Incidence, risk factors and skin manifestations of post-thrombotic syndrome: a four-year follow-up of patients included in the EDITH study

Purpose

Despite appropriate therapy 10 to 100% of patients with deep vein thrombosis (DVT) of the lower limbs will develop post-thrombotic syndrome (PTS). The aim of this study was to evaluate the incidence of PTS in the EDITH cohort and to estimate the association between initial patients’ characteristics and the risk of development of PTS.

Methods

One hundred and eighty patients included in the EDITH study for a first event of DVT of the lower limbs without clinical signs of venous insufficiency were recalled 4 years after their initial thrombotic event. PTS was diagnosed according to the Villalta score.

Results

Ninety-five patients (45 men, mean age 50.7 ± 16.9 years) were evaluated for PTS. Among them, 28.4% (95% CI 19.3–37.5) developed PTS but none had severe PTS. The most frequent clinical signs of PTS were varicose veins (59%), corona phlebectatica (48%), swelling leg (30%) and pigmented dermatitis (26%). No single risk factor was associated with PTS development (age, sex, BMI thrombophilia, etiology, localization, recurrence, symptomatic DVT and familial history of DVT).

Conclusion

PTS is a frequent disease. However, lack of uniformity of diagnosis criteria in the different studies does not make possible the estimation of PTS risk factors.     

Étiologies et évolution des hépatites granulomateuses : étude rétrospective de 21 cas consécutifs

Purpose

To assess the etiologies and outcome of liver granulomatosis.

Methods

We analyzed all consecutive liver granulomatosis diagnosed in our internal medicine department from 2000 to 2008.

Results

Among 471 liver biopsies, 21 disclosed evidence of liver granulomatosis (4.5%), in sixteen women (76%) and five men, with a median age of 41 years. Thirteen were caucasians (62%). At the time of diagnosis, six (28.5%) had isolated abnormal liver function tests, and fifteen (71.4%) presented with clinical manifestations. The underlying cause was identified in 18 cases (85.7%). Eleven (52.3%) were systemic diseases: five (23.8%) primary biliary cirrhosis, two (9.5%) primary sclerosing cholangitis, two (9.5%) common variable immunodeficiency, one (4.7%) Sjögren's syndrome, and one (4.7%) Behçet's disease. Two (9.5%) patients had sarcoidosis. Three (14.3%) liver granulomatosis were of infectious origin (tuberculosis, schistosomiasis, and hepatitis C virus), two (9.5%) were neoplastic (Hodgkin's lymphoma and liver cell adenoma), and three (14.3%) were idiopathic. With a median of 38 months of follow-up, four patients (19%, two common variable immunodeficiency and two sarcoidosis) developed portal hypertension, independently of cirrhosis. One patient died of cryptococcosis.

Conclusion

In accordance with other European studies, systemic diseases are the main causes of hepatic granulomas. Liver granulomatosis related to common variable immunodeficiency and sarcoidosis are at risk of portal hypertension.     

Atteintes cardiovasculaires de la maladie de Behçet

Vascular involvement is a common complication of Behçet's disease (BD) and affects up to 40% of BD patients. These complications worsen the prognosis of BD. The concept of vasculo-Behçet has been adopted for cases in which vascular complications dominate the clinical features. Vascular manifestations affect particularly young men, during the first years following onset of the disease. Venous complications are the most frequent vascular complications, affecting 14 to 40% of BD patients. Superficial and deep lower limb thrombosis is the most frequent venous complications but one third of venous thrombosis concern large vessels (such as cerebral venous thrombosis, pulmonary embolism, and inferior or superior vena cava, etc.). Budd-Chiari syndrome is the worst prognostic factor increasing mortality by 9 times. Arterial complications (2 to 17% of BD patients) include aneurysms and occlusions/stenosis. Main locations of arterial lesions are aortic (abdominal and thoracic), femoral, pulmonary and iliac arteries. Aneurysms are the most severe arterial complications, particularly pulmonary aneurysms associated with a high risk of massive bleeding. Cardiac complications (up to 6% of BD patients) include pericarditis, endocardial lesions (aortic regurgitation and less often mitral insufficiency), myocardial lesions (myocardial infarction, myocarditis and endomyocardial fibrosis) and intracardiac thrombosis (right ventricle and atrium). Coronary lesions complicated to myocardial infarction are the most severe cardiac complications. Treatment is based on corticosteroids and immunosuppressive drugs. The use of anticoagulation in venous thrombosis is still controversial.     

