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Made available in DSpace on 2022-04-29T08:30:47Z (GMT). No. of bitstreams: 0 Previous issue date: 2021-07-01 Objective: Sjögren syndrome in children is a poorly understood autoimmune disease. We aimed to describe the clinical and diagnostic features of children diagnosed with Sjögren syndrome and explore how the 2016 ACR/EULAR classification criteria apply to this population. Methods: An international workgroup retrospectively collected cases of Sjögren syndrome diagnosed under 18 years of age from 23 centres across eight nations. We analysed patterns of symptoms, diagnostic workup, and applied the 2016 ACR/EULAR classification criteria. Results: We identified 300 children with Sjögren syndrome. The majority of patients n = 232 (77%) did not meet 2016 ACR/EULAR classification criteria, but n = 110 (37%) did not have sufficient testing done to even possibly achieve the score necessary to meet criteria. Even among those children with all criteria items tested, only 36% met criteria. The most common non-sicca symptoms were arthralgia [n = 161 (54%)] and parotitis [n = 140 (47%)] with parotitis inversely correlating with age. Conclusion: Sjögren syndrome in children can present at any age. Recurrent or persistent parotitis and arthralgias are common symptoms that should prompt clinicians to consider the possibility of Sjögren syndrome. The majority of children diagnosed with Sjögren syndromes did not meet 2016 ACR/EULAR classification criteria. Comprehensive diagnostic testing from the 2016 ACR/EULAR criteria are not universally performed. This may lead to under-recognition and emphasizes a need for further research including creation of paediatric-specific classification criteria. Division of Pediatric Rheumatology Department of Pediatric and Adolescent Medicine Mayo Clinic Division of Rheumatology Department of Pediatrics University of Utah School of Medicine Division of Rheumatology The Children's Hospital of Philadelphia Department of Internal Medicine and Pediatrics The University of Chicago Medical Center Department of Pediatrics Duke University School of Medicine Department of Pediatrics Hematology and Oncology Medical University of Gdansk Children's Mercy Hospital University of Missouri-Kansas City Division of Rheumatology Irccs Ospedale Pediatrico Bambino Gesù Department of Pediatric Rheumatology Institute of Rheumatology Department of Pediatrics Columbia University Medical Center Division of Rheumatology Children's National Hospital George Washington University School of Medicine and Health Sciences National Institutes of Health National Institute of Arthritis and Musculoskeletal and Skin Diseases Department of Pediatrics University of Texas McGovern Medical School at Houston Division of Pediatric Rheumatology Children's Healthcare of Atlanta Emory University School of Medicine Instituto de Puericultura e Pediatria Martagão Gesteira Universidade Federal Do Rio de Janeiro Pediatric Rheumatology Hospital Sant Joan de Déu Universitat de Barcelona Barcelona Rheumatology Unit Department of Medicine School of Medical Science University of Campinas Division of Rheumatology Department of Pediatrics University of Indiana School of Medicine Riley Hospital for Children at Indiana University Health Pediatric Rheumatology Unit São Paulo State University (UNESP), Botucatu Rheumatology Service Royal Children's Hospital Melbourne and Murdoch Children's Research Institute Division of Pediatric Allergy Immunology and Rheumatology Department of Pediatrics College of Medicine University of Florida Division of Oral Medicine Department of Oral and Maxillofacial Diagnostic Sciences College of Dentistry University of Florida Rheumatology Department Hospital General Universitario Gregorio Marañón Division of Immunology Boston Children's Hospital Department of Pediatrics Harvard Medical School Department of Rheumatic Diseases Tokyo Metropolitan Tama Medical Center Division of Rheumatology Allergy and Immunology Stead Family Department of Pediatrics Carver College of Medicine University of Iowa Pediatric Rheumatology Unit São Paulo State University (UNESP), Botucatu

Multisystem inflammatory syndrome associated with the SARS-CoV-2 pandemic has recently been described in children (MIS-C), partially overlapping with Kawasaki disease (KD). We hypothesized that (a) MIS-C and prepandemic KD cytokine profiles may be unique and justify the clinical differences observed, and (b) SARS-CoV-2-specific immune complexes (ICs) may explain the immunopathology of MIS-C. Seventy-four children were included: 14 with MIS-C, 9 patients positive for SARS-CoV-2 by PCR without MIS-C (COVID), 14 with prepandemic KD, and 37 healthy controls (HCs). Thirty-four circulating cytokines were quantified in pretreatment serum or plasma samples and the presence of circulating SARS-CoV-2 ICs was evaluated in MIS-C patients. Compared with HCs, the MIS-C and KD groups showed most cytokines to be significantly elevated, with IFN-gamma-induced response markers (including IFN-gamma, IL-18, and IP-10) and inflammatory monocyte activation markers (including MCP-1, IL-1 alpha, and IL-1RA) being the main triggers of inflammation. In linear discriminant analysis, MIS-C and KD profiles overlapped; however, a subgroup of MIS-C patients (MIS-C-plus) differentiated from the remaining MIS-C patients in IFN-gamma, IL-18, GM-CSF, RANTES, IP-10, IL-1 alpha, and SDF-1 and incipient signs of macrophage activation syndrome. Circulating SARS-CoV-2 ICs were not detected in MIS-C patients. Our findings suggest a major role for IFN-gamma in the pathogenesis of MIS-C, which may be relevant for therapeutic management.

To establish the prevalence of incidental prostate cancer after transrectal ultrasound guided prostatic biopsy and subsequent suprapubic prostatic adenomectomy and to compare it with a similar group of patients who did not underwent biopsy before surgery. To evaluate treatment, outcomes, and disease progression in patients with incidental prostate cancer.Retrospective study of 549 suprapubic adenomectomy performed between 1996-2001 (6 yr.), comparing the group of patients with biopsies before surgery vs. the group of patients without biopsies.291 (53%) patients did not undergo biopsy before adenomectomy. 258 (47%) underwent biopsies. 25 incidental prostate cancers were detected, 19 (76%) in the group of no biopsy and 6 (24%) in the biopsy group. 88% pT1a and 12%pT1b. Mean Gleason score 4.5 (3-7). 84% of the patients did not receive treatment (21) ("wait and see"); 8% (2) androgen blockade; 8% (2) finasteride (2). Three patients (12%) in the group of no biopsy had disease progression. Mean follow-up was 48.1 months (22-96). No case of cancer-specific mortality was detected.Global prevalence of incidental prostate cancer in our series of patients undergoing suprapubic prostatic adenomectomy was 4.55%. Prevalence was higher in the group of patients without previous biopsy (3.46%) than in the biopsy group (1.09%). Tumor progression was 12% and cancer specific survival 100% after a mean follow-up of 48.1 months (22-92). Previous prostatic biopsy in patients with suspicions digital rectal examination or elevated PSA diminishes the prevalence of incidental prostate cancer. Watchful waiting may be a valid option in some cases.