La Revue de médecine interne
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Headache, visual disturbances and shoulder and hip girdle pain are frequent symptoms of consultation and the diagnosis of giant cell arteritis (GCA) can be evoked in these situations. However, GCA is a rare disease, and the management modalities of a clinical suspicion of GCA are poorly described, which warranted this study. ⋯ Some suspected GCA patients would be managed by their GPs. Imaging was little used for the diagnosis of GCA and TAB remained the preferred diagnostic examination. The initial prednisone equivalent dose varied between GP and other specialists.
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Autoimmune and autoinflammatory diseases (AIDs) are a heterogeneous group of diseases. They can occur in childhood and account for significant morbidity and mortality. Transitioning from pediatric to adult healthcare can be difficult for patients and their families. ⋯ This requires the active participation of the patient, his or her family, as well as the pediatric and adult medical teams. The transition process involves multidisciplinary care and dedicated therapeutic education programs. Finally, the identification of medical specialists by region, trained in rare AIDs and accompanied by expert patients, may improve the management of patients with rare AIDs from adolescence to adulthood.
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Review Case Reports
[ANCA vasculitis preceded by isolated joint manifestations. Report of two cases and literature review].
Joint manifestations of ANCA (antineutrophil cytoplasmic antibody) associated vasculitis (AAV) are extremely common in the progression of systemic damages. However, the joint involvement is rarely isolated. The diagnosis and the treatment are difficult in this particular situation and few data are available on the topic. ⋯ Isolated joint manifestations of ANCA vasculitis are rare but can lead to a delay in diagnosis. ANCA vasculitis should be considered in seronegative symmetrical polyarthritis by looking for ANCA in a second line biological test. Methotrexate is the first treatment option to be considered. In case of insufficient response or failure, rituximab seems an interesting option in this context.
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Case Reports Randomized Controlled Trial
[Idiopathic systemic capillary leak syndrome: 2 cases with misleading presentation].
Idiopathic systemic capillary leak syndrome (ISCLS) also known as Clarkson syndrome is a rare and sudden life-threatening entity. Three consecutive phases are described. A first non-specific prodromal phase often manifests as "flu-like" symptoms and precedes capillary leak phase with major hypovolemic and distributive shock leading to serious and frequent multiorgan dysfunction syndrome (MODS). Severe hypovolemia contrasts with edema, and hemoconcentration with hypoalbuminemia. ISCLS is characterized by these two clinical and biological paradoxes. Subsequent recovery phase exhibits organ function restoration along with interstitial/intravascular volumes normalization. The latter occurs spontaneously and systematically in patients surviving from leak phase. ⋯ ISCLS pathophysiology remains unknown but certainly implies transitory endothelial dysfunction. Impossibility of randomized controlled trial for this exceptional disease led to based-on-experience therapeutic guidelines implying symptomatic care (cardiac output surveillance, nephroprotection, prudent fluid intake, prudent vasoactive amine use) and specific therapies (intravenous aminophylline during severe flares). Although enhancing controversial and even deleterious effects during the acute phase, polyvalent immunoglobulins are effective for relapse prevention. Syndromic diagnosis is difficult, but its precocious finding constitutes a key-element in better outcome before organ failure.