Real-life prevalence of progressive fibrosing interstitial lung diseases.

The concept of progressive fibrosing interstitial lung disease (PF-ILD) has recently emerged. However, real-life proportion of PF-ILDs outside IPF is still hard to evaluate. Therefore, we sought to estimate the proportion of PF-ILD in our ILD cohort. We also determined the proportion of ILD subtypes within PF-ILD and investigated factors associated with PF-ILDs. Finally, we quantified interobserver agreement between radiologists for the assessment of fibrosis. We reviewed the files of ILD patients discussed in multidisciplinary discussion between January 1st 2017 and December 31st 2019. Clinical data, pulmonary function tests (PFTs) and high-resolution computed tomography (HRCTs) were centrally reviewed. Fibrosis was defined as the presence of traction bronchiectasis, reticulations with/out honeycombing. Progression was defined as a relative forced vital capacity (FVC) decline of ≥ 10% in ≤ 24 months or 5% < FVC decline < 10% and progression of fibrosis on HRCT in ≤ 24 months. 464 consecutive ILD patients were included. 105 had a diagnosis of IPF (23%). Most frequent non-IPF ILD were connective tissue disease (CTD)-associated ILD (22%), hypersensitivity pneumonitis (13%), unclassifiable ILD (10%) and sarcoidosis (8%). Features of fibrosis were common (82% of CTD-ILD, 81% of HP, 95% of uILD). After review of HRCTs and PFTs, 68 patients (19% of non-IPF ILD) had a PF-ILD according to our criteria. Interobserver agreement for fibrosis between radiologists was excellent (Cohen's kappa 0.86). The main diagnosis among PF-ILD were CTD-ILD (36%), HP (22%) and uILD (20%). PF-ILD patients were significantly older than non-F-ILD (P = 0.0005). PF-ILDs represent about 20% of ILDs outside IPF. This provides an estimation of the proportion of patients who might benefit from antifibrotics. Interobserver agreement between radiologists for the diagnosis of fibrotic ILD is excellent.

Does naming accuracy improve through self-monitoring of errors?

This study examined spontaneous self-monitoring of picture naming in people with aphasia. Of primary interest was whether spontaneous detection or repair of an error constitutes an error signal or other feedback that tunes the production system to the desired outcome. In other words, do acts of monitoring cause adaptive change in the language system? A second possibility, not incompatible with the first, is that monitoring is indicative of an item's representational strength, and strength is a causal factor in language change. Twelve PWA performed a 615-item naming test twice, in separate sessions, without extrinsic feedback. At each timepoint, we scored the first complete response for accuracy and error type and the remainder of the trial for verbalizations consistent with detection (e.g., "no, not that") and successful repair (i.e., correction). Data analysis centered on: (a) how often an item that was misnamed at one timepoint changed to correct at the other timepoint, as a function of monitoring; and (b) how monitoring impacted change scores in the Forward (Time 1 to Time 2) compared to Backward (Time 2 to Time 1) direction. The Strength hypothesis predicts significant effects of monitoring in both directions. The Learning hypothesis predicts greater effects in the Forward direction. These predictions were evaluated for three types of errors--Semantic errors, Phonological errors, and Fragments--using mixed-effects regression modeling with crossed random effects. Support for the Strength hypothesis was found for all three error types. Support for the Learning hypothesis was found for Semantic errors. All effects were due to error repair, not error detection. We discuss the theoretical and clinical implications of these novel findings.

Subdivision of frontal cortex mechanisms for language production in aphasia.

Ventrolateral prefrontal cortex (VLPFC) has long been linked to language production, but the precise mechanisms are still being elucidated. Using neuropsychological case studies, we explored possible sub-specialization within this region for different linguistic and executive functions. Frontal patients with different lesion profiles completed two sequencing tasks, which were hypothesized to engage partially overlapping components. The multi-word priming task tested the sequencing of co-activated representations and the overriding of primed word orders. The sequence reproduction task tested the sequencing of co-activated representations, but did not employ a priming manipulation. We compared patients' performance on the two tasks to that of healthy, age-matched controls. Results are partially consistent with an anterior-posterior gradient of cognitive control within lateral prefrontal cortex (Koechlin & Summerfield, 2007). However, we also found a stimulus-specific pattern, which suggests that sub-specialization might be contingent on type of representation as well as type of control signal. Isolating such components functionally and anatomically might lead to a better understanding of language production deficits in aphasia.