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Biomedical subjects

Eric Liozon

Publications and source records attributed to Eric Liozon.

17 recordsLinked to original sources

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.

Aged↗

Generalized eczema craquele as a presenting feature of systemic lymphoma: report of seven cases.

Eczema craquele, or asteatotic eczema, has been associated with malignant lymphoma although this is rare. Since 1986, we have observed seven patients, six men and one woman, mean age 71.5 years (range 43-86 years), with systemic lymphoma and concurrent eczema craquele. Five patients had T-cell lymphoma, one had a B-cell lymphoma and one had Hodgkin's disease. All patients shared several characteristics: (1) a synchronous onset of eczema craquele and lymphoma, (2) generalized eczema, (3) absence of alternative disease or conditions that could favour the onset of eczema craquele, and (4) eczema refractory to topical corticosteroids and emollients, but which resolved upon lymphoma remission and invariably recurred with the lymphoma relapse. All the patients except one died within 1 year, most with active lymphoma. The finding of recalcitrant generalized eczema craquele should prompt a search for lymphoma, particularly in older men. Lymphoma-associated eczema craquele has most characteristics of paraneoplastic syndromes and may be a hallmark of aggressive lymphoma.

Adult↗

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.

1-Alkyl-2-acetylglycerophosphocholine Esterase↗

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.

Acute Disease↗

[Management of intermittent fever in the adult].

Episodic fever is a difficult diagnostic challenge, more enigmatic than prolonged fever of unknown origin. Patients are younger, and have longer history of fever before admission. Despite the use of extensive diagnostic procedures 50% of the cases remain undiagnosed. A meticulous physical examination and history remains an important tool for registering potentially diagnostic clues useable in the diagnostic process. In the absence of clues, a staged diagnostic protocol can be used to search elements contributing to the diagnosis (laboratory investigations including chest X-ray and ultrasonography of upper abdomen, imaging techniques, endoscopies and histologic investigations. When diagnosis is not established and patient deteriorate, empiric therapeutic trial is started to presumptive diagnoses. If not, patient must be carefully followed as an out patient basis and symptomatically treated, periodic reassessment will in time reveal the development of overt disease.

Adult↗

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.

Adult↗

Neurologic presentation of Whipple disease: report of 12 cases and review of the literature.

We report 12 cases of Whipple disease in patients with prominent neurologic symptoms, along with 122 cases of Whipple disease with nervous system involvement reported in the literature. We analyzed the clinical signs and results of additional examinations in 2 groups: the first group included patients with predominantly but not exclusively neurologic signs, and the second included patients with clinically isolated neurologic presentation of the disease. Whipple disease is a multisystemic infectious disease due to Tropheryma whippelii that may present with prominent or isolated symptoms of either the central or the peripheral nervous system. Recent reports stress the importance of polymerase chain reaction (PCR) analysis of cerebrospinal fluid, magnetic resonance imaging (MRI) during follow-up, and prolonged antibiotic therapy with drugs able to cross the blood-brain barrier. Cerebrospinal fluid should be analyzed repeatedly during follow-up, and treatment should be discontinued only when the results of PCR assay performed on cerebrospinal fluid are negative. Other examinations to be done include searching for gastrointestinal tract involvement with multiple duodenal biopsies and searching for systemic involvement with lymph node biopsies, which should be analyzed with light microscopy, electron microscopy, and PCR. When all examinations are negative, if Whipple disease is suspected and a lesion is found on brain MRI, a stereotactic cerebral biopsy should be performed. Treating Whipple disease with long-term trimethoprim-sulfamethoxazole is usually effective, but the use of third-generation cephalosporins in case of incomplete response deserves further attention.

Actinomycetales Infections↗