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Pulmonary Manifestations of Systemic Disease: Mechanisms of Pulmonary Fibrosis |

Clinico-Radiological and Autoimmune Profile Correlation in Patients With Autoimmune Featured Interstitial Lung Disease (AIF-ILD): An Observational Study in Indian Scenario FREE TO VIEW

Rahul Sharma, DM; Deepak Talwar, DM; Mandeep Tuteja, DTCD
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Metro Center for Respiratory Disease, Metro Multispeciality Hospital, Noida, India


Copyright 2016, American College of Chest Physicians. All Rights Reserved.


Chest. 2016;149(4_S):A456. doi:10.1016/j.chest.2016.02.475
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SESSION TITLE: Mechanisms of Pulmonary Fibrosis

SESSION TYPE: Original Investigation Slide

PRESENTED ON: Sunday, April 17, 2016 at 09:45 AM - 11:15 AM

PURPOSE: Subset of interstitial lung disease (ILD) patients with clinical and/or serological features of connective tissue disease (CTD), not meeting their established diagnostic criteria, is classified as autoimmune featured ILD (AIF-ILD). Despite increasing prevalence, there is great paucity of literature about the profile of this entity. Hence this study aimed to characterize the clinical, radiological and serological attributes of patients diagnosed as AIF-ILD at an Indian tertiary care centre.

METHODS: This retrospective observational study included all patients evaluated for idiopathic interstitial pneumonia and reclassified as AIF-ILD, at ILD clinic of a tertiary care centre over last 5 years. The medical record files of enrolled patients were evaluated and details pertaining to demographic profile, clinical manifestations, laboratory results including auto antibody testing, and high resolution computerized tomography (HRCT) chest findings were recorded and analysed.

RESULTS: Twenty eight patients (86% females) with a mean age of 56.9 + 12.2 years were examined. The most common extrathoracic clinical symptom reported was joint pains (46%) and dry eyes/mouth (32%). Fourteen patients (50%) reported multiple symptoms (>3). The autoimmune antibody testing showed high ANA and RA factor positivity (40% each) followed by Anti Ro (21.2%) and Scl70 (17.8%) positivity. Twelve patients (42.8%) had two and 3 (10.7%) had three or more abnormal serologic test. HRCT images revealed a typical usual interstitial pneumonia (UIP) pattern in 35.7% (n=10) and non specific interstitial pneumonia (NSIP) pattern in 32% of study population. The lung biopsy done in three patients showed NSIP pattern predominantly (66%).

CONCLUSIONS: AIF-ILD is a subgroup of ILD which is determined on basis of systematic clinical and serologic examination with distinct radiological patterns on HRCT. The study showed that radiological and /or pathological diagnosis of otherwise idiopathic UIP or NSIP when associated with incomplete picture of CT-ILD may be compatible with AIF-ILD.

CLINICAL IMPLICATIONS: Autoimmune profile correlation must always be done before making the diagnosis of idiopathic ILD to diagnose AIF-ILD which has been shown to have better prognosis in literature.

DISCLOSURE: The following authors have nothing to disclose: Rahul Sharma, Deepak Talwar, Mandeep Tuteja

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