Abstract

Background Lung involvement especially interstitial lung disease (ILD), can be the first manifestation of an underlying connective tissue disorder (CTD). About 25% of ILD occurs in the context of an “undifferentiated” CTDs, characterized by signs and symptoms that are not specific for any of the described CTD entities, now known as IPAF. Nailfold capillaroscopy (NFC) is an important tool, which helps us in early recognition of microvascular changes in patients with ILD Objectives To study the various patterns on Nailfold Capillaroscopy in patients of Interstitial Pneumonia with autoimmune features (IPAF) and compare them with those having Idiopathic Interstitial Pneumonia (IIP). Methods The study population consisted of 50 patients each of IIP and IPAF who fulfilled ERS/JRS/ALAT 2011 revised diagnostic criteria for IIP and ERS/ATS classification criteria for Interstitial pneumonia with autoimmune features respectively. The study also included 50 age and sex matched controls, having normal respiratory examination clinically, normal CXR and normal PFTs. All patients underwent NFC at room temperature and the following parameters were recorded: capillary density, presence of megacapillaries, tortousity, avascular areas, disarrangement and neo-angiogenesis. Results Our study consisted of 23 (46%) female patients and 27 (54%) male patients for IPAF group and 19 (38%) female patients and 31 (62%) male patients for IIP group. The mean capillary density was significantly reduced in IPAF group and also had presence of abnormal capillary morphologic patterns (microhemorrhages, neoangiogenesis and megacapillaries). Conclusion This single centre study found that Nailfold Capillaroscopy (NFC) is an important adjunct to differentiate between patients of IPAF and IIP demonstrating a higher frequencies of abnormalities (microhemorrhages, megacapillaries and reduced capillary density) among patients with IPAF.

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