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Idiopathic pulmonary fibrosis: Epidemiology, natural history and pathophysiology
Published in Muhunthan Thillai, David R Moller, Keith C Meyer, Clinical Handbook of Interstitial Lung Disease, 2017
Zulma Yunt, Jeffrey J Swigris, Amy L Olson
Studies conducted prior to the development of the 2002 consensus statement on the classification of the idiopathic interstitial pneumonias (IIPs) reported that nearly 30% of subjects with IPF were alive 10 years from diagnosis (40–42). Given that idiopathic non-specific interstitial pneumonia (i-NSIP) was – for the first time – given ‘provisional diagnosis’ status in this 2002 consensus statement and NSIP has been associated with a longer survival than IPF (the estimated 10-year survival in NSIP is 73.2%), it is likely that many of the long-term survivors referred to in this document had underlying i-NSIP (43).
Test Paper 5
Published in Teck Yew Chin, Susan Cheng Shelmerdine, Akash Ganguly, Chinedum Anosike, Get Through, 2017
Teck Yew Chin, Susan Cheng Shelmerdine, Akash Ganguly, Chinedum Anosike
Nitrofurantoin is used to treat urinary tract infections. Acute pulmonary toxicity manifests radiologically with diffuse bilateral, predominantly basal heterogeneous opacities. Non-specific interstitial pneumonia (NSIP) is the most common histopathologic manifestation of chronic toxicity.
Breathlessness in Pregnancy: Respiratory Causes
Published in Tony Hollingworth, Differential Diagnosis in Obstetrics and Gynaecology: An A-Z, 2015
Usual interstitial pneumonia (UIP) and non-specific interstitial pneumonia (NSIP) (formerly known as ‘fibrosing alveolitis’) usually occur later in life, but may be associated with autoimmune diseases, which occur more frequently in young women, such as rheumatoid disease, scleroderma, and systemic lupus erythematosus, and should, therefore, be considered in the differential diagnosis of breathlessness in pregnancy. Other rarer forms of interstitial lung disease include acute interstitial pneumonia and respiratory bronchiolitis interstitial lung disease. Progressive breathlessness and cough are typical, with bilateral, fine, mid-late inspiratory crackles on auscultation. Finger clubbing may be present, but is often absent in earlier and milder disease. Chest X-ray usually shows peripheral bibasal interstitial shadowing, but HRCT scanning is necessary to define the type of disease and likely response to treatment. Lung function testing, as with the other interstitial lung diseases, reveals a reduced transfer factor (diffusion capacity).
Annual variation rate of KL-6 for predicting acute exacerbation in patients with rheumatoid arthritis-associated interstitial lung disease
Published in Modern Rheumatology, 2021
Nozomi Tanaka, Keisuke Nishimura, Daisuke Waki, Keiichiro Kadoba, Hiroyuki Murabe, Toshihiko Yokota
Several studies have reported that the UIP pattern on HRCT is a risk factor for the development of AE or mortality in CTD-ILD, including RA [1,2,18]. However, the UIP pattern was not statistically associated with AE in RA-ILD patients in our study. We considered two reasons for the discrepancy between the results of previous studies and our results. First, the definitions of UIP vary between studies [19,20]. A retrospective cohort study reported that the non-specific interstitial pneumonia (NSIP)/UIP pattern, defined according to the ATS/ERS/JRS/ALAT guidelines for IPF (2018) [21], was the major HRCT pattern in RA-ILD, and the correlation between the HRCT pattern and prognosis of RA-ILD could vary because patients with the NSIP/UIP pattern may be forcibly diagnosed with either the NSIP or UIP pattern [18]. In this study, we classified only definite UIP as the UIP pattern by referring to the study of Hozumi et al. [1], and patients with early UIP or a NSIP/UIP pattern may have been included in the non-UIP pattern group. This partially explains why the UIP pattern was not a significant prognostic factor in this study. Second, the HRCT pattern can change over time. Our study suggests that the proportion of UIP patterns increased from the time of RA-ILD diagnosis to AE onset. It is conceivable that the degree or pattern of fibrosis may differ, depending on the timing of CT examination in the long course of RA-ILD; thus, the prediction of the onset of AE using CT images at only one point in time may be difficult.
Diagnosing complications and co-morbidities of fibrotic interstitial lung disease
Published in Expert Review of Respiratory Medicine, 2019
George A. Margaritopoulos, Maria A. Kokosi, Athol U. Wells
Regarding participation in randomized, double-blind, placebo-controlled trials, it should be stressed that in the context of IIPs, these trials are mainly focused on IPF because it represents the most prevalent ILD. Other entities such as non-specific interstitial pneumonia (NSIP), due to their rarity, are unlikely to be the focus of individual trials and the treatment of PH in these patients remains problematic. However, recent evidence supports the idea that IPF and other forms of ILD could be studied together in future trials of targeted PH therapy. In severe disease (DLco <35%) there is no difference in terms of mortality between IPF and NSIP [84]. Moreover, a recent study showed no difference in invasive pulmonary hemodynamics, functional capacity, or symptoms between patients with PH and IPF or fibrotic NSIP in a placebo-controlled trial of bosentan, a dual endothelin receptor antagonist (ERA) [85].
Scleroderma-related interstitial lung disease: principles of management
Published in Expert Review of Respiratory Medicine, 2019
Aparna Das, Anupam Kumar, Andrea Valeria Arrossi, Subha Ghosh, Kristin B. Highland
Scleroderma is a disorder characterized by auto-antibody formation, vascular dysfunction and abnormal fibroblast function resulting in increased deposition of extracellular matrix. Interstitial lung disease (ILD) represents a common comorbidity in patients with scleroderma (systemic sclerosis, SSc). Evidence of interstitial changes in the lung maybe seen in up to 90% of patients and at least 50% have physiological impairment evident on pulmonary function tests [1]. Nonspecific interstitial pneumonia (NSIP) is the most common radiologic and histopathologic pattern seen, followed by usual interstitial pneumonia (UIP) [2]. ILD is more prevalent in diffuse cutaneous systemic sclerosis, but also occurs in limited SSc [3]. SSc-ILD typically occurs within the first few years of diagnosis of SSc, with the greatest decline in FVC occurring within the first four years of disease [3]. The prevalence of ILD increases with time from diagnosis and results in significant morbidity and mortality accounting for approximately 35% of SSc deaths [4]. Success with pharmacologic agents in slowing the progression of SSc-ILD remains modest. Nevertheless, several medications have demonstrated modest benefit and other treatments are under investigation.