Arthrite érosive du poignet : une manifestation rare de la maladie de Behçet

It is believed that arthritis in Behçet's disease is usually non erosive and not associated with crippling. We report a 38-year-old female who presented with Behçet's disease and an erosive arthritis of the left wrist.     

Validation of a clinical prediction rule for the diagnosis of deep vein thrombosis of the lower limbs in primary care

Purpose

Patients with suspected deep vein thrombosis (DVT) are often managed on an outpatient basis. The aim of the study was to validate a clinical prediction rule specifically for use in primary care to help physicians in their decision to start anticoagulant therapy while awaiting ultrasound examination.

Patients and methods

Between September 2007 and October 2008, 194 general practitioners prospectively included patients with clinically suspected DVT without clinically suspected pulmonary embolism. All patients underwent a standardized clinical assessment in order to collect items included in the clinical prediction rule (personal history of venous thromboembolism +1, immobilization in previous month +1, estrogen contraceptive +2, active malignancy +3, swelling of the calf +1, the presence of an alternative diagnosis more likely than that of DVT–3. DVT unlikely if score < 2, likely if score ≥ 2).

Results

Among the 164 included patients, 56 (34%) had DVT of them 28 (17%) had a proximal DVT. Proportions of confirmed DVT were 29% in the unlikely group and 43% in the likely group against 26% and 63% respectively in the derivation study.

Conclusions

This clinical prediction rule might not fulfill the required conditions to be considered as a usable help in the ambulatory management of DVT. Variations of the cut-off value could enhance its performance.     

Épidémiologie des thromboses veineuses des membres supérieurs : étude rétrospective de 160 thromboses aiguës

Aim

To describe the clinical features and etiologies of upper limb venous thrombosis (ULVT).

Syndrome de Sweet : étude rétrospective de 47 cas

Purpose

Also called acute febrile neutrophilic dermatosis, Sweet's syndrome is an inflammatory disorder with a prominent cutaneous expression. It is characterized by a variety of manifestations, clinical and histological findings. The objective of this study was to describe their clinical, pathological and therapeutic characteristics.

Methods

We report on a series of 47 patients who presented a Sweet's syndrome, collected in our institution in Tunis between 1997 and 2011.

Results

The patient population consisted of 11 men and 36 women. The mean age was 47 years with extreme ranging from 28 to 74 years. An associated disorder was observed in ten patients: inflammatory disease (three cases), inflammatory bowel disease (two cases), tuberculosis (three cases) and diabetes (three cases). One case of pregnancy was observed. Cutaneous lesions consisted of erythematous plaques or nodules. Lesions were located mainly on the upper or lower extremities. All biopsy specimens demonstrated a dermal infiltrate composed predominantly of neutrophils. Fibrinoid necrosis and intramural inflammation were observed in eight cases.

Conclusion

The skin disorder can precede, follow, or appear concurrent with the diagnosis of an associated disease which requires careful surveillance.     

Implantación de endoprótesis para el tratamiento del síndrome de vena cava superior maligno: serie de 56 pacientes de un solo centro

Objective

To report a series of stenting procedures for the treatment of malignant superior vena cava (SVC) syndrome.

Material and methods

A review conducted from October 2005 to July 2013 retrieved 56 consecutive patients treated for symptomatic malignant SVC syndrome with stenting.

