Best clinical practice in the treatment of juvenile systemic sclerosis: expert panel guidance - the result of the International Hamburg Consensus Meeting December 2022

Introduction: Juvenile systemic sclerosis (jSSc) is an orphan disease with a prevalence of 3 in 1,000,000 children. Currently there is only one consensus treatment guideline concerning skin, pulmonary and vascular involvement for jSSc, the jSSc SHARE (Single Hub and Access point for pediatric Rheumatology in Europe) initiative, which was based on data procured up to 2014. Therefore, an update of these guidelines, with a more recent literature and expert experience, and extension of the guidance to more aspects of the disease is needed.Areas covered: Treatment options were reviewed, and opinions were provided for most facets of jSSc including general management, some of which differs from adult systemic sclerosis, such as the use of corticosteroids, and specific organ involvement, such as skin, musculoskeletal, pulmonary, and gastroenterology.Expert opinion: We are suggesting the treat to target strategy to treat early to prevent cumulative disease damage in jSSc. Conclusions are derived from both expert opinion and available literature, which is mostly based on adult systemic sclerosis (aSSc), given shared pathophysiology, extrapolation of results from aSSc studies was judged reasonable

The clinical course of SARS-CoV-2 positive neonates

The COVID-19 pneumonia was firstly reported in Wuhan, China, in December 2019. The disease had a rapid spread all over the word becoming an international public health emergency. Limited data were available on COVID-19 positive neonates. We reviewed relevant literature to understand the clinical course of disease and transmission routes in affected neonates. The aim of the study was evaluating the clinical course and prognosis of SARS-CoV-2 positive neonates. Based on current literature, the hypothesis of vertical transmission of SARS-CoV-2, though conceivable, remains unproven. A research conducted on PubMed database from December 2019 to April 27, 2020 revealed that were reported 25 neonates affected by SARS-CoV-2. Main symptoms were fever, cough, or shortness of breath but often these neonates did not show other symptoms during length stay in hospital. No deaths occurred

Association Between T2-related Comorbidities and Effectiveness of Biologics in Severe Asthma.

RATIONALE: Previous studies investigating comorbidity impact on biologic effectiveness have been relatively small, of short duration, and have not compared biologic classes. OBJECTIVES: To determine the association between T2-related comorbidities and biologic effectiveness in adults with severe asthma (SA). METHODS: This cohort study used International Severe Asthma Registry data (n=21 countries, 2017-2022) to quantify pre- to post-biologic change for four outcomes (annual asthma exacerbation rate, % predicted FEV1 (ppFEV1), asthma control, and long-term oral corticosteroid daily dose [LTOCS]) in patients with/without allergic rhinitis (AR), chronic rhinosinusitis +/- nasal polyps (CRS+/-NP), NP, or eczema/atopic dermatitis (AD). MAIN RESULTS: Of 1765 patients, 1257, 421, and 87 initiated anti-IL-5/5R, anti-IgE, and anti-IL-4/13 therapies, respectively. In general, pre- to post-biologic improvements were noted in all four asthma outcomes assessed, irrespective of comorbidity status. However, patients with comorbid CRS+/-NP experienced 23% (95% CI 10-35%, p<0.001) fewer exacerbations/year and had 59% (95% CI: 26-102%, p<0.001) higher odds of better post-biologic control than those without CRS+/-NP. Similar estimates were noted for those with comorbid NP (22% less exacerbations and 56% higher odds of better post-biologic control). Patients with SA and CRS+/-NP had an additional ppFEV1 improvement of 3.2% (95% CI: 1.0-5.3; p=0.004), a trend that was also noted in those with comorbid NP. The presence of AR or AD were not associated with pre- to post-biologic effect for any outcome assessed. CONCLUSIONS: These findings highlight the importance of systematic comorbidity evaluation. The presence of CRS+/-NP or NP may be considered a predictor of biologic effectiveness in patients with severe asthma