Anti-SAE autoantibody in dermatomyositis: original comparative study and review of the literature.

OBJECTIVE: Among specific autoantibodies in DM, the anti-small ubiquitin-like modifier activating enzyme (SAE) antibody is rare. We aim to describe the clinical characteristics, cancer prevalence, and muscle pathology of anti-SAE-positive DM. METHODS: Patients with a diagnosis of DM and sera positive for the anti-SAE antibody were recruited from 19 centres in this retrospective observational study. The available muscular biopsies were reviewed. We conducted a comparison with anti-SAE-negative DM and a review of the literature. RESULTS: Of the patients in the study (n = 49), 84% were women. Skin involvement was typical in 96% of patients, with 10% having calcinosis, 18% ulceration and 12% necrosis; 35% presented with a widespread skin rash. Muscular disease affected 84% of patients, with mild weakness [Medical Research Council (MRC) scale 4 (3, 5)], although 39% of patients had dysphagia. Muscular biopsies showed typical DM lesions. Interstitial lung disease was found in 21% of patients, mainly with organizing pneumonia pattern, and 26% of patients showed dyspnoea. Cancer-associated myositis was diagnosed in 16% of patients and was responsible for the majority of deaths, its prevalence being five times that of the general population. IVIG therapy was administered to 51% of the patients during the course of the disease. Comparison with anti-SAE-negative DM (n = 85) showed less and milder muscle weakness (P = 0.02 and P = 0.006, respectively), lower creatinine kinase levels (P < 0.0001) and less dyspnoea (P = 0.003). CONCLUSION: Anti-SAE positive DM is a rare subgroup associated with typical skin features but a potentially diffuse rash, a mild myopathy. Interstitial lung disease defines an organizing pneumonia pattern. Cancer associated DM prevalence is five times that of the general population. TRIAL REGISTRATION: ClinicalTrials.gov, http://clinicaltrials.gov, NCT04637672.

Pathology Assessments of Multiple Organs in Fatal COVID-19 in Intensive Care Unit vs. Non-intensive Care Unit Patients.

Purpose: The objective of the present study was to provide a detailed histopathological description of fatal coronavirus disease 2019 (COVID 19), and compare the lesions in Intensive Care Unit (ICU) and non-ICU patients. Methods: In this prospective study we included adult patients who died in hospital after presenting with confirmed COVID-19. Multiorgan biopsies were performed. Data generated with light microscopy, transmission electron microscopy (TEM) and RT-PCR assays were reviewed. Results: 20 patients were enrolled in the study and the main pulmonary finding was alveolar damage, which was focal in 11 patients and diffuse in 8 patients. Chronic fibrotic and inflammatory lesions were observed in 18 cases, with acute inflammatory lesions in 12 cases. Diffuse lesions, collapsed alveoli and dystrophic pneumocytes were more frequent in the ICU group (62.5%, vs. 25%; 63%, vs. 55%; 87.5%, vs. 54%). Acute lesions (82%, vs. 37.5%; p = 0.07) with neutrophilic alveolitis (63.6% vs. 0%, respectively; p = 0.01) were observed more frequently in the non-ICU group. Viral RNA was detected in 12 lung biopsies (60%) up to 56 days after disease upset. TEM detected viral particles in the lung and kidney biopsy samples up to 27 days after disease upset. Furthermore, abundant networks of double-membrane vesicles (DMVs, a hallmark of viral replication) were observed in proximal tubular epithelial cells. Conclusion: Lung injury was different in ICU and non-ICU patients. Extrapulmonary damage consisting in kidney and myocardial injury were more frequent in ICU patients. Our TEM experiments provided the first description of SARS-CoV-2-induced DMVs in kidney biopsy samples-a sign of intense viral replication in this organ.

[Gastric glomus tumors. Interest of ultrasound endoscopic biopsies for the diagnosis. About two cases]. / Les tumeurs glomiques gastriques. Intérêt des biopsies sous échoendoscopie pour le diagnostic. À propos de deux cas.

