Childhood multisystem inflammatory syndrome associated with COVID-19 (MIS-C): Distinct from Kawasaki disease or part of the same spectrum?

One of the most challenging and intriguing phenomena observed during the COVID-19 pandemic has been the multisystem inflammatory syndrome in children (MIS-C). Patients with this condition present with some clinical features similar to those of Kawasaki disease (KD) and display signs and symptoms that are uncommon or rarely occur in this disorder, such as gastrointestinal complaints and myocarditis, often leading to myocardial failure and shock. In addition, patients\u2019 age is older than that of children with classic KD. Management is based on administering intravenous immunoglobulin, glucocorticoids, and anakinra in the most severe instances. It is still debated whether MIS-C and KD are different illnesses or represent a disease continuum

The role of the dermatologist in Raynaud’s phenomenon: a clinical challenge

Raynaud’s phenomenon (RP) is a functional vascular disorder involving extremities. In his practice, the dermatologist may frequently encounter RP which affects mainly women and is categorized into a primary benign form and a secondary form associated with different diseases (infections, drugs, autoimmune and vascular conditions, haematologic, rheumatologic and endocrinologic disorders). Still today, the differential diagnosis is a clinical challenge. Therefore, a careful history and a physical examination, together with laboratory tests and nailfold capillaroscopy, is mandatory. RP is generally benign, but a scheduled followâ up for primary RP patients should be established, due to risk of evolution to secondary RP. A combination of conservative measures and medications can help in the management of RP. The importance of avoiding all potential physical, chemical and emotional triggers, as well as quitting smoking, should be strongly suggested to the patient. As firstâ line treatment, dihydropyridine calcium channel blockers should be used. If this approach is not sufficient, prostacyclin derivatives, phosphodiesterases inhibitors and endothelin receptor antagonists can be considered as secondâ line treatment. In cases of acute ischaemia, nifedipine and intravenous prostanoids are helpful. In refractory cases, botulinum injections have shown a significant benefit. The approach to the RP patients requires therefore a coordinated care of specialists together with the primary care physician.Peer Reviewedhttps://deepblue.lib.umich.edu/bitstream/2027.42/144681/1/jdv14914_am.pdfhttps://deepblue.lib.umich.edu/bitstream/2027.42/144681/2/jdv14914.pd

Exuberant calcinosis and acroosteolysis. A diagnostic challenge

A case of exuberant acroosteolysis and subcutaneous tissue calcinosis in the absence of skin involvement is presented. Different hypotheses are discussed following the clinical unfolding of the case in practice

Ambrisentan response in connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH) - A subgroup analysis of the ARIES-E clinical trial.

OBJECTIVE: Pulmonary arterial hypertension (PAH) is a condition which may lead to right ventricular failure and early mortality and is an important complication in patients with connective tissue disease (CTD). Previously, the endothelin A selective receptor antagonist, ambrisentan, demonstrated efficacy and safety in treating patients with PAH due to WHO Group I etiologies. These analyses describe the 3-year efficacy and safety of ambrisentan in patients specifically with CTD associated PAH (CTD-PAH). METHODS: Patients with CTD-PAH participating in the ARIES-1 and -2 clinical trials and their long-term extension were evaluated. Efficacy evaluations including 6-min walk distance (6MWD), clinical worsening, and survival were collected at routine study visits. Additional analyses of 6MWD categorical (30 m) breakpoints were conducted to determine any relationship between 6MWD and a prognostic threshold for survival. RESULTS: 124 patients with CTD-PAH were evaluated. 62.6%, 57.3%, and 58.2% of CTD-PAH patients treated with ambrisentan exhibited increases in 6MWD at 1-, 2-, and 3- years respectively. At 3 years, 64% of patients were free from clinical worsening and 76% of patients were still alive (Kaplan-Meier estimates). Identified factors holding prognostic relevance for survival include: baseline functional class, CTD-PAH subgroup, patient sex, improvement in 6MWD ≥30 m over the first 12 weeks of treatment, the most recent 6MWD, and a 6MWD absolute threshold of 222 m. CONCLUSION: These first analyses of the 3-year treatment of CTD-PAH patients with ambrisentan revealed fewer clinical worsening events and improved survival compared to historical controls. Key exercise parameters were also identified which appear important in guiding treatment

Computed Tomography Predictors of Mortality or Disease Progression in Systemic Sclerosis–Interstitial Lung Disease: A Systematic Review

Objective: Although interstitial lung disease (ILD) is a major cause of morbidity and mortality in systemic sclerosis (SSc), its prognostication remains challenging. Given that CT represents the gold standard imaging technique in ILD assessment, a systematic review on chest CT findings as predictors of mortality or ILD progression in SSc-ILD was performed. Materials and Methods: Three databases (Medline, Embase, and Web of Science) were searched to identify all studies analyzing CT mortality or ILD progression predictors in SSc-ILD, from inception to December 2020. ILD progression was defined by worsening of forced vital capacity and/or CT ILD findings. Manuscripts not written in English, with not available full-text, not focusing on SSc-ILD or with SSc-ILD not extrapolated, otherwise with overlap syndromes, pediatric patients, <10 cases or predictors other than CT features were excluded. Results: Out of 3,513 citations, 15 full-texts (2,332 patients with SSc-ILD) met the inclusion criteria. ILD extent and extensive ILD, ILD densitometric analysis parameters, fibrotic extent and reticulation extent resulted as independent mortality predictors. Extensive ILD is also an independent predictor of death, need for supplemental oxygen or lung transplantation. Honeycombing extent is an independent risk factor for respiratory mortality. Independent predictors of ILD progression were not identified. Conclusions: ILD extent and extensive ILD independently predict mortality in SSc-ILD on CT, as well as ILD densitometric analysis, fibrotic extent and reticulation extent. Extensive ILD is also a predictor of death, need for supplemental oxygen, or lung transplantation. Honeycombing extent predicts respiratory mortality. CT predictors of ILD progression need to be further investigated. Systematic Review Registration: https://www.crd.york.ac.uk/prospero/, PROSPERO, identifier: CRD420202005001

Lifestyle and dietary habits of patients with gout followed in rheumatology settings

Diet and lifestyles modification are core aspects of the non-pharmacological management of gout, but a poor consistency with suggested guidelines is reported. This study aimed to investigate dietary and lifestyle habits of patients with gout followed in rheumatology settings. Data were retrieved from the baseline dataset of the KING study, a multicentre cohort study of patients with gout followed in rheumatology settings. Dietary habits were assessed with the Italian National Institute of Statistics (ISTAT) food-frequency questionnaire and compared with reported data about general population. The relative increase of exposure was estimated by standardized prevalence ratios adjusted for gender, age and geographical distribution. The study population included 446 patients, with a mean age of 63.9 years and a M/F ratio of 9:1. Compared to the Italian population, gouty patients showed a higher prevalence of obesity [1.82 (1.52-2.18)] and a higher consumption of wine [1.85 (1.48-2.32)] and beer [2.21 (1.68-2.90)], but a lower prevalence of smoking and a lower intake of liquor. They showed a lower intake of red meat [0.80 (0.71-0.91)], but a similar intake of other tested dietary factors. Gouty patients' lifestyle is still partially different from the recommended

Treatment patterns of anti-TNF agents in Italy: an observational study

Rheumatoid arthritis is the most common form of chronic infl ammatory rheumatism. This autoimmune disease leads to progressive joint damage, functional disability, impaired quality of life and, in some cases, shortened life expectancy