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1.

Purpose

To report on the various clinical presentations, etiological diagnosis, prognosis and treatment of patients with scleritis evaluated at a tertiary care eye center.

Methods

Retrospective, monocentric study on a series of 32 patients in a tertiary center.

Results

The mean age of included patients with scleritis was 46.8 years (range, 22 to 77 years). Nineteen patients were women and 13 were men. Twenty-six patients (81%) had anterior scleritis (15 nodular, 8 diffuse and 3 necrotizing), six (19%) had posterior scleritis. Unilateral inflammation was present in 24 patients (75%). Twelve out of the 32 patients (37.5%) had an underlying systemic disease: granulomatosis with polyangiitis (n = 3), Behçet's disease (n = 2), unspecified inflammatory arthritis (n = 2), psoriatic arthritis (n = 1), ankylosing spondylitis (n = 1), sarcoidosis (n = 1), Cogan's syndrome (n = 1) and ulcerative colitis (n = 1). Six patients (18.8%) were suspected of having infectious disease with herpes virus: clinical context and positive treatment response with oral valacyclovir. Systemic agents and topical agents were required in 28 patients (87.5%). The first line therapy was mainly oral non-steroidal anti-inflammatory drugs in 15 patients (47%) and oral corticosteroids in 8 (25%). Immunosuppressive drugs were required in 6 patients. The mean follow-up was 16.3 months. Six patients (19%) had a decrease in visual acuity.

Conclusion

The number of systemic disease in our series is similar to the main series in the literature. Treatment with valaciclovir might be effective in patients with suspected herpes simplex scleritis.  相似文献   

2.

Purpose

Granulomatous interstitial nephritis (GIN) are identified in 0.5 to 1,3% of all renal biopsies. Renal outcome and treatment modalities are not clearly established in the literature.

Methods

We retrospectively analyzed a case series of 44 GIN identified among all renal biopsies performed between 1984 and 2005 in the Rhône-Alpes area.

Results

The study population included 25 men and 19 women with a mean age of 56 years, and mean diagnostic delay was 11 months. Renal function was severely impaired (mean creatinine clearance 24 mL/min). Proteinuria was observed in 77% (mean value 0,9 g/24 h) of the patients and associated with microscopic hematuria and leukocyturia in 30% and 25%, respectively. The most common diagnosis was sarcoidosis (25%, n = 11), followed by drug-induced GIN (9%, n = 4), tuberculosis (6,8%, n=3), hemopathy-related paraneoplastic GIN (6,8%, n = 3), HIV infection (n = 1) and chronic renal allograft rejection (n = 1). In other patients, no aetiology was found (48%, n = 21). Severity of renal failure justified hemodialysis in 34% (n = 15) of the patients. Three patients underwent renal transplantation. Nonetheless, renal outcome was generally favorable: renal function improved in 41% (n = 18) and stabilized in 34% (n = 15) of patients.

Conclusions

Sarcoidosis, drug-induced and infections represent the main causes of GIN. Histologic features are not specific enough to determine the aetiology. Corticosteroids is the gold standard in sarcoidosis, drug-induced, and idiopathic GIN. Treatment is etiologic in the other cases.  相似文献   

3.

Background

In this study, we examine the effect of previous percutaneous intervention on the rate of adverse perioperative outcome in patients undergoing coronary artery bypass graft surgery (CABG).

Methods

Outcomes of 240 CABG patients, collected consecutively in an observational study, were compared. Gp A (n = 35) had prior PCI before CABG and Gp B (n = 205) underwent primary CABG.

Results

Statistically significant results were obtained for the following preoperative criteria: previous myocardial infarction: 48.6% vs 36.6% (P = 0.003), distribution of CAD (P = 0.0001), unstable angina: 45.7% vs 39% (P = 0.04). For intraoperative data, the total number of established bypasses was 2.6 (GpA) vs 2.07 (Gp B) (P = 0.017), with the number of arterial bypass grafts being: 20% vs 13% (P = ns). Regarding the postoperative course, no significant difference in troponine I rate, 24-hour bleeding: 962 ml (Gp A) vs 798 ml (Gp B) (P = 0.004), transfusion (PRBC unit): 3.63 (Gp A) vs 2.5 (Gp B) (P = 0.006). Previous PCI emerged as an independent predictor of postoperative in-hospital mortality (OR 2.24, 95% CI [1.52–2.75], P < 0.01).

