sclerodermie

Interstitial lung disease is a frequent complication of systemic sclerosis and has now become the leading cause of death in this disorder. It mainly occurs during the first five years after the diagnosis of systemic sclerosis. Various risk factors are associated with the occurrence of interstitial lung disease, including the presence of anti-topoisomerase I antibodies (Scl-70) and the diffuse cutaneous form of systemic sclerosis. The most common radio-pathological presentation is nonspecific interstitial pneumonia, followed by usual interstitial pneumonia...

OBJECTIVES: To assess laser Doppler-recorded postocclusive reactive hyperemic responses in vibration-induced Raynaud's phenomenon and compare it with primary and secondary to sclerodermy Raynaud's phenomenon. MATERIAL AND METHODS: Thirty patients with vibration-induced Raynaud's phenomenon and 30 healthy controls and patients with primary and secondary to sclerodermy Raynaud's phenomenon were investigated. Fingerpulp skin blood flow was monitored by laser Doppler flowmetry during postocclusive reactive hyperemia test...

OBJECTIFS: L'atteinte rénale au cours des maladies systémiques est fréquente et de présentation variable. Le but de notre travail est de relever les particularités cliniques, étiologiques, thérapeutiques et pronostiques de l'insuffisance rénale liée aux maladies systémiques. MéTHODES: Étude rétrospective menée au service de médecine interne de l'Hôpital Habib Thameur sur une période de 15 ans, colligeant 28 cas d'IR au cours des maladies systémiques. Le diagnostic de l'IR était essentiellement biologique...

BACKGROUND: In patients with systemic sclerosis (scleroderma, SSc), impaired hand function greatly contributes to disability and reduced quality of life, and is insufficiently relieved by currently available therapies. Adipose tissue-derived stromal vascular fraction (SVF) is increasingly recognised as an easily accessible source of regenerative cells with therapeutic potential in ischaemic or autoimmune diseases. We aimed to measure for the first time the safety, tolerability and potential efficacy of autologous SVF cells local injections in patients with SSc with hand disability...

OBJECTIVES: To identify clinical, functional and health-related quality of life (HRQoL) correlates of clinically significant symptoms of anxiety and depression in patients with systemic sclerosis (SSc). METHODS: Three-hundred-and-eighty-one patients fulfilling the American College of Rheumatology and/or the Leroy and Medsger criteria for SSc were assessed for visceral involvement, disability and HRQoL (assessed by SF-36). Clinically significant symptoms of anxiety and depression were evaluated with the Hospital Anxiety Depression Scale (HAD) (defined cut-off≥8)...

As a limited form of sclerodermy, CREST syndrome is characterized by calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia, which determine the acronym CREST. Calcinosis is a particularly difficult entity to treat given the paucity of effective options described in the literature. Treatment of finger calcinosis has a wide range of possibilities depending on the extent of calcifications and the involvement of deep structures. From a surgical point of view, whereas simple removal is adequate in minor outpatient cases, a radical debridement in the major and more painful cases seems required...

OBJECTIVES: To assess the association of gender with clinical expression, health-related quality of life (HRQoL), disability, and self-reported symptoms of depression and anxiety in patients with systemic sclerosis (SSc). METHODS: SSc patients fulfilling the American College of Rheumatology and/or the Leroy and Medsger criteria were assessed for clinical symptoms, disability, HRQoL, self-reported symptoms of depression and anxiety by specific measurement scales...

Esophageal involvement occurs in about 80% of patients with systemic sclerosis, with a marked diminution of peristaltic pressures in the distal two-thirds of the esophagus. Our aims were to more fully characterize esophageal motility disorders in systemic sclerosis using high-resolution manometry (HRM) and to determine predictive factors of esophageal involvement. Fifty-one patients (46 females) with systemic sclerosis were included in this retrospective study. Esophageal motility was characterized with HRM...

OBJECTIVE: Digital ulcers are the most frequent vascular manifestations of systemic sclerosis (SSc). Clinical features of patients with prior or current digital ulcers have not been extensively described. This cross-sectional analysis of a large multicenter cohort compared the characteristics of SSc patients with prior or current digital ulcers with those never affected. METHODS: Patients with prior/current digital ulcers or never affected were identified in the cohort of SSc patients enrolled in the French ItinérAIR-Sclérodermie registry...

