Abstract Background The aims of the present study were to determine both clinical manifestations and outcome of anti-PL7 patients with antisynthetase syndrome (ASS). Methods The medical records of 15 ...consecutive anti-PL7 patients with biopsy proven ASS were retrospectively analyzed without prior selection. Results Anti-PL7 patients exhibited polymyositis (n = 14) and dermatomyositis (n = 1); extra-pulmonary manifestations of ASS included: Raynaud's phenomenon (40%), mechanic's hands (33.3%), joint impairment (26.7%), pericardial effusion (20%) and esophageal/gastrointestinal involvement (20%). The outcome of myositis was as follows: remission/improvement (91.7%) and deterioration (8.3%). Fourteen patients (93.3%) experienced interstitial lung disease (ILD). ILD preceded ASS diagnosis (n = 5), was identified concomitantly with ASS (n = 8) and occurred after ASS diagnosis (n = 1). Patients could be divided into 3 groups according to their presenting lung manifestations: acute onset of lung disease (n = 1), progressive onset of lung signs (n = 11) and asymptomatic patients exhibiting abnormalities consistent with ILD on PFT and HRCT-scan (n = 2). No patient had resolution of ILD, whereas 64.3% and 35.7% experienced improvement and deterioration of ILD, respectively. ILD resulted in respiratory insufficiency requiring O2 therapy in 14.3% of cases. Two patients died. Predictive parameters of ILD deterioration were: DLCO < 45% at ILD diagnosis and HRCT-scan pattern of usual interstitial pneumonia (UIP). Conclusion Our series mainly underscores that ILD is frequent in anti-PL7 patients, leading to high morbidity. Our study further suggests that patients with predictive factors of ILD deterioration may require more aggressive therapy, especially the group of patients with DLCO < 45% at ILD diagnosis and UIP pattern on HRCT-scan.
Since the previous French guidelines were published in 2017, substantial additional knowledge about idiopathic pulmonary fibrosis has accumulated.
Under the auspices of the French-speaking Learned ...Society of Pulmonology and at the initiative of the coordinating reference center, practical guidelines for treatment of rare pulmonary diseases have been established. They were elaborated by groups of writers, reviewers and coordinators with the help of the OrphaLung network, as well as pulmonologists with varying practice modalities, radiologists, pathologists, a general practitioner, a head nurse, and a patients' association. The method was developed according to rules entitled "Good clinical practice" in the overall framework of the "Guidelines for clinical practice" of the official French health authority (HAS), taking into account the results of an online vote using a Likert scale.
After analysis of the literature, 54 recommendations were formulated, improved, and validated by the working groups. The recommendations covered a wide-ranging aspects of the disease and its treatment: epidemiology, diagnostic modalities, quality criteria and interpretation of chest CT, indication and modalities of lung biopsy, etiologic workup, approach to familial disease entailing indications and modalities of genetic testing, evaluation of possible functional impairments and prognosis, indications for and use of antifibrotic therapy, lung transplantation, symptom management, comorbidities and complications, treatment of chronic respiratory failure, diagnosis and management of acute exacerbations of fibrosis.
These evidence-based guidelines are aimed at guiding the diagnosis and the management in clinical practice of idiopathic pulmonary fibrosis.
Bronchoalveolar lavage (BAL) is a major diagnostic tool in lung diseases, including viral respiratory infections. We aimed to better define the situations where viral tests should be performed on BAL ...fluid (BALF). We retrospectively studied all cases where viral tests immunofluorescence, immunocytochemistry, viral culture, and/or polymerase chain reaction (PCR) were performed on BALF during a period of 1 year (2008) in our institution. We compared the characteristics of patients with virus-positive versus virus-negative BALF. Of the 636 BALF samples sent to the microbiology laboratory, 232 underwent viral tests. Of these, 70 (30 %) were positive and identified 85 viruses: herpes simplex virus (HSV)-1 (
n
= 27), cytomegalovirus (CMV,
n
= 23), Epstein–Barr virus (EBV,
n
= 18), human herpesvirus (HHV)-6 (
n
= 12), respiratory syncytial virus (RSV,
n
= 3), rhinovirus (
n
= 1), and adenovirus (
n
= 1). The variables associated with positive viral tests on univariate analysis were immunosuppression human immunodeficiency virus (HIV), corticosteroids >10 mg/day for ≥3 weeks, or other immunosuppressive therapy, ground-glass attenuations on computed tomography (CT) scanning, late-onset ventilator-associated pneumonia (VAP), and durations of (i) hospital stay, (ii) intensive care unit (ICU) stay, and (iii) mechanical ventilation before BAL (
p
< 0.01 for each comparison). On multivariate analysis, only immunosuppression odds ratio (OR) 6.4, 95 % confidence interval (CI) 2.8–14.3,
p
< 0.0001 and ground-glass attenuations (OR 3.7, 95 % CI 1.8–7.7,
p
= 0.0004) remained associated with virus-positive BAL. None of the viral tests performed on BALF for the initial assessment of diffuse infiltrative lung disease (
n
= 15) was positive. PCR improved the diagnostic yield of viral tests on BALF by 50 %. Testing for viruses on BALF should be mostly restricted to immunocompromised patients with acute respiratory diseases and/or patients with unexplained ground-glass attenuations on CT scanning.
