•Unenhanced chest CT is indicated for patients with suspected COVID-19 presenting with dyspnea, polypnea or arterial blood oxygen desaturation.•Most typical CT features of COVID-19 pneumonia include ...bilateral and multifocal ground-glass opacities predominating in the peripheral, posterior and basal parts of the lungs.•Between 15 and 30% of hospitalized patients with COVID-19 progress to acute respiratory distress syndrome, which is the main cause of mortality.
The current COVID-19 pandemic has highlighted the essential role of chest computed tomography (CT) examination in patient triage in the emergency departments, allowing them to be referred to “COVID” or “non-COVID” wards. Initial chest CT examination must be performed without intravenous administration of iodinated contrast material, but contrast material administration is required when pulmonary embolism is suspected, which seems to be frequent in severe forms of the disease. Typical CT features consist of bilateral ground-glass opacities with peripheral, posterior and basal predominance. Lung disease extent on CT correlates with clinical severity. Artificial intelligence could assist radiologists for diagnosis and prognosis evaluation.
Skin manifestations among GATA2‐deficient patients Polat, A.; Dinulescu, M.; Fraitag, S. ...
British journal of dermatology (1951),
March 2018, 2018-03-00, 20180301, 2018-03, Volume:
178, Issue:
3
Journal Article
Peer reviewed
Open access
Summary
GATA2 mutations have been identified in various diseases, such as MonoMAC syndrome, Emberger syndrome, familial myelodysplastic syndrome, acute myeloid leukaemia and dendritic cell, monocyte, ...B‐cell and natural killer‐cell deficiency. These syndromes present a wide range of clinical features, dominated by severe infections and haematological disorders such as myelodysplastic syndrome. Up to 70% of patients with GATA2 mutations have dermatological features, mainly genital or extragenital warts, panniculitis or erythema nodosum and lymphoedema. We report three patients presenting with common dermatological and haematological features leading to the diagnosis of GATA2 deficiency, but also with skin manifestations that have not been previously described: gingival hypertrophy, macroglossitis and glossitis and granulomatous lupoid facial lesions. Dermatologists can encounter patients with GATA2 mutations and should recognize this disorder.
What's already known about this topic?
GATA2 deficiency is a recently described genetic disorder featuring mainly haematological manifestations and infectious conditions.
Dermatological features include warts and other infectious disorders, lymphoedema and panniculitis/erythema nodosum.
What does this study add?
We report three cases of patients with GATA2 mutations with dermatological features not previously described.
Linked Comment: Nanda. Br J Dermatol 2018; 178:593–594.
Abstract The aims of the present study were to: compare the characteristics between antisynthetase syndrome (ASS) patients with anti-Jo1 antibody and those with anti-PL7/PL12 antibody. The medical ...records of 95 consecutive patients with ASS were reviewed. Seventy-five of these patients had anti-Jo1 antibody; the other patients had anti-PL7 (n = 15) or anti-PL12 (n = 5) antibody. At ASS diagnosis, the prevalence of myalgia (p = 0.007) and muscle weakness (p = 0.02) was significantly lower in the group of anti-PL7/PL12-positive patients than in those with anti-Jo1 antibody; median value of CK (p = 0.00003) was also lower in anti-PL7/PL12 patients. Anti-Jo1 positive patients developed more rarely myositis resolution (21.3% vs. 46.2%); in addition, the overall recurrence rate of myositis was higher in anti-Jo1 positive patients than in patients with anti-PL7/PL12 antibody (65.9% vs. 19.4%). Anti-Jo1-positive patients, compared with those with anti-PL7/PL12 antibody, more often experienced: joint involvement (63.3%vs. 40%) and cancer (13.3% vs. 5%). By contrast, anti-PL7/PL12 positive patients, compared with those with anti-Jo1 antibody, more commonly exhibited: ILD (90% vs. 68%); in anti-PL7/PL12 positive patients, ILD was more often symptomatic at diagnosis, and led more rarely to resolution of lung manifestations (5.6% vs. 29.4%). Finally, the group of anti-PL7/PL12 positive patients more commonly experienced gastrointestinal manifestations related to ASS (p = 0.02). Taken together, although anti-Jo1 positive patients with ASS share some features with those with anti-PL7/PL12 antibody, they exhibit many differences regarding clinical phenotype and long-term outcome. Our study underscores that the presence of anti-Jo1 antibody results in more severe myositis, joint impairment and increased risk of cancer. On the other hand, the presence of anti-PL7/PL12 antibody is markedly associated with: early and severe ILD, and gastrointestinal complications. Thus, our study interestingly indicates that the finding for anti-Jo1 and anti-PL7/PL12 antibodies impacts both the long-term outcome and prognosis of patients with ASS.
