Assessing Competencies, Needs, and Satisfaction With the Transition From Pediatric to Adult Health Care in Rheumatology: Development and Validation of the Transition-KompAZ.

Objective: The aim of this study was to develop and psychometrically validate a self-report instrument to assess (1) competencies, (2) needs, and (3) satisfaction among youth transitioning from pediatric to adult rheumatology.

Methods: The Transition-KompAZ was developed in several steps with conceptual and psychometric analyses. To test its psychometric properties, the instrument was administered to adolescents and young adults (AYAs; 16-25 years) with inflammatory rheumatic diseases before (group 1) or after (group 2) transfer to adult rheumatology.

Disease flares with baricitinib dose reductions and development of flare criteria in patients with CANDLE/PRAAS.

Objectives: Patients with chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature/proteasome-associated autoinflammatory syndrome (CANDLE/PRAAS) respond to the janus kinase inhibitor 1/2 inhibition with baricitinib at exposures higher than in rheumatoid arthritis. Baricitinib dose reductions to minimise exposure triggered disease flares which we used to develop 'flare criteria'.

Methods: Of 10 patients with CANDLE/PRAAS treated with baricitinib in an open-label expanded-access programme, baricitinib doses were reduced 14 times in 9 patients between April 2014 and December 2019.

[Rheumatic diseases - Transition from pediatric to adult-oriented care].

Adolescence and young adulthood represent a vulnerable phase of life, especially for young people with a chronic rheumatic disease. On the one hand, the chronic disease can impair the biopsychosocial development of young people. On the other hand, risk behaviour common in adolescence and young adulthood can negatively influence the course and outcome of the rheumatic disease.

JAK1/2 inhibition with baricitinib in the treatment of autoinflammatory interferonopathies.

Background: Monogenic IFN-mediated autoinflammatory diseases present in infancy with systemic inflammation, an IFN response gene signature, inflammatory organ damage, and high mortality. We used the JAK inhibitor baricitinib, with IFN-blocking activity in vitro, to ameliorate disease.

Methods: Between October 2011 and February 2017, 10 patients with CANDLE (chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperatures), 4 patients with SAVI (stimulator of IFN genes-associated [STING-associated] vasculopathy with onset in infancy), and 4 patients with other interferonopathies were enrolled in an expanded access program.

The multifaceted presentation of chronic recurrent multifocal osteomyelitis: a series of 486 cases from the Eurofever international registry.

Objectives: Chronic non-bacterial osteomyelitis (CNO) or chronic recurrent multifocal osteomyelitis (CRMO) is an autoinflammatory disorder characterized by sterile bone osteolytic lesions. The aim of this study was to evaluate the demographic data and clinical, instrumental and therapeutic features at baseline in a large series of CNO/CRMO patients enrolled in the Eurofever registry.

Methods: A web-based registry collected retrospective data on patients affected by CRMO/CNO.

Building a transitional care checklist in rheumatology: A Delphi-like survey.

Objectives: To design a transitional care checklist to be used by and facilitate the work of health professionals in providing transitional care for children with a chronic rheumatologic disease and their families.

Methods: A Delphi-like study among an international expert panel was carried out in four steps: (1) a working group of 6 specialists established a draft; (2) a web-survey among a panel of international experts evaluated it; (3) a 2-day consensus conference with an expert panel discussed items not reaching agreement; (4) a web-survey among the panel of international experts with the list of reformulated items.

Results: The first draft of the checklist included 38 items in 3 phases of transition and 5 age groups.

EULAR/PReS standards and recommendations for the transitional care of young people with juvenile-onset rheumatic diseases.

To develop standards and recommendations for transitional care for young people (YP) with juvenile-onset rheumatic and musculoskeletal diseases (jRMD). The consensus process involved the following: (1) establishing an international expert panel to include patients and representatives from multidisciplinary teams in adult and paediatric rheumatology; (2) a systematic review of published models of transitional care in jRMDs, potential standards and recommendations, strategies for implementation and tools to evaluate services and outcomes; (3) setting the framework, developing the process map and generating a first draft of standards and recommendations; (4) further iteration of recommendations; (5) establishing consensus recommendations with Delphi methodology and (6) establishing standards and quality indicators. The final consensus derived 12 specific recommendations for YP with jRMD focused on transitional care.