[Hypertension in scleroderma: A vital emergency]. / L'HTA au cours de la sclérodermie systémique : une urgence vitale.

PURPOSE: In systemic sclerosis, hypertension is feared because it is often heralding severe renal impairment. The objective of our study was to identify the frequency of arterial hypertension and clarify its etiologies in this condition. PATIENTS/METHODS: Our study was prospective. From January 2008 to May 2012, we have included all patients over the age of 16 years which featured a systemic scleroderma meeting the criteria for classification of Leroy and Medsger modified. Blood pressure was systematic and hypertension was defined as a greater than 140/90 mmHg PAS/PAD. RESULTS: We have collected 60 patients. It was 50 women and 10 men with an average age of 41.1 ans ± 13.03. Arterial hypertension was noted in sixteen patients (26.7%) with an average age of 48.8 years ± 13.21. It was nine diffuse cutaneous systemic scleroderma of six limited cutaneous scleroderma and one case of scleroderma sine scleroderma. Etiologic research hypertension had concluded to a renal cause in 12 patients. It was five scleroderma renal crisis (SRC), three vascular nephropathies, four chronic kidney failure (CKD) including three terminals and a moderate CKD. An 'essential' so-called HTA was observed in four patients. Hypertension was a major sign that reported five cases of SRC. These patients had received treatment anti hypertensive and renal extra cleansing. DISCUSSION: Hypertension is common in systemic scleroderma. Our data approximates of literature when its frequency and severity. CONCLUSION: Hypertension is a major warning sign that under no circumstances should overlook it or novo or secondary aggravation. Its support must be very early under penalty to put at stake the life-threatening in particular during the CRS.

[Immunological abnormalities in myelodysplastic syndromes. Prospective study (series of 40 patients)]. / Manifestations immunes associées aux syndromes myélodysplasiques. Etude prospective de 40 patients.

UNLABELLED: The association of myelodysplastic syndromes (MDS) with auto-immune diseases and humoral disorders have already been reported. In this prospective study we tried to estimate type and the frequency of immunological associated diseases among patients affected by MDS. PATIENTS-METHODS: In this prospective study, auto-immune disease and humoral immunity disorders were systematically searched during MDS and conversely MDS searched during cytopenia. All MDS secondary to chemotherapy and the children's MDS were excluded. The MDS diagnosis was established according to FAB criteria and patients were classified in two groups A or B according to presence (group A) or not (group B) of dysimmune manifestations. RESULTS: Forty patients(19 males and 21 females, mean age of 56,6 years) with MDS have been enrolled during this period (group A: 20 patients). Associated diseases are following: systemic lupus erythematosus (three), lupus-like syndrome(one), sarcoidisis(one), Sjogrën syndrome(one), polyarthritis (two), chronic liver diseases (three), autoimmune thyroid diseases (two), pyoderma gangrenosum (one), Crohn's disease(one), haemolytic anaemia (one), and pericardial effusion(one). CONCLUSION: A wide spectrum of auto-immune manifestations is frequently reported in myelodysplastic syndromes. Further studies are necessary for discuss the current physiopathological hypothesis with their therapeutic relevance.