Arterial stiffness predicts severe progression in systemic sclerosis: the ERAMS study.

OBJECTIVE: The ERAMS study addressed the value of arterial stiffness in predicting the severity of systemic sclerosis. METHODS: ERAMS was a prospective multicentre cohort study including patients with definite systemic sclerosis. Arterial stiffness was measured by the standardized non-invasive QKd 100-60 method. Clinical evaluation, biological measurements, functional respiratory tests and cardiac Doppler echography were performed at inclusion then each year until 3 years' follow-up was completed. Progression was defined as mild (articulations, muscle, oesophagus or skin involvement) or severe (lung, heart or kidney involvement) by a critical event committee. The prediction of severe progression was studied for QKd 100-60 as well as clinical and biological data at baseline by univariate and multivariate analysis. RESULTS: Ninety-nine patients were included (81 women, 18 men, mean age 57 years, standard deviation 12.5). Although their blood pressure profile was normal, half the patients had increased arterial stiffness (QKd 100-60<200 ms). There was a significant relationship between age-adjusted arterial stiffness and decrease in carbon dioxide diffusion (P<0.03) or haemoglobin rate (P<0.01). By univariate analysis, severe progression after 3 years was predicted by age (P=0.04), lung involvement (P=0.04), diffusion of lung carbon oxide (DLCO) (P<0.01), skin score (P=0.02), haemoglobin (P<0.01) and baseline Qkd 100-60 divided into two classes according to the median (P<0.01). By multivariate analysis, only haemoglobin rate [odds ratio (OR) 0.4, 95% confidence interval (CI) 0.2-0.9] and QKd 100-60 (OR 19.6, 95% CI 1.2-308.2) predicted severe progression of systemic sclerosis. CONCLUSION: The measurement of arterial stiffness by the QKd method is a useful objective method for assessing the prognosis of systemic sclerosis independently from other data.

Routine vs extensive malignancy search for adult dermatomyositis and polymyositis: a study of 40 patients.

OBJECTIVE: To identify potential risk factors and the yield of routine screens for early detection of malignancy associated with dermatomyositis (DM) and polymyositis (PM). DESIGN: Retrospective study of malignancies in all patients with DM or PM followed up between the years 1981 and 2000 and a review of the relationship of DM and PM to malignancy, the usefulness of various tests or examinations for malignancy search, and the patients' course. SETTING: Departments of internal medicine and dermatology in a teaching hospital. PATIENTS: Forty consecutive adult patients with DM (33 cases) or PM (7 cases). MAIN OUTCOME MEASURES: (1) Rate of false-negative results of routine workup and yield (percentage of positive results) of blind malignancy search and (2) comparison of 16 characteristics in patients with malignancy vs those without. RESULTS: Malignancy occurred in 16 patients: 13 with DM and 3 with PM. In all cases, the diagnosis of malignancy was made concurrently with or shortly after the diagnosis of DM or PM. Factors associated with malignancy were recruitment in the internal medicine department (P =.02), constitutional symptoms (P<.01), a rapid onset of DM or PM (P =.02), the lack of Raynaud phenomenon (P<.01), and a higher mean erythrocyte sedimentation rate (P<.01) and creatine kinase level (P<.01). Initial routine search failed to discover 4 malignancies, 3 of which were discovered at an advanced stage by more extensive investigations. The positive result yield of blind malignancy search was only 13% (11 of 87), but reached 28% (5 of 18) for blind abdominal-pelvic and thoracic computed tomographic scans. CONCLUSION: Extensive search for malignancy, particularly computed tomographic scans, may be warranted in at least a subset of patients with DM or PM and risk factors of malignancy.

Concurrent temporal (giant cell) arteritis and malignancy: report of 20 patients with review of the literature.

