La Revue de médecine interne
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Sjögren's syndrome (SS) is a systemic orphan disease. It is characterized by the involvement of epithelial tissues leading to the term of autoimmune epithelitis. New classification criteria have been developed in 2016. ⋯ Major advances have been made in this field: pathogeny is better understood, new predictors are available and progresses have been made in the management of this severe complication. Research in the field of SS is very dynamic as illustrated by the high number of therapeutic trials. There is hope that these innovations, reviewed in the present article, will have potential significant repercussions for the patients in the next few years.
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Primary open-angle glaucoma is a progressive chronic optic neuropathy, typically bilateral, that occurs after the age of 40 years. It is the second leading cause of irreversible blindness in the world. ⋯ Diagnosis and monitoring of primary open-angle glaucoma arebased on both analysis of structural alteration, by clinical examination of optic disc completed by imaging tests (Ocular Coherence Tomography), and functional alterations, by visual field tests. The only effective treatment to slow primary open-angle glaucoma progression is the reduction of the intraocular pressure with anti-glaucomatous eye drops, laser or surgical treatments.
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Case Reports
[Acute carpal tunnel syndrome caused by a thrombosis of a persistent median artery: 2 case reports].
Carpal tunnel syndrome is a common peripheral neuropathy, usually idiopathic or post-traumatic due to the compression of the median nerve. Numbness and paresthesias in the median nerve distribution are the most common symptoms associated with this condition. Persistent median artery is a rare anatomic variation, thrombosis of this additional artery can be responsible for an acute carpal tunnel syndrome, and patients frequently complain about coldness and acute hand swelling. ⋯ These thrombosis might also be due to traumatic causes. No guidelines are currently available to help physicians for the management of carpal tunnel syndrome from thrombosed persistent median artery. Antiplatelet therapy, statin, anticoagulant might be helpful, and surgery has sometimes be reported as effective.
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Hemophagocytic lymphohistiocytosis (HLH) is a rare syndrome frequently secondary to infectious disease, especially in immuno-compromised patients. We report a HLH secondary to disseminated nocardiosis and Streptomyces spp pulmonary infection. ⋯ Recent diagnosis of HLH without obvious etiology should lead to etiological investigation, including the search for infections with slow-growing bacteria such as Nocardia or Streptomyces spp.
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Sideroblastic anemia is a rare cause of microcytic anemia, which is characterized by ring sideroblasts on bone marrow aspirate. This anemia can be congenital or acquired. ⋯ Pyridoxine deficiency must be sought in sideroblastic anemia in patients at risk.