Anakinra as a first-line therapy for systemic juvenile idiopathic arthritis when nonsteroidal anti-inflammatory drug treatment fails: A single-center French retrospective study.

Introduction: Anakinra has dramatically improved the management of systemic juvenile idiopathic arthritis (SJIA) over the last decade. Nevertheless, management remains inconsistent; corticosteroids are still frequently used. We analyzed the course of SJIA in children treated with anakinra according to the time of treatment initiation after disease onset.

Systemic lupus international collaborating clinics-2012 and European league against rheumatism/American college of rheumatology-2019 classification criteria for systemic lupus erythematosus associated with childhood-onset auto-immune cytopenia.

Introduction: Systemic Lupus Erythematosus (SLE) can be diagnosed using the 2012 criteria of the Systemic Lupus International Collaborating Clinics (SLICC) and, more recently, the 2019 criteria of the European League Against Rheumatism/American College of Rheumatology (EULAR/ACR). Hematological involvement is scored differently by these classifications. Our objective was to compare both criteria in a cohort of children with autoimmune cytopenia (AIC)-associated SLE.

Pediatric refractory chronic immune thrombocytopenia: Identification, patients' characteristics, and outcome.

Refractory chronic immune thrombocytopenia (r-cITP) is one of the most challenging situations in chronic immune thrombocytopenia (cITP). Pediatric r-cITP is inconsistently defined in literature, contributing to the scarcity of data. Moreover, no evidence is available to guide the choice of treatment.

Antinuclear antibody-associated autoimmune cytopenia in childhood is a risk factor for systemic lupus erythematosus.

Autoimmune cytopenia (AIC) in children may be associated with positive antinuclear antibodies (ANA) and may progress to systemic lupus erythematosus (SLE). We evaluated the risk of progression to SLE of childhood-onset ANA-associated AIC. In the French national prospective OBS'CEREVANCE cohort, the long-term outcome of children with ANA-associated AIC (ANA titer ≥1/160) and a subgroup of children who developed SLE were described.

Impact of age at diagnosis, sex, and immunopathological manifestations in 886 patients with pediatric chronic immune thrombocytopenia.

Pediatric chronic immune thrombocytopenia (cITP) is a heterogeneous condition in terms of bleeding severity, second-line treatment use, association with clinical and/or biological immunopathological manifestations (IMs), and progression to systemic lupus erythematosus (SLE). No risk factors for these outcomes are known. Specifically, whether age at ITP diagnosis, sex, or IMs impact cITP outcomes is unknown.

4C3 Human Monoclonal Antibody: A Proof of Concept for Non-pathogenic Proteinase 3 Anti-neutrophil Cytoplasmic Antibodies in Granulomatosis With Polyangiitis.

Granulomatosis with polyangiitis (GPA) is a severe autoimmune vasculitis associated with the presence of anti-neutrophil cytoplasmic antibodies (ANCA) mainly targeting proteinase 3 (PR3), a neutrophilic serine proteinase. PR3-ANCA binding to membrane-bound PR3 on neutrophils induce their auto-immune activation responsible for vascular lesions. However, the correlation between PR3-ANCA level and disease activity remains inconsistent, suggesting the existence of non-pathogenic PR3-ANCA.

Prospective study of anterior chest wall involvement in ankylosing spondylitis and psoriatic arthritis.

A prospective study of anterior chest wall involvement was conducted in 50 ankylosing spondylitis patients and 50 psoriatic arthritis patients in the absence of palmoplantar pustulosis. All patients underwent a physical examination, tomograms, and a radionuclide bone scan. Magnetic resonance imaging with gadolinium was done in some cases.

[Dermatomyositis associated with two neoplasms].

A case of dermatomyositis in a patient with two different malignancies is reported. Skin lesions cleared only following exeresis of the second tumor. This case provides the opportunity for a discussion of the main theories put forward to explain relationships between dermatomyositis and malignant disease.

[Incidence of signs indicating psoriatic rheumatism in radiological involvement of fingers and toes. Apropos of 193 cases of psoriatic arthropathy].

On the basis of 193 cases of psoriatic arthropathy, the authors sought the incidence and rapidity of onset of signs suggestive of this type of rheumatic disorder when there are radiological lesions of the fingers and toes. They conclude that when such radiological lesions exist, signs suggestive of psoriatic arthropathy are frequent (90%), early (85% of cases of psoriatic arthropathy present for less than 5 years) and predominantly involve the distal parts of the fingers and toes (85.5%).

[HLA-B and psoriatic rheumatism. Study of 193 cases].

Study of the HLA-B grouping of 193 patients with psoriatic arthropathy (PA), compared with that of a control series consisting of 2,706 healthy subjects representative of the French population, revealed a statistically significant link between PA and antigens B27, B17 and B16. In contrast to reported findings of earlier studies, the authors found no link between an HLA-B antigen and any particular topographical or anatomical form of PA. Antigen B27 was not statistically significantly linked with axial forms of PA nor did it protect against peripheral forms.