Results

SVC stenting was attempted in 56 patients (46 males, 10 females), aged 34-84 years (mean 59.3).The success rate was 49/57 (86%). Success was associated with the type of obstruction classified as: group 1 (a —SVC stenosis, or b —unilateral innominate vein occlusion with contralateral innominate vein stenosis and normal SVC), group 2 (SVC occlusion excluding bilateral innominate vein occlusion) and group 3 (bilateral innominate vein occlusion irrespective of SVC status). Success rates were 100% (39/39), 75% (9/12) and 16.6% (1/6), respectively. These differences were significant for group 1 versus group 2 + 3 (p < 0.001) and for group 2 versus group 3 (p = 0.032). Acute complications occurred in 9 patients. Patients in whom acute complications occurred were older than the others (67.8 vs. 57.6 years, p = 0.019). The procedure-related death rate was 3.5% (n = 2). Stent occlusion occurred in 3.5% (n = 2). The patient survival was poor (median 2.6; range < 1-29.6 months), independently of the success of stenting.

Conclusions

Stenting for malignant SVC syndrome provides immediate and sustained symptomatic relief that lasts until death in this set of patients with a short life expectancy and restores the central venous access for administration of chemotherapy. Technical failure was associated with SVC occlusions and primarily with bilateral innominate vein occlusion.     

Riesgo de fenómenos tromboembólicos en pacientes con enfermedad inflamatoria intestinal

Introduction

Inflammatory bowel disease (IBD) is an independent risk factor for thromboembolic phenomena (TEP). We evaluated the prevalence and the possible risk factors associated with developing TEP in patients with IBD in our center.

Material and methods

Data were retrospectively collected from January 1995 to December 2011 from 23 patients. A total of 61% were diagnosed with Crohn's disease (CD) and 39% with ulcerative colitis (UC) according to routine criteria.

Results

When the Montreal classification was used, 58% of the patients with CD had an inflammatory pattern (B1), 25% a stenosing pattern (B2) and 17% a fistulizing pattern (B3). Half the patients had ileocolic involvement (L3), one-third had colonic involvement (L2) and the remainder had ileal involvement (L1). Among patients with UC according to the Montreal classification, 78% had extensive colitis (E3), 11% had left colonic involvement (E2) and 11% had proctocolitis (E1). During the event, almost half the patients with UC had severe inflammatory activity (S3; 44%), 33% had mild-moderate activity (S1: 22%, S2: 11%) and only 22% were in remission (S0). Overall, at the time of the TEP, 48% of the patients had mild-moderate activity and 22% had severe activity. Likewise, 44% were hospitalized at the time of the event. In UC, an increase in the prevalence of TEP was found in admitted patients (66%). None of the patients had a family history of TEP, two patients (9%) had associated thrombophilia and 26% were active smokers. There were no TEP during pregnancy. Only one patient was taking contraceptive pills when the event occurred. The most frequent forms of TEP were deep vein thrombosis of the legs (55%) followed by pulmonary thromboembolism (25%).

Conclusions

TEP are relatively frequent in patients with IBD, with a strong impact on morbidity and mortality. In our series, risk factors for these events were more extensive involvement (any of the groups) and severe inflammatory activity. No significant association between classical risk factors such as the use of contraceptives, pregnancy, coagulation disorders or smoking and the risk of TEP were found.     

La maladie de Behçet chez la femme

Purpose

Behçet's disease (BD) is a multisystemic inflammatory disease characterized, on the one hand, by the frequency of benign mucocutaneous and articular manifestations, and on the other hand by the severity of ocular, neurological, vascular and digestive manifestations which may threaten functional or vital prognosis. The male BD predominance is obvious in many retrospective studies and a few studies only have focused on BD in women.

Methods

In this study, we report the epidemiological, clinical, outcome and therapeutic data of a series of 71 female BD compared to a series of 111 male BD. This retrospective study was conducted in the internal medicine department of Monastir university hospital over a 19-year period (1990 to 2008), including all patients followed-up and treated for BD.

Results

The comparison of clinical manifestations of BD between men and women showed the predominance of erythema nodosum (P = 0.016), necrotic pseudofolliculitis (P = 0.001), and joint involvement (P = 0.009) in women, while genital ulcers (P = 0.039), ocular involvement (P = 0.003) and vascular (P = 0.002) involvement were more common in men.

