[Complaints, needs of patients with systemic sclerosis: a better understanding for a better care]. / Plaintes et besoins des patients atteints de sclérodermie systémique : une meilleure connaissance afin d'améliorer le suivi.

PURPOSE: Systemic sclerosis (ScS) is very heterogeneous in its clinical presentation and its therapeutic care is not codified. A better knowledge of the patients' needs and complaints could improve the patient educational strategies and their global care. METHODS: A self-administered questionnaire aimed to the ScS patient was developed by subspecialty physicians and nurses involved in patient education. It was a cross-sectional study that also included several validated scales: the health control locus scale, the Mactar, HAD and sHAQ scales. RESULTS: One hundred and eight patients (91 women; 18 limited ScS, 71 limited cutaneous ScS, 19 diffuse ScS) filled in the questionnaires. Fatigue was the main complaint in all types of ScS, independently of the ScS type. The aesthetic discomfort mentioned by the patients suffering from cutaneous sclerosis or from telangectasia was important and reached 52±33mm on a 100-mm visual scale. It was more common in the patients presenting a diffuse form of the illness but the difference did not reach a statistical significance (P=0.06). Twenty-seven percent of the patients said they were very or extremely worried because of the degradation of their physical appearance. The functional discomfort linked to the cutaneous sclerosis was rated 50±32mm on a 100-mm visual scale. The intensity of the pain, the importance of the functional discomfort linked to the sclerosis and the intensity of the dyspnea were correlated to the sHAQ (P<0.001). Patients having more frequent recurrent digital ulcers had higher sHAQ scores (P=0.04). The repercussions on the professional life were linked to fatigue first, to the Raynaud's syndrome and to arthralgia. The repercussions on the personal life were mainly linked to the fatigue, the pain and the dyspnea. The patients' compliance was good. CONCLUSION: Fatigue, pain, dyspnea and discomfort linked to sclerosis are major chronic symptoms of the patients with ScS. Identifying the needs and complaints of the patients with ScS should help to improve their care by implementation of an educational program.

[Visual hallucinations and giant cell arteritis: the Charles Bonnet syndrome]. / Hallucinations visuelles et maladie de Horton : à propos du syndrome de Charles Bonnet.

In patients with visual hallucinations, diagnostic strategy is unclearly codified. In patients known to have giant cell arteritis, the main diagnostic assumption is disease relapse. Indeed, this should lead to rapid corticosteroid therapy. However, the Charles Bonnet syndrome, that is a poorly known etiology of visual hallucinations usually observed in elderly people, should be part of the differential diagnosis. We report a 87-year-old woman, with a 2-year history of giant cell arteritis who was admitted with an acute onset of visual hallucinations and who met all the criteria for Charles Bonnet syndrome.

[Superficial cranial venous thromboses preceding the diagnosis of giant cell arteritis]. / Thromboses veineuses superficielles céphaliques satellites d'une artérite temporale de Horton, une manifestation exceptionnelle.

Temporal arteritis is a large-vessel vasculitis predominantly affecting the external carotid and its branches. Venous thrombosis is rarely found at the onset of temporal arteritis, particularly when venous symptoms precede arterial involvement. We report the case of a 70-year-old woman consulting for bilateral superficial frontal venous thrombosis. Superficial bilateral temporal venous thrombosis occurred under adequate anticoagulation before the onset of arterial symptoms suggestive of temporal arteritis. We then discuss the pathophysiology of venous thrombosis in patients with temporal arteritis.

Advanced glycation end-products: implications for diabetic and non-diabetic nephropathies.

Glycation is the process whereby sugars bind to the free amine residues of proteins. These newly formed modified molecular species are known as 'advanced glycation end-products', or AGEs. AGE toxicity may occur through at least three mechanisms: interaction with the receptor for AGEs (RAGE); tissue deposition; and in situ glycation. AGEs trigger proinflammatory, profibrotic and procoagulant cellular responses that are capable of damaging tissues, often targeting particular organs. In diabetic patients, the conditions needed to promote AGE formation are all present, and are further accentuated by accompanying renal failure. The aim of this review is to outline the involvement of AGEs in the various forms of renal pathology associated with diabetic and non-diabetic nephropathies. AGEs are present in all renal compartments in diabetic patients, including the vessels, glomeruli, tubules and interstitium. Many cell types may be activated-specifically, endothelial, tubular and mesangial cells, and podocytes. AGEs play a major role in the accumulation of extracellular matrix, as occurs in diabetic glomerulosclerosis, and are also involved in most diabetic (renovascular, microangiopathic and glomerular) and non-diabetic renal injury associated with progressive glomerulosclerosis and ageing.