Glomus tumors are rare mesenchymal tumors, mostly benign, although a few malignant cases have been described in the literature. These tumors are usually localized subcutaneously or subcutaneously, however they may exceptionally be gastric localized. We report two new observations diagnosed on biopsies performed by echo endoscopy. The first was a 66-year-old man and the second a 78-year-old man. In both cases, the lesions were on astral, nodular, and very limited location. The biopsies revealed a tumor proliferation of trabecular architecture, composed by monomorphic cells, with eosinophilic cytoplasm, medium size, with a rounded, regular nucleus, without mitosis or necrosis, with distended and branched capillaries on the periphery. In immunohistochemical studies, tumor cells expressed smooth muscle actin and caldesmone. The proliferation index KI 67 was very low. In practice, however, preoperative diagnosis remains difficult because there is no typical appearance in imaging or echo-endoscopy to distinguish them from other gastric parietal tumors. To date, there is no consensus on the therapeutic management of gastric glomus tumors. However, endoscopic resection by dissection under the mucosa associated with endoscopic monitoring seems to be a method of choice as well as a way to postpone open or laparoscopic surgery, especially when these tumors are small (<30mm).

[Pathologist occupational hazards: Results of a survey for the French case]. / Risques professionnels chez les pathologistes : résultats d'une enquête française.

INTRODUCTION: The profession of pathologist exposes to various risks, notably infectious, physical and chemical. The objective of this study was to make an inventory of these occupational risks to which pathologists are subjected and to evaluate the pathologies that they presented. A particular attention was given to microscopic and screen work as they can induce musculoskeletal or ophthalmic disorders, and stress-related psychological disorders. METHOD: An anonymous online questionnaire containing 54 questions had been sent by mail to pathologists via the French Society of Pathology and the Syndicate of French Pathologists. RESULTS: Five hundred and twelve pathologists responded to the survey. Thirty-eight percent reported musculoskeletal disorders in the last 6 months. Visual disturbances concerned 73.4% of respondents. In the last 12 months, 33.3% of pathologists had been injured or had had mucosal projections during macroscopic or autopsy specimens. The frequency of infectious diseases was low (6.2%) as well as that of cancers (3.9%). Psychological disorders such as depression or burnout were reported by 16.7% of respondents. Pathologists seemed happy at work and had a good overall lifestyle. Few doctors had medical follow-up and few had benefited from ergonomic advice and training on the risks of chemicals. CONCLUSION: The results of this study showed the interest of a medical surveillance adapted to the pathologies presented by the pathologists. Educational and preventive measures should be introduced early in the career, focusing on ergonomics and learning about chemical and biological hazards.

Squamous Cell Carcinoma Antigen-encoding Genes SERPINB3/B4 as Potentially Useful Markers for the Stratification of HNSCC Tumours.

BACKGROUND/AIM: The squamous cell carcinoma antigen (SCCA), encoded by the genes SERPINB3/B4, is a tumour marker produced by head and neck squamous cell carcinoma (HNSCC). We aimed to examine SERPINB3/B4 mRNA levels and its clinical significance in the therapeutic context. MATERIALS AND METHODS: We retrieved mRNA expression levels, clinical, pathological and genomic data for 520 HNSCC from The Cancer Genome Atlas (TCGA). RESULTS: HNSCC tumours express high levels of SERPINB3/B4 mRNA. SERPINB3 expression differs depending on Human papillomavirus (HPV) infection status, primary tumour location, grade and differentiation, extension to lymph nodes and extracapsular spread. Interestingly, we observed an association between SERPINB3/B4 and the presence of tumour immune infiltrate as well as the expression of the immune checkpoint regulators PD-L1/PD-L2 that depended on HPV status. CONCLUSION: Our findings point to potential interest of SERPINB3/B4 for the stratification of HNSCC patients in the therapeutic context.