Conclusion

Our results confirmed the results of previous studies regarding the benign nature of BD in women compared to men.     

Sclérites,aspects cliniques, étiologiques et thérapeutiques : à propos d’une série de 32 observations

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.     

Thromboses veineuses profondes distales isolées des membres inférieurs : épidémiologie et prise en charge

Isolated distal deep-vein thromboses (DVT) are infra-popliteal DVT without involvement of proximal veins or pulmonary embolism (PE). They can affect deep calf (tibial anterior, tibial posterior, or peroneal) or muscular (gastrocnemius or soleal) veins. They represent half of all lower limbs DVT. Proximal and distal DVTs differ in terms of risk factor profile, proximal DVT being more frequently associated with chronic risk factors and distal DVT with transient ones. Their natural history (rate of spontaneous proximal extension) is debated leading to uncertainties on the need to diagnose and treat them with anticoagulant drugs. In the long term, the risk of venous thromboembolic recurrence is lower than that of proximal DVT and their absolute risk of post-thrombotic syndrome is unknown. French national guidelines suggest treating with anticoagulants for 6 weeks a first episode of isolated distal DVT provoked by a transient risk factor and treating for at least 3 months unprovoked or recurrent or active cancer-related distal DVT. The use of compression stockings use is suggested in case of deep calf vein thrombosis. Ongoing therapeutic trials should provide important data necessary to establish an evidence-based mode of care, especially about the need to treat distal DVT at low risk of extension with anticoagulants.     

Delirium tremens : étude rétrospective de 39 observations

Introduction

Alcohol withdrawal delirium (AWD) is one of the most serious complications of alcohol withdrawal. The daily practice of linking activity in addiction shows that this condition is not uncommon.

Patients and methods

We extracted from the hospital database patients’ admissions for which a diagnosis of AWD (F104) was registered between 2004 and 2009. The review of discharge reports allowed to select the patients for which the medical records were analyzed.

Results

Thirty-nine patients admitted for DT were selected. Mean age was 48 years (male 89.7%). A majority of patients (89.7%) were admitted through the emergency department. In about a half of cases (51.3%), blood alcohol concentration was null at admission. The main reasons for the admission were alcohol withdrawal syndrome (17.9%), fall/malaise (15.4%), and generalized seizures (12.8%). Approximately one-third of the patients had a medical history of seizures and 23.1% of AWD. No patient had severe liver failure. Six patients (15.4%) were transferred to intensive care. Two patients developed AWD after surgery. No deaths have been observed.

Conclusion

Improving medical knowledge of AWD is a way to make this complication even more uncommon.     

Maladie de Behçet de l’enfant

Behçet's disease is a chronic multisystem vasculitis of unknown etiology. The disease is commonly described in young adults but can occur in childhood. Diagnosis is based on clinical manifestations since there are no pathognomonic laboratory findings. Early diagnosis in children is challenging due to the insidious nature of the disease and the low sensitivity of adult criteria in the pediatric population. The purpose of this review is to describe the demographic and clinical features of Behçet's disease in childhood, its complications and recent advances in therapeutics highlighting differences with the adult onset disease.     

Immunosuppressants reduce venous thrombosis relapse in Behçet's disease

Objective

To investigate and describe the long‐term outcome of venous thrombosis in patients with Behçet's disease (BD).

Methods

In a retrospective cohort of 807 BD patients, a reported 296 patients (36.7%) (73.3% male, median age 30 years [interquartile range 24–36 years]) met the international classification criteria for BD and had venous thrombosis. We assessed factors associated with thrombosis relapse and mortality.