Conclusion

Patients with prior PCI presented for CABG with more severe CAD. Thirty-day mortality and morbidity were significantly higher in patients with prior PCI.  相似文献   

4.
5.

Purpose

Infectious aortic aneurysms are rare, being responsible of less than 3% of aortic aneurysms. In this study, we report the clinical characteristics and the outcome of patients presenting with infectious aortic aneurysms in an internal medicine unit.

Methods

Diagnosis of infection-related aortic aneurysm was obtained using: computed tomographic scan; and blood cultures, cultures and molecular biology testing of aortic wall and intra-aneurismal thrombus.

Results

The 10 consecutive patients included in this study consisted in eight men and two women with a mean age of 61.7 years. Patients presented with fever (n = 9), asthenia (n = 2), abdominal (n = 4) or chest pain (n = 1), lumbar pain (n = 3). Computed tomographic scan showed aneurysm involving both thoracic and abdominal aorta (n = 1), abdominal (n = 8) or thoracic aorta (n = 1). Isolated microorganisms were: positive Gram cocci (70%) and negative Gram bacilli (30%). All patients underwent both medical and surgical therapy. Outcome was favorable in nine patients; the remaining patient died from aneurismal aortic rupture.

Conclusion

Clinical manifestations revealing infectious aortic aneurysms are variable, including aneurysm rupture as well as atypical abdominal pain with inflammatory syndrome. These latter presentations are more common in patients, who are hospitalized in internal medicine. Our study underlines that this clinical pattern should not be ignored, in order to avoid both diagnostic and therapeutic delay that could lead to life-threatening complications and poor prognosis.  相似文献   

6.

Introduction

Renal involvement is uncommon in sarcoidosis, occurring in less than 5% of the patients. Diagnostic delay should be minimal to improve the outcome.

Methods

From 1996 to 2009, 78 patients were seen for sarcoidosis in the Department of Internal Medicine of Rouen University hospital.

Results

Five patients (6.4%) had renal involvement. Diagnosis of renal involvement and sarcoidosis were concomitant in two patients while in the three remaining patients, renal manifestations occurred during the course of sarcoidosis. The five patients with renal manifestations exhibited: isolated sarcoid granulomatous interstitial nephritis (n = 2), sarcoid granulomatous interstitial nephritis and nephrocalcinosis (n = 2), renal failure due to hypercalcemia (n = 1).

Conclusion

This series underlines that renal function tests should be performed systematically both during initial evaluation and the follow-up of patients with sarcoidosis.  相似文献   

7.

Purpose

Cardiac amyloidosis is rare. The objective of this study was to report on a case series of 14 patients with cardiac amyloidosis and to study the prognostic factors.

Methods

Monocentric retrospective study of all adult patients who presented with cardiac amyloidosis, diagnosed at the Georges-Pompidou European hospital in Paris between 2003 and 2011.

Results

Fourteen patients were identified (10 men and four women). Median age at diagnosis was 66.5 years. Twelve patients were diagnosed with AL amyloidosis, one with AA amyloidosis, and one with transthyretin amyloidosis. All patients presented cardiac manifestations: heart failure (n = 9), rhythm disorders (n = 6). Eight patients presented extra-cardiac manifestations of amyloidosis: renal (n = 8), gastrointestinal (n = 5). Troponin serum level was increased in eight patients and BNP level was superior to 400 pg/L in 12 patients. When performed, the cardiac magnetic resonance imaging (MRI) showed, in six patients out of seven, chamber dilatation, concentric hypertrophy or late enhancement. Among patients with cardiac failure at diagnosis (n = 9), seven died with a median survival of 1 month duration. Factors of poor prognosis were, in our study, heart failure, elevated levels of troponin and BNP, and the AL amyloidosis subtype.

Conclusion

Cardiac amyloidosis, especially the AL type, has a very poor prognosis, essentially because of an underlying multiple myeloma and heart failure.  相似文献   

8.

