juvenile polyarteritis nodosa

Certain vasculitides have an increased prevalence among patients with familial Mediterranean fever (FMF). Subsequently, it was noticed that patients with certain rheumatic diseases had an increased carrier rate for mutations in the MEFV gene including seronegative spondyloarhtropatheis, Henoch Schönlein purpura, polyarteritis nodosa and some forms of juvenile idiopathic arthritis. Furthermore in populations where the disease is rare, certain polymorphisms have been associated with a severe inflammatory complication in arthritis...

BACKGROUND: Infliximab and etanercept have been included in the Israeli national list of health services since 2002 for rheumatoid arthritis and juvenile idiopathic arthritis, and since 2005 for psoriatic arthritis and ankylosing spondylitis. The regulator (Ministry of Health and health funds) mandates using fixed doses of infliximab as the first drug of choice and prohibits increased dosage. For other indications (e.g., vasculitis), anti-tumor necrosis factor therapy is given on a "compassionate" basis in severe refractory disease...

OBJECTIVE: Temporal artery vasculitis (TAV) in patients younger than 50 years is extremely rare. A case of TAV in an 18-year-old man is described here, followed by a literature review regarding cases of all types of vasculitic involvement of the temporal arteries in the young. METHODS: Review of the English literature on vasculitis involving the temporal arteries in young patients, based on a PubMed search. RESULTS: Less than 40 cases of vasculitic involvement of temporal arteries in the young have been described...

Central nervous system (CNS) manifestations are not rare in pediatric rheumatic diseases. They may be a relatively common feature of the disease, as in systemic lupus erythematosus (SLE) and Behçet's disease. Direct CNS involvement of a systemic rheumatic disease, primary CNS vasculitis, indirect involvement secondary to hypertension, hypoxia and metabolic changes, and drug associated adverse events may all result in CNS involvement. We have reviewed the CNS manifestations of SLE, Behçet's disease, Henoch-Schönlein purpura, polyarteritis nodosa, juvenile idiopathic arthritis, juvenile ankylosing spondylitis, familial Mediterranean fever, scleroderma, sarcoidosis, Wegener's granulomatosis, Takayasu's arteritis, CINCA syndrome, Kawasaki disease, and primary CNS vasculitis; and adverse CNS effects of anti-rheumatic drugs in pediatric patients...

Tumor necrosis factor (TNF) blockade has been used successfully to treat a number of rheumatic disorders that have a substantial burden of illness. In children, the TNF antagonists are used mainly for the treatment of juvenile idiopathic arthritis (JIA). There are, however, a variety of rare systemic inflammatory diseases, in which TNF blockade appears promising. Preliminary data in adults suggest that several forms of vasculitis appear to be responsive to TNF antagonists-Behcet's disease, polyarteritis nodosa, Wegener granulomatosis, among others...

Polyarteritis nodosa is a rare vasculitis of small and medium arteries. It can occur in a systemic form with multi-organ involvement, or as a limited form confined to the skin, muscles, joints and peripheral nerves called cutaneous polyarteritis nodosa. Both forms are rare in adults and even more in children. The caues of this vasculitis remain unknown but some viruses and bacteria have been implicated, specially, Streptococcus. We present the case of a 6-year-old child who developed cutaneous polyarteritis nodosa following a probable streptococcal infection...

OBJECTIVE: Juvenile Polyarteritis nodosa (PAN) and Kawasaki Disease (KD) are disseminated vasculitides of unknown cause affecting small- and medium-sized vessels in children. We present an unusually severe case that fulfilled criteria for both KD and PAN. The diagnosis, overlapping clinical features, and treatment options for the 2 diseases are discussed. METHODS: A 3-year-old girl with systemic vasculitis is presented. We compare our case to 4 other cases reported in the literature which presented with a similar diagnostic dilemma...

OBJECTIVE: To characterize pediatric patients who had been diagnosed with polyarteritis nodosa (PAN) through necrotizing vasculitis of the small and mid-size arteries or those with characteristic findings on angiograms data were collected. STUDY DESIGN: Pediatricians were asked to classify their patients into one of the four suggested groups for juvenile PAN. Twenty-one pediatric centers worldwide participated with 110 patients. RESULTS: The girl:boy ratio was 56:54, with a mean age of 9...

