Drug provocation testing with ASA confirms the diagnosis of multiple NSAID-induced urticaria/angioedema in up to 92% of cases with an unequivocal clinical history, when reactions occur within 1 h and more than two different NSAIDs are involved.

Atopic dermatitis (AD) has a high incidence in heart-transplant children, and the reason why there is more AD after transplantation is still unknown. We conducted a cross-sectional study comparing 11 AD and 11 non-AD age-matched heart-transplant children, to assess which immune alterations are related to AD in these patients. AD patients had been transplanted at a younger age compared to non-AD, indicating that age at transplant may be determinant in the onset of AD. The earlier thymectomy in AD heart-transplant children favored the presence of more differentiated phenotypes in the T cell compartment. We observed a clear reduction in the T-helper 1/T-helper 2 (Th1/Th2) ratio in AD children. This Th2 polarization was related to eosinophilia and high immunoglobulin E levels, but also to an impaired regulatory T cell (Treg) suppression, which could be secondary to an exhaustion of the Treg compartment. Interestingly, AD patients were free of rejection episodes (0/11) in comparison to non-AD children (4/11). We propose that a predominant Th2 phenotype may prevent the emergence of Th1 responses associated with graft rejection. A more differentiated Treg phenotype could also play a role in preventing acute rejection in the first year posttransplant. Our findings provide useful insights and knowledge for the better understanding of atopic disorders in transplanted children. K E Y W O R D Sallergy, clinical research/practice, comorbidities, heart transplantation/cardiology, immune regulation, immunobiology, pediatrics, T cell biology, thymus/thymic biology, translational research/science

The aim of the European Journal of Rheumatology is to cover various aspects of rheumatology for its readers, encompassing the spectrum of diseases with arthritis, musculoskeletal conditions, autoinflammatory diseases, connective tissue disorders, osteoporosis, translational research, the latest therapies and treatment programs. European Journal of Rheumatology publishes original articles, invited reviews, case based reviews, letters to the editor and images in rheumatology. The publication language of the journal is English. Accepted manuscripts are copy-edited for grammar, punctuation, and format. Once the publication process of a manuscript is completed, it is published online on the journal's webpage as an aheadof-print publication before it is included in its scheduled issue. A PDF proof of the accepted manuscript is sent to the corresponding author and their publication approval is requested within 2 days of their receipt of the proof.

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Only in the last calendar period (HAART) was a significant decrease observed in the prevalence of mucocutaneous manifestations with HIV infection associated with an increase in CD4+ T cells. In addition, we found a strong association between children who had mucocutaneous manifestations with an infectious aetiology and a more cytopathic (X4/SI) viral phenotype.

Food allergy is a pathological immune reaction that identifies certain harmless food proteins, usually tolerated by the majority of the people, as a threat. The prevalence of these food allergies is increasing worldwide and currently affects 8% of children. Exacerbated reactions to milk, egg and peanut are the most frequent in the pediatric population. It is well known that allergic diseases are a type 2 T-helper (Th2) immune response, characterized by the elevated production of IgE antibodies. However, little is known about the immune mechanisms responsible for the development of clinical tolerance toward food allergens. Recent studies have suggested the key role of regulatory T cells (Tregs) in controlling allergic inflammation. In this review, we discuss the importance of Tregs in the pathogenesis of food allergy and the acquisition of oral tolerance in children. Further investigation in this area will be crucial for the identification of predictive markers and the development of new therapies, which will represent a clinical and social benefit for these allergic diseases.

The diagnosis of mast cell activation syndromes (MCAS) is defined by 3 criteria: 1) typical clinical signs and symptoms of acute, recurrent (episodic) and systemic mast cell activation (MCA), 2) increase in tryptase level to plus 20 % + 2 ng/ml within a 1–4 hours after onset of the acute crisis, 3) response of MCA symptoms to antimediator therapy. Classification of MCAS requires applying highly sensitive and specific methodological approaches for assessing clonal bone marrow mast cells (BM MCs) at low frequencies. The Spanish Network on Mastocytosis (REMA) score is successfully used as predictive model for selecting MCAS candidates for BM studies based on a high probability of having an underlying clonal mast cell disorder (c-MCD). In this article, we propose a diagnostic algorithm and focus in the practical evaluation and management of patients with suspected MCAS.

Chronic recurrent multifocal osteomyelitis (CRMO) is a rare idiopathic autoinflammatory bone disease consisted on non-infective inflammation of bones. Diagnostic approach is challenging and required exclusion of other causes such as malignancies or infections. Non-steroid anti-inflammatory drugs (NSAIDs) and corticosteroids are usually applied as first-line therapy in CRMO patients; however, some cases require more intensive therapy with second-line agents to control disease activity. We hereby describe the use of colchicine as a non-conventional second-line disease-modifying anti-rheumatic drug in two pediatric patients with CRMO refractory to NSAIDs and corticosteroids. Our data indicate that colchicine might prove an important area for future research as a potential therapeutic option with easy administration, low cost and a good safety profile, in CRMO patients refractory to first-line therapy.