Reply to Moodley and to Ravaglia <i>et al.</i>

Raghu, Ganesh; Rémy‐Jardin, Martine; Richeldi, Luca; Wilson, Kevin C.

doi:10.1164/rccm.201810-2006le

Cited by 43 publications

(90 citation statements)

References 7 publications

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“…Vasakova et al 5 also propose that the diagnosis of HP be divided into ''confident,'' ''probable,'' ''possible,'' and ''unlikely to be HP,'' depending on a combination of exposure history, imaging, presence of specific serum IgG, and lavage lymphocytosis (.20% of lymphocytes favoring HP), with biopsy intended as the final arbiter in cases falling into the probable, possible, and unlikely categories. This breakdown is conceptually similar to that proposed in the recent IPF guidelines 7 and suffers from many of the same problems. Using a biopsy to confirm or deny a diagnosis of HP should work for classic nonfibrotic (subacute) HP (see pathology description below), but it is likely to be a problem with fibrotic forms of HP, in which separation from other types of ILD, especially UIP/IPF, can be very difficult (see below).…”

Section: Nomenclature/classificationsupporting

confidence: 66%

Centrilobular Fibrosis in Fibrotic (Chronic) Hypersensitivity Pneumonitis, Usual Interstitial Pneumonia, and Connective Tissue Disease–Associated Interstitial Lung Disease

Churg

2020

Archives of Pathology &Amp; Laboratory Medicine

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Context.— Various pulmonary diseases can produce centrilobular (peribronchiolar) fibrosis, which may be isolated or associated with other patterns of more diffuse fibrosis. The major forms of interstitial lung disease in which centrilobular fibrosis is found are fibrotic (chronic) hypersensitivity pneumonitis, connective tissue disease–associated interstitial lung disease, and (a disputed issue) usual interstitial pneumonia/idiopathic interstitial fibrosis. Objective.— To review recent literature that addresses separation of these entities. Data Sources.— Data comprised recent publications. Conclusions.— In a specially constructed multidisciplinary discussion exercise, it was found that peribronchiolar metaplasia affecting more than half the bronchioles or more than 2 foci of peribronchiolar metaplasia per square centimeter of biopsy area was strongly associated with a confident diagnosis of fibrotic hypersensitivity pneumonitis. Giant cells or granulomas were only found in cases with a greater than 50% diagnostic confidence in hypersensitivity pneumonitis. Conversely, greater numbers of fibroblast foci per square centimeter and increasing measured amounts of subpleural fibrosis favored a diagnosis of usual interstitial pneumonia. Recent data also suggest that centrilobular fibrosis can be found in usual interstitial pneumonia, although the presence of centrilobular fibrosis statistically favors an alternate diagnosis. Connective tissue disease is a major confounder because many patterns are very similar to fibrotic hypersensitivity pneumonitis or usual interstitial pneumonia. Genetic abnormalities, such as the MUC5B minor allele overlap, in these conditions and at this point cannot be used for discrimination. Thus, the separation of fibrotic hypersensitivity pneumonitis and usual interstitial pneumonia remains a difficult problem. Accurate biopsy diagnosis of all of these diseases requires correlation with imaging and clinical findings, and is crucial for treatment.

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Section: Nomenclature/classificationsupporting

confidence: 66%

Centrilobular Fibrosis in Fibrotic (Chronic) Hypersensitivity Pneumonitis, Usual Interstitial Pneumonia, and Connective Tissue Disease–Associated Interstitial Lung Disease

Churg

2020

Archives of Pathology &Amp; Laboratory Medicine

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“…Eligible patients were aged ≥18 years who had been diagnosed with FILD in accordance with the previously established criteria [1,2]. Exclusion criteria were SpO 2 > 88% during the baseline CWRET, need for high concentration oxygen at rest (FiO 2 > 50%), coexistence of chronic obstructive pulmonary disease (COPD) (forced expiratory volume in 1 sec [FEV1] / forced vital capacity [FVC] < 0.70), pneumothorax, pneumomediastinum, an unstable disease, and a history of acute exacerbation within the last 1 month.…”

Section: Study Populationmentioning

confidence: 99%

“…Fibrotic interstitial lung diseases (FILD) are progressive chronic lung diseases, including idiopathic pulmonary fibrosis (IPF) and other forms of ILD [1,2]. Patients with FILD often experience exertion dyspnea and reduced exercise capacity, which leads to impairment of health status and poor prognosis [3][4][5].…”

