Maheshwari U, Gupta D, Aggarwal A N, Jindal S K
Department of Pulmonary Medicine, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Indian J Chest Dis Allied Sci. 2004 Jan-Mar;46(1):23-6.
To study the clinical profile of patients with idiopathic pulmonary fibrosis (IPF) and methods used for diagnosis.
Prospective analysis of symptoms, signs, radiology and lung biopsy of patients freshly diagnosed to have IPF over a 16-month period.
During the study period, 76 patients (35 men) with a mean age of 50.6 +/- 11.9 years were diagnosed to have IPE Breathlessness (98.6%) and dry cough (92.1%) were the most common presenting symptoms. Transbronchial lung biopsy (TBLB) was performed in 38 (50%) patients. Histopathological examination revealed features consistent with IPF in 35 (92.1%) patients; two of the remaining three patients underwent open lung biopsy. Other patients were diagnosed based on clinical features and high resolution chest tomography (HRCT) findings. HRCT was performed in 69 (90.8%) patients; all had features suggestive of diffuse interstitial fibrosis.
IPF is diagnosed more commonly now than in the past. Indian patients may be developing the disease a decade earlier than their counterparts in the West. TBLB and HRCT are useful in establishing diagnosis. IPF should be considered a distinct clinical entity rather than a diagnosis of exclusion.
研究特发性肺纤维化（IPF）患者的临床特征及诊断方法。
对在16个月期间新诊断为IPF的患者的症状、体征、影像学及肺活检进行前瞻性分析。
在研究期间，76例患者（35例男性）被诊断为IPF，平均年龄为50.6±11.9岁。呼吸困难（98.6%）和干咳（92.1%）是最常见的首发症状。38例（50%）患者接受了经支气管肺活检（TBLB）。组织病理学检查显示35例（92.1%）患者的特征符合IPF；其余3例患者中的2例接受了开胸肺活检。其他患者根据临床特征和高分辨率胸部断层扫描（HRCT）结果进行诊断。69例（90.8%）患者进行了HRCT检查；所有患者均有提示弥漫性间质纤维化的特征。
现在IPF的诊断比过去更为常见。印度患者患该病的时间可能比西方患者早十年。TBLB和HRCT有助于确诊。IPF应被视为一种独特的临床实体，而非排除性诊断。