Purpose

The reported prevalence of cardiac complications is variable in patients with Churg-Strauss syndrome (15–92%) and depends on diagnostic tools. Diagnosis at early stage of heart involvement is crucial, resulting in appropriate management.

Methods

We report three patients who developed cardiac manifestations, revealing Churg-Strauss syndrome. The diagnosis of cardiac involvement was obtained using cardiac magnetic resonance imaging (MRI).

Results

Two patients were males and the remaining one was a female. Presenting clinical manifestations were: cardiac failure (n = 1) and retrosternal pain (n = 2). Laboratory findings disclosed: high blood count of eosinophils (range: 6000–11 000/mm3); antineutrophil cytoplasmic antibodies were positive in a single patient. Cardiac MRI demonstrated: (1) late gadolinium enhancement (n = 3), involving mainly the apical and mid-cavity left ventricular segments; (2) impaired left ventricular function (n = 2), mean left ventricular ejection fraction being: 51%; and (3) pericardial effusion (n = 3). Outcome was favourable after institution of combined therapy with prednisone and cyclophosphamide (n = 2); one patient also underwent plasma exchanges.

Conclusion

Our case series underlines that MRI is a helpful tool in the diagnosis of Churg-Strauss syndrome-related cardiac complications. We further suggest that clinical assessment of patients with Churg-Strauss syndrome should include cardiac MRI, in order to detect cardiac involvement at an early stage; indeed, because cardiac manifestations are predictive factors of poor prognosis, diagnosis at early stages of cardiac involvement may result in improvement of patients management.  相似文献   

9.

Objective

Evaluate the diagnostic and prognostic input of head-up tilt test in the exploration of unexplained syncope.

Method

Between January 2009 and December 2012, all patients undergoing a head-up tilt test for recurrent syncope were studied. Follow-up data were obtained using telephone interviews and medical record reviews.

Results

A head-up tilt test was realized in 77 patients (47.8 ± 20 years, 53% female) for an exploration of syncope. The tilt test elicited syncope or pre-syncope in 57 patients (74%). The positive response included vaso-vagal syncope in 53 patients and psychogenic syncope in 4 patients. After a mean follow-up of 32 ± 11 months (range 6–54 months), 90% of patients had not a recurrence of syncope. Of note, the incidence of recurrence was the same regardless of whether the patients had a positive (n = 5/48; 10%) or a negative head-up tilt test response (n = 2/19; 10%).

Conclusion

The tilt test has a certain diagnostic value in the exploration of unexplained syncope. Recurrence rate of syncope after a tilt test is low. However, our study suggests no correlation between head-up tilt test results and the likelihood of recurring syncope.  相似文献   

10.

Purpose

The objective of this study was to determine the clinical relevance and the diagnostic significance of positive antinuclear antibodies (ANA) without identified antigenic target by the usual characterization technique.

Patients and methods

Retrospective study conducted in the Laboratory of Immunology of Habib Bourguiba Hospital (Sfax, Tunisia) during 18 months. The inclusion criteria were the presence of an ANA titer greater or equal to 1/320 with negative characterization result. ANA screening was performed by indirect immunofluorescence (IIF) on Hep2 cells. Each positive serum was tested by IIF on Crithidia luciliae (anti-native DNA) and by immunodot (anti-nucleosome, anti-histone, anti-Sm, anti-RNP, anti-SSA, anti-SSB, anti-Scl 70, anti-PM-Scl, anti-Jo1, anti-PCNA and anti-ribosomal protein). Sera of systemic lupus erythematosus (SLE), myositis, and scleroderma patients were tested for anti-Ku, anti-PL7, anti-PL12 and anti-Ro-52 using dot myositis.

