As standard drug treatment of allergic rhinitis (AR) is not completely satisfactory, allergen immunotherapy (AIT) represents the only current treatment with the potential to modify the natural history. House dust mite (HDM) allergy is very common. The aim of the current experience was to describe the clinical profile of HDM-allergic patients with AR who received AIT in a real world model, such as allergy clinics. Globally, 239 patients (126 adults and 113 children; 107 females and 132 males; mean age 21 years, age range 6–56 years) were evaluated. AIT was prescribed in 59 patients (24.7%), 44 adults (35%) and 15 children (13.3%). The current findings deriving from this real world multicentre study are consistent with previous investigations on HDM-AIT and define some clinical characteristics of the eligible candidate to this treatment. In fact, severity of ocular-nasal symptoms and over-use of symptomatic medications may typify the ideal candidate to HDM-AIT and SLIT was the preferred choice.
Kawasaki disease (KD) is an acute vasculitis of unknown etiology that predominantly affects children < 5 years of age. It is now the leading cause of acquired heart disease in the pediatric age in developed countries1.
Objective
To compare clinical features and treatments of patients with systemic juvenile idiopathic arthritis (sIJA) and adult-onset Still’s disease (AOSD).
Methods
The clinical charts of consecutive patients with sJIA by International League of Association of Rheumatology criteria or AOSD by Yamaguchi criteria were reviewed. Patients were seen at a large paediatric rheumatology referral centre or at 10 adult rheumatology academic centres. Data collected included clinical manifestations, inflammation biomarkers, systemic score, macrophage activation syndrome (MAS), parenchymal lung disease, disease course, disability, death, and medications administered.
Results
166 patients (median age at diagnosis 5 years) with sJIA and 194 patients with AOSD (median age at diagnosis 41 years) were included. The frequency of fever, rash, arthralgia, abdominal pain, MAS, parenchymal lung disease, and increased acute phase reactants and ferritin were comparable between the two cohorts. Patients with sJIA had a higher prevalence of arthritis, whereas patients with AOSD had experienced more frequently leucocytosis and extra-articular organ involvement. Patients with AOSD were given more commonly low-dose corticosteroids, whereas biologic DMARDs were administered first-line more frequently in patients with sJIA.
Conclusion
We found remarkable disparities in the prevalence of clinical manifestations between the two illnesses, which may partly depend on their classification by different criteria.
Background: Hyperferritinemic syndromes are systemic inflammatory disorders characterized by a dysfunctional immune response, which leads to excessive activation of the monocyte-macrophage system with hypercytokinemia and may pursue a rapidly fatal course. Case presentation: We describe two patients of 11 and 9 years of age with hyperferritinemic syndromes, one with impending macrophage activation syndrome (MAS) and one with overt MAS, who were refractory or intolerant to conventional therapies, but improved dramatically with canakinumab. Conclusions: Our report indicates that canakinumab may be efficacious in the management of hyperferritinemic syndromes, including MAS.
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