OBJECTIVE: An algorithm for the detection of pulmonary arterial hypertension (PAH), based on the presence of dyspnea and the findings of Doppler echocardiographic evaluation of the velocity of tricuspid regurgitation (VTR) and right-sided heart catheterization (RHC), which was applied in a large multicenter systemic sclerosis (SSc) population, estimated the prevalence of PAH to be 7.85%. The aim of this observational study was to investigate the incidence of PAH and pulmonary hypertension (PH) during a 3-year followup of patients from the same cohort (the ItinérAIR-Sclérodermie Study)...

OBJECTIVES: This longitudinal study investigated survival, risk factors and causes of death in the multicentre ItinérAIR-Sclérodermie cohort of patients with SSc without severe pulmonary fibrosis or severe left heart disease at baseline. METHODS: At 3-year follow-up, vital status was obtained from investigators or French national death records. Causes of death were classified as SSc-related or otherwise. Data were censored at 37 months, time of death or loss to follow-up, whichever was earlier...

Digital ulcers are a frequent event in systemic scleroderma. Despite their frequency, there is currently no consensus about their management. Curative treatment is based on local care and vasodilatating agents, together with the prevention of superinfection. Preventive measures require the elimination of risk factors and reeducation. For multiple recurrent ulcers, endothelin receptor inhibitors or prostacyclin derivatives are also prescribed. Surgery is useful in the case of substantial retraction or bothersome calcifications...

OBJECTIVES: To describe presentation and outcome of patients with scleroderma renal crisis (SRC). METHODS: SRC was defined as rapidly progressive oliguric renal insufficiency and/or rapidly progressive arterial hypertension occurring during the course of systemic sclerosis (SSc). Chronic dialysis-free survival was analysed using multivariate Cox proportional hazards regression models. The risk for developing SRC associated with corticosteroid (CS) exposure during the preceding 1- or 3-month periods was analysed according to a case-crossover design...

Classification criteria of scleroderma aim to delineate the different clinical forms of the disease, which are associated with different prognoses. The basic classification between localized and systemic scleroderma remains pertinent. Among the former, the problems are essentially esthetic or sometimes functional; but the second may yield many other problems which are pertinent to function but also to life, owing to the involvement of body organs, including lungs, heart, digestive tract, kidneys and locomotor system...

CONTEXT AND OBJECTIVES: Pulmonary hypertension (PHT) represents one of the severest complications and is life-threatening for patients suffering from systemic sclerosis (SSc). In France, the modalities for screening and treating PHT related to SSc are not well codified and no consensus has been reached. We conducted a survey among physicians inscribed on the list of the French Research Group on Sclerosis (GRFS - Groupe de Recherche Francais sur la Sclerodermie) to gather information on the status of the management of PHT related to SSc...

PURPOSE: Pulmonary arterial hypertension (PAH) is a severe complication of scleroderma. Its prevalence varies from 5% to 35% in the literature. A systematic yearly screening is recommended for early detection and management of PAH, but no precise algorithm is yet available. METHODS: From literature analysis as well as evaluation of medical needs and practices, a multidisciplinary board of experts proposed an algorithm for the screening of PAH in scleroderma. RESULTS: This algorithm is based on a precise Doppler echocardiography methodology for the purpose of screening scleroderma patients for PAH...

OBJECTIVE: To investigate the metric properties and the validity of the Medical Outcome Study Short Form 36 (SF-36), a questionnaire to assess the quality of life, in patients with either diffuse or limited systemic sclerosis (SS), and to examine the effect of the disease on quality of life. METHODS: Cross sectional study of 86 patients with a SS (64 diffuse SS, 22 limited SS). Disease severity was assessed by clinical examination, pulmonary functional tests and Health Assessment Questionnaire (HAQ) modified for scleroderma...

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The authors report two observations of T.A.O. placed under the heading of "Systems Affections" after the discovery of immunologic abnormalities. Vascular troubles in L.E.A.D. and generalised sclerodermis are, apart from the Raynaud Syndrom, exceptional. They oriente specially towards a T.A.O., and in absence of caracteristic symptoms, they retard the diagnosis of a connective. The reader is reminded of the characteristics of the "Buerger Affection" which must no longer be considered as unique anatomoclinic entity, due to modern immunologic and phlebologic exams...