Results

Sera of 90 patients were studied: 18 men and 72 women (average age: 44 years). Drug-induced ANA was found in eight patients. The most frequent clinical symptoms were joint (56.7%), cutaneous (54.4%) and constitutional symptoms (45.6%). The diagnosis of an autoimmune disease was suspected in 49 patients (54.5%) and confirmed in 30 (33.3%) including 20 cases of connective tissue disease: myositis (n = 6), scleroderma (n = 5), Sjögren's syndrome (n = 3), SLE (n = 4), rheumatoid arthritis (n = 6) and antiphospholipid syndrome (n = 4). Other autoimmune diseases were less frequent. The anti-Ku antibody was detected in the majority of patients with connective tissue disease. The diagnosis of non-autoimmune diseases was established in 25.5% of patients. Eighteen patients (20%) had no diagnosis orientation.

Conclusion

Our study demonstrated the diagnostic value of the presence of ANA even in the absence of known antigenic target, confirmed the role of the IIF as “gold standard” test for ANA screening, and suggested the usefulness of the addition of Ku antigen in the immunodot classic profile.  相似文献   

11.

Purpose

Retroperitoneal fibrosis (RPF) is a rare disease with an expanding etiologic spectrum. We aimed to analyze non-invasive diagnosis strategy, associated disorders, monitoring, treatment and prognosis.

Methods

Retrospective cohort study in a single tertiary center.

Results

Eighteen RPF cases (11 males) followed between 1996 and 2009 were reviewed. Blood CRP level was high in all cases before treatment. CT scan, associated or not with MRI or 18-FDG PET-scan, confirmed the diagnosis in 15 patients. Histological analysis of a surgical biopsy specimen was performed in only three cases. Ten patients suffered retroperitoneal fibrosis secondary to systemic vasculitis (granulomatosis with polyangeitis, n = 1, Takayasu aortitis, n = 2), systemic fibrosis with Riedel thyroiditis (n = 1) and atheromatous periaortitis (n = 6). Fifteen patients were treated with corticosteroids with a mean treatment duration of 60 months (12–228). Dependency to corticosteroids was recorded in ten patients. Patients with fibrosis related to vasculitis were younger, had a higher CRP level, more frequent corticosteroid dependency and a higher relapse rate. Relapses were successfully treated with steroids. Immunosuppressive treatment was only prescribed in the setting of systemic vasculitis. No patient died, after a 6 ± 2 years follow-up. Late relapses could occur, sometimes years after steroid therapy cessation.

Conclusion

In our study, RPF occurred as a secondary disorder in 60% of the cases. Disease extension, relapse rate and treatment response varied according to the underlying cause of RPF, pleading for an extensive and systematic initial assessment. Since no death or end-stage renal insufficiency was observed, RPF might be considered as a steroid-sensitive and benign disorder.  相似文献   

12.

Purpose

The occurrence of acute pancreatitis in systemic lupus erythematosus (SLE) is known but rare, and is exceptionally the presenting manifestation. Its pathogenesis is multifactorial, and it is difficult to separate what belongs to vasculitis, thrombotic phenomena in the context of an associated antiphospholipid syndrome, or iatrogenic complications. We report on six cases of lupus pancreatitis.

Methods

This is a retrospective monocenter study of 110 patients with SLE. The diagnosis of lupus pancreatitis was established after exclusion of other causes of pancreatitis.

Results

Five women and one man (5.4%) with a mean age of 36.3 years presented with lupus pancreatitis. In four patients the pancreatitis was concurrent with the diagnosis of SLE and it occurred later during an exacerbation of the disease in the two remaining patients. In all patients, pancreatic manifestations were associated with other organ involvement. Clinical manifestations were: abdominal pain (n = 6), vomiting (n = 3), and fever (n = 3). Elevated pancreatic enzyme was noted in all cases. All patients were treated by high doses of glucocorticoids. The outcome was favorable in five patients, and one patient died.

Conclusion

Pancreatitis may be the presenting manifestation of SLE. Its pathogenesis is often multifactorial. The outcome is usually favorable with corticosteroids.  相似文献   

13.

Purpose

Heart failure is a common disease and its progression to end-stage heart failure is responsible of high mortality. The aim of this retrospective study was to assess the access to integrated palliative care to the usual management, 6 months prior to their death, and especially during the last hospitalization.

Patients and methods

A retrospective study was performed in patients who died of heart failure in 2009 in two hospitals. The analysis was performed on 20 cases of each institution. The records of consecutive patients were included in an anti-chronological order from 31st December 2009.