Despite the fact that in recent decades many advances have been made in classification, outcomes measurements and therapeutic regimens, connective tissue diseases remain a challenge for paediatric rheumatologists. Corticosteroids are still the first choice therapy in juvenile systemic lupus erythematosus (SLE); however, it is time for a new therapeutic approach with immunosuppressant drugs in order to obtain a more prolonged survival and reduced organ system damage. A prevention of relapse of thromboembolic phenomena using low-dose aspirin is strongly suggested in children with anticardiolipin antibodies, particularly in those with SLE...

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No abstract text is available yet for this article.

OBJECTIVE: We analysed our experience with the use of iloprost for the treatment of critical ischaemic digits (ID) in children with connective tissue diseases (CTD) in order to assess its safety and efficacy. METHODS: This was a retrospective analysis of paediatric patients with CTD who were treated with iloprost for critical ID resistant to conventional therapy. Information on demographics, clinical and laboratory features, the regimen of iloprost treatment and outcome were collected...

Two, full sibling, Welsh springer spaniel presented at 8 and 18 mo of age with rapidly progressive ataxia, recumbency, and pyrexia. The spinal cord contained extensive subdural hemorrhage and, in 1 dog, suppurative and necrotizing arteritis in the dura. The findings suggest a familial form of canine juvenile polyarteritis syndrome.

INTRODUCTION: Some necrotizing vasculitis may be associated with familial Mediterranean fever (FMF). We report a new case of polyarteritis nodosa (PAN) that preceded the diagnosis of FMF. OBSERVATION: A young woman of Turkish origin had a long childhood history of inflammatory arthralgia and myalgia, leading to the provisional diagnosis of chronic juvenile arthritis, then, after a confirmative muscle biopsy, to the diagnosis of PAN, whose outcome remained benign...

PURPOSE: To describe a case of ocular tilt reaction caused by vasculitic lesions in the midbrain in a child with polyarteritis nodosa. DESIGN: Observational case report. METHODS: A 5-year-old girl with a chronic illness developed diplopia associated with a left head tilt, right hypertropia, torsional nystagmus, slowed vertical saccades and poor convergence. Fundoscopic examination demonstrated conjugate leftward torsion of the eyes consistent with a sustained ocular tilt reaction...

Ocular inflammation occurs in many patients with systemic rheumatic disease. The best examples are rheumatoid arthritis, juvenile rheumatoid arthritis, temporal arteritis, systemic lupus erythematosus, Wegener's granulomatosis, polyarteritis nodosa, relapsing polychondritis, and Adamantiades-Behçet's disease. Ocular inflammation may precede the symptoms of the systemic disease and can be helpful in systemic diagnosis. After diagnosis, ocular inflammation can mark the severity of the systemic condition. Thus, prompt diagnosis and treatment of inflammatory conditions of the eye are warranted and may be sight- and life-saving...

OBJECTIVE: To examine the role of inflammatory rheumatic diseases and comparable conditions in the etiology of severe uveitis leading to visual impairment and blindness. METHODS: A retrospective study based on the Finnish Register of Visual Impairment. At the end of 1996, the Finnish Register of Visual Impairment included 296 uveitis patients in whom uveitis was the main cause of visual impairment. The patient records were examined retrospectively to investigate the etiology of severe uveitis...

Classic polyarteritis nodosa is a multisystem inflammatory disease associated with necrotizing vasculitis of small and medium arteries. In most cases, the causes of polyarteritis nodosa remain unknown, but viruses (HBV, HCV, HIV) and microbes (especially streptococcus) have been considered as etiologic or contributing factors. A 13-year-old boy was admitted with fever, skin lesions, polyarthritis and muscle involvement. A muscle biopsy demonstrated a necrotizing vasculitis and antistreptolysin titre was tremendously increased...

Because of complex immunologic, renal, infectious, fertility, and psychological issues, renal disease secondary to rheumatic disease represents one of the most challenging medical problems for subspecialist and primary care physician alike. However, adolescents with renal disease respond better to treatment than do adults. This chapter addresses the renal manifestations of rheumatic diseases, current treatment options, and complications associated with treatment and long-term prognosis.

Pediatric autoimmune cardiovascular disease can cause serious, sometimes life threatening sequelae on the pediatric population. Valvular, myocardial, and pericardial involvement causing morbidity and mortality can occur in association to rheumatic heart disease, systemic lupus erythematosus, and juvenile rheumatoid arthritis. Serious and potentially life threatening coronary artery involvement can occur in patients with childhood polyarteritis nodosa, Takayasu arteritis, and Kawasaki disease.