Results

There were a total of 586 venous thrombosis events, including 560 cases of deep thrombosis and 26 cases of superficial thrombosis. Deep venous thrombosis events included 323 cases of limb thrombosis (55.1%), 77 cases of cerebral venous thrombosis (13.1%), 57 cases of pulmonary embolism (9.7%), 63 cases of vena cava lesions (10.7%), 14 cases of Budd‐Chiari syndrome (2.4%), and 13 cases of cervical vein thrombosis (2.2%). One hundred of 296 patients (33.8%) experienced at least 1 venous thrombosis relapse. The mortality rate was 6.4% (19 of 296 patients) after a median followup of 4.75 years (interquartile range 2–7 years). In univariate analysis, death was associated with cardiac involvement (P = 0.026) and Budd‐Chiari syndrome (P = 0.004). In multivariate analysis, the use of immunosuppressive agents was found to prevent relapse of venous thrombosis (hazard ratio 0.27 [95% confidence interval 0.14–0.52], P = 0.00021), and there was a trend toward prevention of relapse with the use of glucocorticoids (hazard ratio 0.62 [95% confidence interval 0.40–0.97], P = 0.058).

Conclusion

Immunosuppressive agents significantly reduce venous thrombosis relapse in BD.

     

Les manifestations oculaires de la maladie de Behçet

Uveitis is the most common ocular symptom in Behçet's disease. It is considered as a diagnostic criterion of the disease. Anterior uveitis is always non granulomatous, and sometimes associated with hypopion. Posterior involvement may include vitritis, retinal infiltrates, sheathing of retinal veins, occlusive vasculitis, and macular edema. Behçet's disease is chronic, and characterized by a spontaneously relapsing and remitting course. Male patients with younger age at onset and worse visual acuity at presentation, have higher risk of visual loss over time. The main goals in the management of patients with Behçet's disease-associated uveitis are rapid suppression of intraocular inflammation, preservation of vision, prevention of recurrences, and achievement of remission sustained after discontinuation of treatment. New therapeutic strategies such as interferon alpha and TNF alpha blockers have dramatically improved the visual prognosis of patients with intraocular inflammation related to this chronic and potentially blinding condition.     

Right to left shunting through communications between the left superior intercostal vein tributaries and the left atrium: A potential cause of paradoxical embolism

Objectives

To investigate the role of collateral venous pathways between the left brachiocephalic vein (LBV) and the left atrium through an arcade comprising the left superior intercostal vein (LSICV), left vertical vein (LVV), and pulmonary veins as a potential cause of paradoxical embolism.

Methods

A retrospective search was performed to find symptomatic patients with negative work up for paradoxical emboli whose chest CT or MR angiography by left arm contrast injection showed a visible right to left shunt through the LSICV/LVV collateral pathway (symptomatic group). We also evaluated the characteristics of this collateral pathway in 150 chest CT angiographies from general referrals (comparison group).

Results

We found 7 symptomatic patients. Initial presentations included neurological symptoms in all patients and episodes of hypoxemia in three patients. Communications between the LBV and left atrium through the LSICV/LVV pathway was seen is all 7 cases and confirmed by transesophageal echocardiography in two. An abnormal LBV was seen in 6 cases (absence in one, stenosis in 5). Moderate superior vena cava stenosis was seen in one and acute right subclavian vein thrombosis in one. Six patients had past history of an upper extremity central line placement. In the comparison group, LSICV was seen in 73 (48%), connecting to the hemiazygos vein in 41, to a LVV in 19 and to the left atrium in 7.

Conclusion

LSICV/LVV collaterals are common and can be a potential route for paradoxical emboli especially when stenosis of a draining vein such as LBV exists.     

L’aphtose récurrente : innocuité du thalidomide à faible dose

Purpose

Thalidomide is an effective treatment for recurrent aphthosis but its effectiveness at low dose has been rarely assessed.

Methods

Single-centre non-randomized retrospective open study.

Results

Forty-seven patients were treated with thalidomide for recurrent aphthosis (41 patients) or Behçet disease (six patients). Remission was obtained with a dose of 25 mg daily. Discontinuation of treatment for side effects was not observed in this case series.

Conclusion

Low dose thalidomide below 50 mg daily is an effective and well tolerated treatment of aphthosis.