Results

For their last hospitalization, 37 patients (93%) were hospitalized in emergency. Within 3 days prior to death, the most frequent symptoms were dyspnea (n = 33, 82%), and pain (n = 30, 75%). Therapeutic most frequently used were oxygen (n = 31, 77%) and analgesics (n = 30, 75%). No patient was seen by a psychologist. The decision to limit treatment for comfort care was reported for 24 patients (60%) and the median of the average time between the decision and death was 2 days (Q1–Q3, 1–5 days).

Conclusion

Patients with terminal heart failure have many symptoms often requiring multidisciplinary care. This type of study relating practices shows that there is still a lot to do to integrate palliative care in the usual management of patients with heart failure.  相似文献   

14.
15.

Background and aims

The International Autoimmune Hepatitis Group developed a simplified score for autoimmune hepatitis. We assessed this “new scoring system” and compared it with the International Autoimmune Hepatitis Group original revised score.

Methods

502 patients were evaluated namely, 428 had liver diseases of various etiology [hepatitis B (n = 109), hepatitis C (n = 100), hepatitis D (n = 4), alcoholic liver disease (n = 28), non-alcoholic fatty liver disease (n = 55), autoimmune cholestatic diseases (n = 77), liver disorders of undefined origin (n = 32) and miscellaneous hepatic disorders (n = 23)], 13 had autoimmune hepatitis/overlap syndromes, 18 had autoimmune hepatitis/concurrent with other liver diseases and 43 had autoimmune hepatitis.

Results

The specificity of the simplified score was similar to that of the revised score (97% vs. 97.9%). The sensitivity in unmasking autoimmune hepatitis in autoimmune hepatitis/overlap syndromes was also similar in both systems (53.8% and 61.5%). However, the sensitivity for autoimmune hepatitis diagnosis in autoimmune hepatitis patients with concurrent liver disorders was lower by the new score (p = 0.001). Liver biopsy proved to be the only independent factor for unmasking autoimmune hepatitis component among patients (p = 0.003).

Conclusion

The simplified score is a reliable and simple tool for excluding autoimmune hepatitis. However, both systems cannot unmask autoimmune hepatitis component efficiently in autoimmune hepatitis patients with concurrent autoimmune or non-autoimmune liver diseases. This study also strongly reiterates the importance of liver biopsy in the work-up of patients.  相似文献   

16.

Aim

We report the first experience of Lyon's university hospital regarding renal denervation to treat patients with resistant essential hypertension.

Patients and methods

Over a one-year period, 17 patients were treated (12 men, 5 women) with renal denervation. Baseline characteristics were as follows: age 56.5 ± 11.5 years, BMI 33 ± 5 kg/m2 and ambulatory blood pressure 157 ± 16/87 ± 13 mmHg with 4.2 ± 1.5 anti-hypertensive treatment.

Results

We did not observe per procedural and early complications. After a median follow-up of 3 months and with the same anti-hypertensive treatment, office systolic blood pressure (SBP) and diastolic blood pressure (DBP) decrease respectively of 20 ± 15 (P < 0.001) and 10 ± 13 mmHg (P = 0.014) (n = 17). After six months of follow-up, ambulatory blood pressure (ABPM) decrease of 17.5 ± 14.9 mmHg (P = 0.027) for SBP and of 10.5 ± 9.6 mmHg (P = 0.029) for DBP (n = 6). Among these patients, five of them were controlled (ABPM inferior to 130/80 mmHg) and electrical left ventricular hypertrophy indexes decreased: R wave in aVL lead of 4 ± 3 mm (P = 0.031), Sokolow index of 3 ± 3 mm (P = 0.205), Cornell voltage criterion of 9 ± 7 mm (P = 0.027) and Cornell product of 1310 ± 1104 (P = 0.027).

Conclusion

Our results are in accordance with data from other centers. On average blood pressure decreases significantly but important inter individual variations are observed. The procedure seems safe.  相似文献   

17.

Purpose

Idiopathic retroperitoneal fibrosis (IRF) is an inflammatory disorder, affecting the aorta and the surrounding vessels and tissues. The prognosis is mainly driven by the risks of chronic kidney disease and relapse. Our aim was to assess the prevalence of chronic kidney disease at follow-up.

