Granulomatosis with polyangiitis mimicking infective endocarditis in an adolescent male

Granulomatosis with polyangiitis (GPA) is a rare but serious small vessel vasculitis with heterogeneous clinical presentation ranging from mainly localised disease with a chronic course, to a florid, acute small vessel vasculitic form characterised by severe pulmonary haemorrhage and/or rapidly progressive vasculitis or other severe systemic vasculitic manifestations. Cardiac involvement is, however, uncommon in the paediatric population. We report a case of a 16-year-old male who presented with peripheral gangrene and vegetation with unusual location on the supporting apparatus of the tricuspid valve, initially considered to have infective endocarditis but ultimately diagnosed with GPA. We provide an overview of the limited literature relating to cardiac involvement in GPA, and the diagnostic challenge relating to infective endocarditis in this context, especially focusing on the interpretation of the antineutrophil cytoplasmic antibody (ANCA) and the characteristic clinical features to identify in order to promptly recognise GPA, since timely diagnosis and treatment are essential for this potentially life-threatening condition

Paediatric and adolescent rheumatic diseases: measures of disease activity

Juvenile idiopathic arthritis, juvenile systemic lupus erythematosus and juvenile dermatomyositis are rare, chronic, multi-systemic rheumatic disorders that can be associated with significant morbidity, not only during childhood, but lifelong. Dedicated disease activity and damage assessment tools are essential to guide clinical management and perform multicentre clinical trials to ensure the best possible care and outcome for children with rheumatic diseases using an evidence-based, treat-to-target approach. This article summarizes the outcome measures most commonly used in paediatric rheumatology

Juvenile dermatomyositis: novel treatment approaches and outcomes

PURPOSE OF REVIEW: The aim of this article is to provide a summary of the recent therapeutic advances and the latest research on outcome measures for juvenile dermatomyositis (JDM). RECENT FINDINGS: Several new international studies have developed consensus-based guidelines on diagnosis, outcome measures and treatment of JDM to standardize and improve patient care. Myositis-specific antibodies together with muscle biopsy histopathology may help the clinician to predict disease outcome. A newly developed MRI-based scoring system has been developed to standardize the use of MRI in assessing disease activity in JDM. New data regarding the efficacy and safety of rituximab, especially for skin disease, and cyclophosphamide in JDM support the use of these medications for severe refractory cases. SUMMARY: International network studies, new biomarkers and outcome measures have led to significant progress in understanding and managing the rare inflammatory myositis conditions such as JDM