The aim of our study was to compare the features at diagnosis in patients with ornithosis to patients with avian acute hypersensitivity pneumonitis (HP). Clinical, biological and radiological ...differences could potentially help clinicians to distinguish these diseases.
We conducted a retrospective study on patients admitted from 2000 to 2016 in three hospitals. Ornithosis was diagnosed based on a positive polymerase chain reaction for Chlamydophila psittaci on respiratory samples and/or a seroconversion while HP was diagnosed on the basis of at least one positive serum precipitin.
Twelve patients with HP and 13 patients with ornithosis were identified. Compared to HP, ornithosis occurred more frequently in males (P=0.047), with less previous respiratory diseases (P=0.01), shorter symptom duration (P=0.03), less frequently bilateral crackles (P=0.004), more severe disease requiring more frequently intensive care admission (P=0.005), higher CRP values (P=0.005) and more profound lymphopenia (P=0.02). Ground glass shadowing on CT scan (P=0.001) or bronchiectasis (P=0.03) were more frequently noted in patients with HP.
Our results suggest that patients with ornithosis and HP have important differences in their clinical, biological, and radiological presentation.
Pulmonary arterial hypertension (PAH) is a severe complication of systemic sclerosis and detecting PAH efficiently remains challenging. The DETECT study has offered in 2013 a composite screening tool ...for PAH. The objective of our study was to compare the indication of right heart catheterisation (RHC) as suggested by the DETECT algorithm with the decisions of a multidisciplinary team.
This prospective monocentric non-interventional study consecutively included systemic sclerosis patients when data required to apply DETECT algorithm were available. We evaluate the number of RHC as requested by this algorithm and confronted it with the indications of RHC suggested by a multidisciplinary group blinded for the result of DETECT algorithm.
In total, 117 systemic sclerosis patients were included. When DETECT algorithm was applied to all patients, RHC was suggested by this algorithm for 70 patients, whereas only 15 indications were required by the multidisciplinary group; among those patients only 7 had PAH. When DETECT algorithm was applied only to the 42 patients with DLCO<60% and disease duration of more than 3 years, RHC was suggested for 31 patients whereas only 13 were indicated by the multidisciplinary group; among those patients only 7 had PAH.
The DETECT algorithm is able to efficiently detect all PAH patients finally diagnosed by our multidisciplinary team. However, it increases by 3 the number of RHC that should be performed.
Hypersensitivity pneumonitis (HP) are typically subacute in their presentation and the diagnosis may be difficult.
We report a case of a hypersensitivity pneumonitis in a 27-years-old woman, caused ...by exposure to mould in an insalubrious mobile home. The initial presentation was with acute respiratory distress syndrome complicating RSV pneumonia, treated with ribavirin and corticosteroids in winter 2013-2014. The diagnosis of hypersensitivity pneumonitis was based on clinical and radiological relapse occurring during winter 2014-2015 with confirmed exposure to antigen with fungal sampling at home, respiratory deterioration with antigen rechallenge and a compatible chest CT-scan.
The diagnosis of hypersensitivity pneumonitis should be considered in similar cases. Treatment is based mainly on removing exposure to the causative antigen.
Pulmonary metastases due to a pancreatic cancer are difficult to diagnose and demonstrate a wide range of radiological patterns. We report the case of a 37-year-old female patient, without past ...medical history, with multicystic lung disease in a context of chronic abdominal pain, fatigue and weight loss. After several months of diagnostic delay, pathological examination of surgical lung biopsies led to the diagnosis of secondary deposits of pancreatic cancer. The clinical and radiogical situation deteriorated quickly with the development of alveolar consolidation and Aspergillus superinfection was then diagnosed. This case illustrates the value of an early decision to undertake surgical lung biopsy in the work-up of multicystic lung disease when cancer is suspected. In addition, in the specific context of cancer, faced with clinical and/or radiological deterioration, it is essential to look for infection, particularly aspergillosis.
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