肿胀性病变和富含IgG 4阳性浆细胞的致密淋巴浆细胞浸润[1]。IgG 4-RD已在多种器官系统中被描述，包括泪腺、唾液腺、肺、胰腺和肾，并且通常对皮质类固醇有有利的反应。IgG 4-RD的病理生理学机制尚不清楚，但认为2型T辅助细胞（Th 2）发挥关键作用[2，3]。我们最近遇到了一名在特发性肺动脉高压（PAH）过程中出现IgG 4-RD的患者。在此之后，我们仔细检查了PAH患者的IgG 4-RD，并发现了另外4例病例。
索引病例患者是一名19岁女性，2000年3月出现进行性劳力性呼吸困难，相当于世界卫生组织功能分级（WHO-FC）II级。她被转诊至庆应义塾大学医院（日本东京），在那里进行了完整的肺动脉高压（PH）评价。右心导管检查显示平均肺动脉压（mPAP）升高67 mmHg，肺动脉楔压正常7 mmHg。没有导致其PH的疾病或基础疾病，因此诊断为特发性PAH。她最初接受华法林和硝苯地平治疗，2002年1月加入口服前列环素类似物贝前列素。1年后，她的呼吸困难恶化（至WHO-FC III），mPAP显著升高（至95 mmHg）。贝前列素被依前列醇替代，剂量逐渐增加至57 ng·kg−1·min−1，导致症状和血流动力学参数改善（mPAP 47 mmHg）。
2007年1月，患者出现慢性鼻窦炎。与此同时，她注意到双侧泪腺、腮腺和下颌下腺以及嘴唇的小唾液腺逐渐增大，并伴有间歇性上腹部疼痛。
To the Editor:
IgG4-related disease (IgG4-RD) is a recently described systemic fibro-inflammatory condition characterised by tumefactive lesions and dense lymphoplasmacytic infiltrates rich in IgG4-positive plasma cells [1]. IgG4-RD has been described in a variety of organ systems, including the lacrimal gland, salivary gland, lungs, pancreas and kidneys, and often responds favourably to corticosteroids. The pathophysiological mechanisms for IgG4-RD remain unclear, but type 2 T-helper (Th2) cells are thought to play a critical role [2, 3]. We recently encountered a patient who developed IgG4-RD during the course of idiopathic pulmonary arterial hypertension (PAH). After this experience, we carefully examined PAH patients for IgG4-RD and found four additional cases.
The index case patient was a 19-year-old female who presented with progressive exertional dyspnoea corresponding to World Health Organization functional class (WHO-FC) II in March 2000. She was referred to Keio University Hospital (Tokyo, Japan), where complete pulmonary hypertension (PH) evaluations were conducted. Right heart catheterisation revealed an elevated mean pulmonary arterial pressure (mPAP) of 67 mmHg and a normal pulmonary arterial wedge pressure of 7 mmHg. There was no condition or underlying disease that accounted for her PH, leading to the diagnosis of idiopathic PAH. She was initially treated with warfarin and nifedipine, and an oral prostacyclin analogue, beraprost, was added in January 2002. 1 year later, her dyspnoea had deteriorated (to WHO-FC III), with marked elevation of the mPAP (to 95 mmHg). Beraprost was replaced by epoprostenol, and the dosage was gradually increased to 57 ng·kg−1·min−1, resulting in improved symptoms and haemodynamic parameters (mPAP 47 mmHg).
In January 2007, the patient developed chronic sinusitis. Simultaneously, she noticed a gradual enlargement of bilateral lacrimal, parotid and submandibular glands, and of minor salivary glands of the lips, in conjunction with intermittent upper abdominal pain …
影响因子：
2.9
作者：
Boswell MG;Zhou W;Newcomb DC;Peebles RS Jr
通讯作者：
Peebles RS Jr