Ren Xiaoxia, Yang Ting, Li Jianmei, Zhang Jing, Geng Jing, Dai Huaping
Department of Pulmonary and Critical Care Medicine, Center of Respiratory Medicine, China-Japan Friendship Hospital, Beijing 100029, P.R. China.
National Clinical Research Center for Respiratory Diseases, Beijing 100029, P.R. China.
Exp Ther Med. 2020 Sep;20(3):2291-2297. doi: 10.3892/etm.2020.8938. Epub 2020 Jun 25.
Idiopathic pulmonary hemosiderosis (IPH) is a rare interstitial lung disease, usually occurring in children or young adults. Although several studies reported on the coexistence of IPH and immune system diseases, the association between these conditions has not been well described. The present study reports on the case of a 21-year-old female patient who presented with bilateral lung abnormalities. The patient was admitted due to a 2-year history of progressive exertional dyspnea, as well as arthralgia and joint swelling in the recent 2 months. During the past 15 years, the patient had been diagnosed with anemia and received repeated blood transfusions. Serial chest CT scans indicated an interstitial pattern. On physical examination, the patient had pale skin with a hemoglobin level of 65 g/l and exhibited finger-clubbing. Arterial blood gas analysis revealed hypoxia. Anticyclic-citrullinated protein antibody and rheumatoid factor were highly positive. Pulmonary function tests revealed restrictive ventilation dysfunction and decreased diffusion capacity. Bronchoscopy and biopsy confirmed diffuse alveolar hemorrhage. Following assessment of the etiology, the diagnosis of IPH was made by exclusion. The patient's symptoms and laboratory findings combined also confirmed the diagnosis of rheumatoid arthritis (RA). After receiving corticosteroid treatment, the patient's condition improved, and she was discharged and followed up. Based on this patient and a review of the literature, the present study demonstrated for the first time that IPH may mediate the development of an RA pathology. Therefore, early diagnosis is important for the timely management of IPH, which may also delay or even prevent the development of immune system diseases, e.g. RA, in patients with IPH. Further attention should be paid to determine the association between IPH and immune system diseases in the clinical setting.
特发性肺含铁血黄素沉着症(IPH)是一种罕见的间质性肺病,通常发生于儿童或青年。尽管有多项研究报道了IPH与免疫系统疾病并存的情况,但这些病症之间的关联尚未得到充分描述。本研究报告了一名21岁女性患者的病例,该患者出现双侧肺部异常。患者因进行性劳力性呼吸困难2年以及近2个月的关节痛和关节肿胀入院。在过去15年中,该患者被诊断为贫血并接受了多次输血。胸部CT连续扫描显示为间质性模式。体格检查发现患者皮肤苍白,血红蛋白水平为65g/l,并出现杵状指。动脉血气分析显示缺氧。抗环瓜氨酸肽抗体和类风湿因子呈高度阳性。肺功能测试显示限制性通气功能障碍和弥散能力下降。支气管镜检查和活检证实为弥漫性肺泡出血。在对病因进行评估后,通过排除法做出了IPH的诊断。患者的症状和实验室检查结果也共同证实了类风湿关节炎(RA)的诊断。接受皮质类固醇治疗后,患者病情好转,出院并进行随访。基于该患者及文献回顾,本研究首次证明IPH可能介导RA病理的发展。因此,早期诊断对于IPH的及时治疗很重要,这也可能延迟甚至预防IPH患者免疫系统疾病(如RA)的发展。在临床环境中应进一步关注确定IPH与免疫系统疾病之间的关联。