Mathew Uthara, Mittal Ankit, Vyas Surabhi, Ray Animesh
Medicine, All India Institute of Medical Sciences, New Delhi, India.
Radiology, All India Institute of Medical Sciences, New Delhi, India.
BMJ Case Rep. 2019 Sep 6;12(9):e230948. doi: 10.1136/bcr-2019-230948.
Interstitial pneumonia with autoimmune features (IPAF) is a recently proposed terminology for interstitial lung disease (ILD) with evidence of autoimmunity that does not meet the criteria for a defined connective tissue disease (CTD). Although ILD is well recognised in patients with established CTD, it is rarely the sole presenting feature of CTD. We report a case of 22-year-old male patient, who presented with progressive shortness of breath for 2 months and had features suggestive of platypnea-orthodeoxia syndrome (POS). Imaging revealed ILD with usual interstitial pneumonia pattern. Patient had features of autoimmune disorder but did not fulfil the criteria for any CTD and hence was labelled as IPAF. His POS was attributed predominantly to the lower lobe disease. The patient responded well to immunosuppressive treatment. A systematic review of literature of all cases with POS due to pulmonary parenchymal involvement has also been done.
具有自身免疫特征的间质性肺炎（IPAF）是最近提出的一个术语，用于描述具有自身免疫证据但不符合明确结缔组织病（CTD）标准的间质性肺疾病（ILD）。虽然ILD在已确诊的CTD患者中已得到充分认识，但它很少是CTD的唯一首发特征。我们报告一例22岁男性患者，该患者出现进行性气短2个月，并具有提示平卧呼吸-直立性低氧血症综合征（POS）的特征。影像学显示为具有普通型间质性肺炎模式的ILD。患者具有自身免疫性疾病的特征，但不符合任何CTD的标准，因此被诊断为IPAF。他的POS主要归因于下叶疾病。该患者对免疫抑制治疗反应良好。我们还对所有因肺实质受累导致POS的病例进行了文献系统综述。
