RESUMO
Introduction Interstitial pneumonia with autoimmune features (IPAF) refers to interstitial lung disease (ILD) with co-existing features of other clinical, serologic, or pulmonary features that suggest the presence of an underlying systemic autoimmune condition that does not fulfill the current rheumatologic criteria for connective tissue disorder (CTD). It is a relatively newly described clinical syndrome with only a handful of reports describing it. This study aimed at studying the clinical, radiological, and laboratory profiles of IPAF patients from a tertiary care hospital in South India. Methods This was an observational cross-sectional study conducted in a tertiary care hospital in South India over a period of one year. Patients diagnosed to have IPAF as per the European Respiratory Society (ERS)/American Thoracic Society (ATS) criteria were included in the study, and their demographics, clinical features, radiological features, and laboratory markers were collected along with a descriptive analysis. Results A total of 14,433 patients were screened during the study period. Twenty-four patients were diagnosed to have IPAF during the study period with a prevalence of 0.17%. Out of these 24 patients, 11 (45.8%) patients were males. The mean (M) ± standard deviation (SD) age was 47.8±10.7 years. Twenty-one (87.5%) of the patients reported having a cough, 18 (75%) patients had breathlessness, and 10 (41.7%) patients had digital clubbing. On radiological imaging, five (20.8%) patients had features of usual interstitial pneumonia (UIP) and 14 (58.3%) had nonspecific interstitial pneumonia (NSIP). On pulmonary function testing, the M±SD forced expiratory volume in the first second (FEV1) was 56.4±13.9%. The M±SD forced vital capacity (FVC) was 44.2±24.1%. The M±SD FEV1/FVC ratio was 0.8±0.04. On performing diffusing capacity of the lungs for carbon monoxide (DLCO), the M±SD was 34.2±21.9%. Of the patients, 95.8% had a positive antinuclear antibody (ANA) while 25% of patients had a positive anti-AMA-M2. Conclusions The prevalence of IPAF in the studied population was very low. IPAF had nonspecific clinical features, pulmonary function tests, and radiological findings. Further large-scale studies are required from different parts of the world in order to understand the epidemiology of IPAF. Research is also required into developing effective management options for IPAF.
RESUMO
Introduction Bronchiectasis is a chronic respiratory disease that can affect patients of all ages and significantly impact the quality of life (QOL) in patients who suffer from it. In spite of its widespread prevalence, and the significant impact on QOL, data on the quantitative impact of bronchiectasis on QOL is lacking. The Quality of Life-Bronchiectasis (QOL-B) is a self-administered patient-reported outcome measure, that was recently developed as a response to the emergent need for such measurement tools to study the impact of bronchiectasis on QOL. Methods We conducted a single-center cross-sectional study to study the correlation between QOL and various other outcome parameters such as exercise capacity, lung functions, co-morbidities, inflammatory markers, and body mass index (BMI). The secondary outcome was to find out various determinants of quality of life in non-cystic fibrosis bronchiectasis (NCFB). Results Forty-four patients who determined the pre-determined criteria for NCFB were enrolled in this study. This study demonstrated a significant impact on the QOL of NCFB patients based on the QOL-B scoring system. Almost all domains of QOL-B were found to be adversely impacted as measured by one or more of the outcome parameters but the FEV1, age, colonization, extension, dyspnea (FACED) score, bronchiectasis severity index (BSI) score, six-minute walk test (6MWD), and FEV1 showed associations across most scales while the other outcome parameters showed varying associations. Conclusions The QOL is significantly reduced in NCFB and it may be quantified using the QOL-B questionnaire. The impact on QOL in NCFB may be assessed using validated tools such as the FACED and BSI scoring systems, as well as other well-established outcome parameters like 6MWD and FEV1 predicted.