OBJECTIVE: To determine the frequency of occurrence of malignancy concurrently with temporal arteritis (TA), as well as features and outcome of the vasculitis in such cases. METHODS: In a series of 271 consecutive patients with TA (219 biopsy-proven), we retrospectively analyzed the frequency and type of malignancy concurrent with vasculitis (less than 1 year before or after), as well as the main features and outcome of TA in this setting. We also surveyed all cases published in the French-British literature. RESULTS: We observed 20 patients with TA and concurrent malignancy and reviewed 27 similar published reports. GCA was documented pathologically in 86% of the cases. The time between diagnosis of TA and that of malignancy averaged 3.5 months (synchronous diagnoses in 27 patients). Various locations of cancers were found, particularly the gastrointestinal tract (9 cases); blood malignancies accounted for 45% of cases (lymphoid disorder in 9, myelodysplastic syndrome in 11, chronic myelogenous leukemia in 1). In our patients, logistic regression analysis failed to demonstrate differences between those with and without malignancy, except for a higher frequency of rheumatic involvement in the former group (60% vs 30%; p = 0.01). The initial response to steroid treatment was good in 92% of 40 assessable patients, and the vasculitis course mirrored that of malignancy in only 2 patients. Regarding the outcome of TA, no differences were observed in our patients with and without malignancy. CONCLUSION: Concurrent malignancy in TA is not a rare finding, being observed in up to 7.4% of the cases. Solid malignancies and hematological disorders, especially myelodysplastic syndromes, may represent precipitating factors for development of TA, which infrequently run a paraneoplastic course. Patients with and without malignancy seem almost indistinguishable regarding features and outcome of TA. Physicians who care for patients with TA should be mindful of this potential association, even in typical cases.

Silent, or masked, giant cell arteritis is associated with a strong inflammatory response and a benign short term course.

OBJECTIVE: To determine the frequency, characteristics, and short term outcome of patients who have biopsy-proven giant cell arteritis (GCA) but no local symptoms that can be attributed to vasculitis inflammation [silent temporal arteritis (TA)] throughout the pretreatment course of the disease or an observational period lasting at least 2 months. METHODS: Of 175 consecutive patients with biopsy-proven GCA, 130 had typical cranial arteritis, 21 had silent vasculitis, and the remaining 24 had either discrete cranial symptoms (19 cases) or isolated extracranial vasculitis (5 cases). We sought to determine which of 15 pretreatment characteristics were associated with silent TA, as compared with typical cranial arteritis, and assessed the short term outcome in these patients. RESULTS: Of 21 patients with silent GCA, 14 met criteria for fever of unknown origin. Aside from their different clinical presentation, this population was characterized by a longer delay in diagnosis (p = 0.003), a higher mean erythrocyte sedimentation rate (p = 0.002), higher C-reactive protein (p = 0.002), and lower levels of albumin (p = 0.01) and hemoglobin (p < 0.0001). Permanent visual loss, which occurred in 24 patients (13.7%), exclusively involved those presenting with symptoms and/or signs suggesting cranial arteritis, especially those with frank cranial arteritis. This complication was associated negatively with the delay in diagnosis (p = 0.01), and marginally with the number of symptoms and/or signs suggesting cranial arteritis recorded in each patient (p = 0.07). Oral prednisone at a mean daily dose of 0.7 mg/kg resulted in satisfactory control of silent TA within 4 weeks in all patients but one, and could subsequently be safely tapered by half in a mean delay of 38 +/- 23 days. No differences were observed between patients with silent TA and other forms of the disease regarding the mean prednisone dose at 3 month followup (18.2 +/- 4.5 vs 20.9 +/- 5.9 mg/day) and 6 month followup (14 +/- 4.4 vs 15.6 +/- 6 mg/day ). CONCLUSION: Silent TA may represent a distinct subset of giant cell arteritis, marked by a protracted inflammatory response and a relatively benign short term outcome, excellent response to corticosteroids, and no visual ischemic events, despite the long period of exposure to this complication before appropriate treatment.

No evidence for a putative involvement of platelet-activating factor in systemic lupus erythematosus without active nephritis.

BACKGROUND: Platelet-activating factor (PAF) seems to be implicated in systemic lupus erythematosus (SLE) patients with associated renal diseases. AIMS: In this study, we ensured the role of PAF in SLE patients without renal complications. METHODS: Blood PAF and acetylhydrolase activity, plasma soluble phospholipase A(2), and the presence of antibodies against PAF were investigated in 17 SLE patients without active nephritis and in 17 healthy controls. RESULTS: Blood PAF levels were not different (p=0.45) between SLE patients (6.7+/-2.8 pg/ml) and healthy subjects (9.6+/-3.1 pg/ml). Plasma acetylhydrolase activity (the PAF-degrading enzyme) was significantly (p=0.03) elevated in SLE patients (57.8+/-6.4 nmol/min/ml) as compared with controls (37.9+/-2.6 nmol/min/ml). Plasma soluble phospholipase A(2) (the key enzyme for PAF formation) was not different (p=0.6) between SLE patients (59.1+/-5.1 U/ml) and controls (54.7+/-2.4 U/ml). Antibodies against PAF were detected only in 3/17 SLE patients. Flow cytometry analysis did not highlight PAF receptors on circulating leukocytes of SLE patients. CONCLUSION: This clinical study highlights no evidence for a putative important role of PAF in SLE patients without active nephritis.