Methods

We retrospectively reviewed the medical records of patients diagnosed for IRF in Seine-Saint-Denis (France) between 1987 and 2011. We collected informations about presentation, radiologic findings and follow-up. Diagnosis of IRF was confirmed when all the following criteria were met: infiltration of the infrarenal aorta or iliac vessels, absence of aneurysmal dilation, lack of clinical suspicion of malignancy.

Results

Thirty patients were identified, with a male/female ratio of 4.9. Mean age was 55 ± 13 years old. The mean creatinine clearance was 66 mL/min/1.73 m2 and the mean CRP was 45 ± 36 mg/L. In 24 (80%) patients, the location of IRF was periaortic and periiliac. Eleven patients (37%) underwent a diagnostic biopsy, and 14 (47%) required an ureteral procedure. A mean follow-up of 63 months was available for 29 patients: 69% relapsed, 7 developed chronic renal disease (24%), and one died of urinary sepsis. Older age (P = 0.023), diabetes (P = 0.007), and initial renal insufficiency (P = 0.05) were associated with a risk of chronic renal insufficiency.

Conclusion

The high frequency of relapses and chronic renal disease emphasizes the need of close follow-up in patients diagnosed with IRF.  相似文献   

18.
19.

Purpose

To date only a few studies regarding pulmonary embolism (PE) in elderly have been published. The aim of this study was to determine the clinical features of PE in elderly patients (≥ 75 years).

Methods

All patients hospitalized for PE in our internal medicine department from January 2005 to December 2010 were included in the study. The aim was to compare the features of PE in elderly patients (≥ 75 years) to those of patients younger than 75 years. The following data were recorded: past medical history, risk factors for venous thrombo-embolism (VTE), clinical features, and PE etiologies.

Results

The population was composed of 64 patients (women 56%) with a median age of 82 years (IQR: 13.5). There was no statistical difference for risk factors of VTE. Syncope was more frequent in elderly patients (33% versus 7%, P = 0.04) whereas thoracic pain predominated in younger patients (36,5% versus 7%, P = 0.005). Chronic obstructive pulmonary disease was more frequent in the past medical history of elderly patients. The diagnostic of PE was less suspected in elderly patients (47% versus 72%, P = 0.035). The etiologies were similar between the two groups.

Conclusion

Our study highlights the frequency of syncope as the presenting feature of PE in elderly, whereas thoracic pain is uncommon. We confirmed the difficulty to diagnose PE in elderly population.  相似文献   

20.

Purpose

Infectious aortic aneurysms are rare conditions, being responsible of 2% of aortic aneurysms. Most published results are surgical case series concerning infected abdominal aorta. In this retrospective study, we assessed clinical features and outcome of patients presenting infectious thoracic aortic aneurysms.

Patients and methods

Diagnosis was based upon a combination of imaging evidence for thoracic aorta aneurysm and evidence for an infective aetiology including a culture of a causative pathogen, or a favourable outcome with anti-infective therapy. Retrospective case series.

Results

Six men and one woman were included, with a mean age of 66 years. All the patient presented at least one cardiovascular risk factor or atherosclerosis localisation. Fever (71%) and chest pain (42%) were the most common clinical presenting manifestations. The causative pathogens were: Staphylococcus aureus (N = 1), Salmonella enteritidis (N = 3) and Candida albicans (N = 1). The contrast-enhanced computed-tomography disclosed an aneurysm whose diameter reached more than 50 mm (N = 5), that increased rapidly in size (N = 5), or presented an inflammatory aspect of the aortic wall (N = 4). Management was both medical and interventional: surgery (N = 3) or endoluminal repair (N = 4). Outcome was favourable in six patients; one patient died from aneurysm-related complications.

Conclusion

Clinical manifestations revealing an infectious thoracic aneurysm are variable. Diagnosis should be considered in patients presenting a rapidly-growing aneurysm, especially in the presence of elevated acute phase reactants. Endoluminal repair constitutes a treatment option. The role of FDG-PET for diagnosis and follow-up remains to be defined.  相似文献   

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