Abstract
In France, most spirometries are performed by pneumologists. Spirometry is difficult to access due to the distance to medical office and long delays for appointments. This lack of ...accessibility contributes to the underdiagnosis of chronic obstructive pulmonary disease (COPD) among patients aged between 40 and 75 years. In recent years, general practitioners (GPs) have been performing spirometry in private practice. However, the extent of this practice is unknown. A French retrospective, repetitive transversal study analysed data from the “Système National des Données de Santé” (SNDS) database. The targeted population was GPs in primary care that performed spirometries between 2010 and 2018, in patients aged between 40 and 75 years. Between 2010 and 2018, 302,674 (7.2%) spirometries were performed in France by GPs in private practices, in patients 40 to 75 years old. 5.4% by “expert GPs” (>60 spirometries/year) and 1.8% by “non-expert GPs”. In “non-expert GPs” (2.8% of French GPs in 2018), the annual number of spirometries increased by 701 each year (
p
< 2.10
4
), the annual number of GPs performing spirometries increased by 114 each year (
p
< 2.10
−5
). Overall, 24.9% of the spirometries performed by GPs were referrals from other GPs. The number of spirometries performed by GPs and the number of GPs performing spirometries has gradually increased over time. However, this increase is inadequate considering the need to early detect and follow up respiratory disorders.
Systemic sclerosis (SSc) is an autoimmune disease associated to fibrotic manifestations. Interstitial lung disease (SSc-ILD), one of the main fibrotic features of SSc, is the first cause of ...SSc-related death. The management of SSc-ILD has recently benefited from the results of key randomised controlled trials. French authorities have approved Nintedanib for the treatment of SSc-ILD, and tocilizumab has recently been approved by the Food and Drug Administration (FDA) in the United-States (US). These recent approvals challenge the management of this fibrotic manifestation of SSc. This narrative literature review, at the crossroad of internal medicine and pulmonology, discusses what could be an up-to date approach, in terms of diagnostic and therapeutic strategies for SSc-ILD, in the light of the results from recent clinical trials.
Hypersensitivity Pneumonitis (HP) is a common immune-mediated interstitial lung disease (ILD) induced by repeated exposure to environmental antigens in susceptible individuals. The most commonly ...known forms are bird fancier's disease and farmer's lung. However, the antigens involved are widely diverse. Therefore, the list of causes of HP is frequently expanding. HP seems to be under-diagnosed owing to its highly heterogeneous presentation in both the non-fibrotic and fibrotic subtypes and could represent up to 15% of all ILDs encountered in clinical practice. However, the recognition of HP cases is essential to ensure appropriate therapy for the patient. Home health care workers' intervention is sometimes critical in this context. In case of confirmed exposure, the diagnosis could be considered with high confidence if the high-resolution computed tomography (HR-CT) shows a typical HP pattern associated with a lymphocytosis over 30% in the broncho-alveolar lavage (BAL). In all other situations, the patients should undergo further investigations and additional histopathological sampling should be considered and submitted to a multidisciplinary team discussion. After diagnosis, antigenic eviction is the rule whenever possible. Corticosteroid treatment is the first-line medical treatment for severe forms and aims to prevent the development of fibrosis. Anti-fibrotic therapy is now an option for patients with progressive ILD and failure of immunomodulatory/immunosuppressive therapies.
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