Section: Introductionmentioning

confidence: 99%

The impact of high-flow nasal cannula oxygen therapy on exercise capacity in fibrotic interstitial lung disease: a proof-of-concept randomized controlled crossover trial

et al. 2020

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Background: Patients with fibrotic interstitial lung disease (FILD) often experience gas exchange abnormalities and ventilatory limitations, resulting in reduced exercise capacity. High-flow nasal cannula (HFNC) oxygen therapy is a novel treatment, whose physiological beneficial effects have been demonstrated in various clinical settings. We hypothesized that HFNC oxygen therapy might be superior to conventional oxygen therapy for improving exercise capacity in FILD patients. Methods: We performed a prospective randomized controlled crossover trial with a high-intensity constant work-rate endurance test (CWRET) using HFNC (50 L/min, FiO 2 0.5) and a venturi mask (VM) (15 L/min, FiO 2 0.5) for oxygen delivery in FILD patients. The primary outcome variable was endurance time. The secondary outcome variables were SpO 2 , heart rate, Borg scale (dyspnea and leg fatigue), and patient's comfort. Results: Seven hundred and eleven patients were screened and 20 eligible patients were randomized. All patients completed the trial. The majority of patients were good responders to VM and HFNC compared with the baseline test (VM 75%; HFNC 65%). There was no significant difference in endurance time between HFNC and VM (HFNC 6.8 [95% CI 4.3-9.3] min vs VM 7.6 [95% CI 5.0-10.1] min, p = 0.669). No significant differences were found in other secondary endpoints. Subgroup analysis with HFNC good responders revealed that HFNC significantly extended the endurance time compared with VM (VM 6.4 [95%CI 4.5-8.3] min vs HFNC 7.8 [95%CI 5.8-9.7] min, p = 0.046), while no similar effect was observed in the VM good responders. Conclusions: HFNC did not exceed the efficacy of VM on exercise capacity in FILD, but it may be beneficial if the settings match. Further large studies are needed to confirm these findings.

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“…Particularly, high-resolution computed tomography (HRCT) is the cornerstone of the paraclinical evaluation of diffuse parenchymal lung diseases. 57,58 Ground-glass opacities (hyperattenuated areas with preserved bronchial and vascular markings), linear opacities, reticulation, and peribronchovascular thickening are the most frequent HRCT signs present at the initial evaluation of IIM-ILD and affect preferentially the lower lobes. 41,59 Areas of consolidation (hyperattenuated areas without preservation of normal bronchial and vascular markings, often with air bronchograms) are also frequent, usually corresponding to areas of organizing pneumonia (OP) 60 (►Fig.…”

Section: Computed Tomographymentioning

confidence: 99%

“…Traction bronchiectasis (bronchial distortion due to mechanical traction by fibrotic tissue), honeycombing (presence of pleural-based, clustered, small-size cystic airspaces with fibrous-thickened walls) and subpleural bands are less frequent. 46 Radiological-pathological correlations in IIM-related ILD are largely derived from what has been learned in idiopathic disease especially idiopathic pulmonary fibrosis (IPF) 58 ; however, few series of lung biopsies and studies of radiological-pathological correlations have been specifically performed in IIM-ILD. 6,61 Several HRCT patterns are associated with IIM-related ILD.…”

Section: Computed Tomographymentioning

confidence: 99%

Lung Diseases in Inflammatory Myopathies

Barba

Mainbourg

Nasser

et al. 2019

Semin Respir Crit Care Med

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Lung involvement is the leading cause of mortality in inflammatory myopathy. A careful assessment of clinical and serologic manifestations especially myositis-associated autoantibodies allows precise classification of the different phenotypes of inflammatory myopathy and stratification of the risk of lung involvement. About three out of four patients with inflammatory myopathy develop interstitial lung disease (ILD), which represents the main cause of morbidity and mortality. In patients with a confirmed diagnosis of inflammatory myopathy, the approach to the diagnosis of ILD includes assessment of clinical and functional severity, evaluation of the high-resolution computed tomography pattern of disease, which often suggests nonspecific interstitial pneumonia or organizing pneumonia. Bronchoalveolar lavage to rule out infection is often performed; however, video-assisted thoracoscopic lung biopsy is now generally discouraged, unless malignancy is suspected. The so-called antisynthetase syndrome characterized by the combination of mechanics' hands, Raynaud' phenomenon, myositis often mild or absent, and presence of one of the anti-tRNA synthetase antibodies is associated with a 70% risk of ILD, especially in subjects with antibodies other than anti-Jo1 antibodies (i.e., anti-PL7 or -PL12 antibodies). Treatment depends on both severity and progression of ILD, often including a combination of corticosteroids and immunosuppressive therapy. Rituximab-based regimen has showed promising results in retrospective studies for the management of refractory or rapidly progressive forms of ILD. Clinical trials are ongoing to evaluate the actual efficacy of this strategy on mortality related to lung disease. Secondary pulmonary complications of inflammatory myopathy include opportunistic infections, aspiration pneumonia, pneumomediastinum, ventilatory failure due to diaphragmatic muscular weakness, drug-induced pneumonitis, and rarely pulmonary hypertension.

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Reply to Moodley and to Ravaglia et al.

Cited by 43 publications

References 7 publications

Centrilobular Fibrosis in Fibrotic (Chronic) Hypersensitivity Pneumonitis, Usual Interstitial Pneumonia, and Connective Tissue Disease–Associated Interstitial Lung Disease

Centrilobular Fibrosis in Fibrotic (Chronic) Hypersensitivity Pneumonitis, Usual Interstitial Pneumonia, and Connective Tissue Disease–Associated Interstitial Lung Disease

The impact of high-flow nasal cannula oxygen therapy on exercise capacity in fibrotic interstitial lung disease: a proof-of-concept randomized controlled crossover trial

Lung Diseases in Inflammatory Myopathies

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