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Siewert, Ricardo Russo, Nakamura, Ichiro, Mielke, Olaf Hermann Hendrik (2014): Psoralis mirnae sp. nov., the first species of the skipper genus from Central America (Lepidoptera: Hesperiidae). Zootaxa 3861 (1): 91-95, DOI: http://dx.doi.org/10.11646/zootaxa.3861.1.6

The juvenile spondyloarthropathies (JSpA) are a group of related rheumatic diseases characterized by involvement of peripheral large joints, axial joints, and entheses (enthesitis) that begin in the early years of life (prior to 16(th) birthday).The nomenclature and concept of spondyloarthropathies has changed during the last few decades. Although there is not any specific classification of JSpA, diseases under the spondyloarthropathy nomenclature umbrella in the younger patients include: the seronegative enthesitis and arthropathy (SEA) syndrome, juvenile ankylosing spondylitis, reactive arthritis, and inflammatory bowel disease-associated arthritis. Moreover, the ILAR criteria for Juvenile Idiopathic Arthritis includes two categories closely related to spondyloarthritis: Enthesitis-related arthritis and psoriatic arthritis.We review the pathophysiology and the use of biological agents in JSpA. JSpA are idiopathic inflammatory diseases driven by an altered balance in the proinflammatory cytokines. There is ample evidence on the role of tumor necrosis factor (TNF) and interleukin-17 in the physiopathology of these entities. Several non-biologic and biologic agents have been used with conflicting results in the treatment of these complex diseases. The efficacy and safety of anti-TNF agents, such as etanercept, infliximab and adalimumab, have been analysed in controlled and uncontrolled trials, usually showing satisfactory outcomes. Other biologic agents, such as abatacept, tocilizumab and rituximab, have been insufficiently studied and their role in the therapy of SpA is uncertain. Interleukin-17-blocking agents are promising alternatives for the treatment of JSpA patients in the near future. Recommendations for the treatment of patients with JSpA have recently been proposed and are discussed in the present review.

Purpose Juvenile Systemic Sclerosis (JSS) is a rare, complex disease. Usually, patients with JSS receive specialized care from a multidisciplinary team in tertiary hospitals. In order to deliver high standard care, systematic clinical practice strategies should be designed. Based on these premises, we developed a JSS Clinic in the year 2008. Objective: To describe the structure and process of a JSS interdisciplinary Clinic, as well as outcomes observed. Methods During the period 2008-2010, patients with a diagnosis of JSS entered a structured follow-up in a dedicated clinic. Disease-specific guidelines and outcome measures were designed and applied. An interdisciplinary team was formed under the leadership of a pediatric rheumatologist: pediatricians, pediatric dermatologists, neurologists, pulmonologists, cardiologists, social workers, nutritionists, kinesiologists , psychiatrists and nurses. The team gathered in the Day Hospital facility of a tertiary pediatric hospital. Prior to the clinic day, short

Macrophage activation syndrome (MAS) is a potentially fatal complication of systemic juvenile idiopathic arthritis (sJIA), whose prompt recognition and treatment are critical. However, early diagnosis of MAS is often challenging and none of the current diagnostic criteria is satisfactory. An international project aimed to develop a new set of classification criteria for MAS was recently started.

Case reports Neuromyelitis optica (NMO, Devic's disease) is a severe autoimmune disorder predominantly involving optic nerves and spinal cord [1]. Usually isolated, it has been associated with systemic autoimmune diseases in adult patients [2]. We report 2 children with a systemic autoimmune disease who developed NMO. Patients were female; NMO symptoms started at age 8 and 12 years. Patient 1 had a diagnosis of Systemic Lupus Erythematosus (SLE) (fever, cytopenias, mesangeal glomerulonephritis, positive ANA, anti-DNA, anti-Sm). She developed vomiting, tremor, hyperreflexia, paresthesia, neurogenic bladder and progressive vision loss 2 years after SLE onset. Neuroradiological investigations disclosed longitudinally extensive transverse myelitis and bilateral optic nerve involvement. Patient 2 had recurrent parotitis for 1 year before she developed vision loss, papilitis, dystonia and paresthesia. She exhibited positive ANA, anti-Ro and antiLa, and objective eye dryness. MRI evidenced lesions in brainstem and spinal cord, and evoked potentials revealed optic nerve involvement. A diagnosis of Sjogren's Syndrome (SS) associated with NMO was made. NMO-Ig was detected in both patients' sera. Intravenous and p.o. high dose steroids, a 6-month course of monthly I.V. cyclophosphamide (up to 1 g/m2/dose), followed by azathioprine (2–3 mg/Kg/day) as maintenance therapy, were used in both (plasmapheresis in one). Visual, motor and sensitive symptoms dramatically improved. Mild relapses occurred in both children; they were successfully treated with steroids. Patients are currently well, with improved vision and residual lesions in MRI. Conclusion NMO can occur in the setting SLE or SS in children. Intensive immunosuppressive therapy may induce remission and prevent visual loss.

Blau syndrome is an autosomal dominant monogenic granulomatous disease associated with gain of function mutations at or near the NACHT domain of NOD2; it is the only form of granulomatous arthritis with a known gene mutation. Although its phenotype has been amply described as a triad of arthritis, uveitis and dermatitis in case series and retrospective cohorts, prospective studies on natural history and outcome have not been done.