The evaluation of the intracavitary effusions by a bedside ultrasound examination.

OBJECTIVE: This study aims to evaluate the bedside use of the pocket-sized ultrasound (US) device for the detection of the intracavitary effusions. METHODS: We randomly enrolled 40 patients admitted to S. Andrea Hospital of Rome. Every patient received a clinical and biochemical evaluation and a bedside US examination to detect and estimate the intracavitary (pleural, pericardial and intra-abdominal) effusions; the US measurements have been compared to the computed tomography (CT) scans (as gold standard). RESULTS: The patients presented a high prevalence of effusions: right pleural 16/40 = 40% (esteemed volume 236.3±500.7 ml, mean±standard deviation m±SD), left pleural 8/40 = 20% (127.0±377.4 ml), pericardial 12/40 = 30% (47.5±72.8 ml) and intra-abdominal effusions 5/40 = 12.5% of cases (110.9±600.6 ml). Linear regression analysis showed a significant correlation between US and CT measurements: pleural r = 0.973 p <  1×10-38, pericardial r = 0.927 p <  1×10-39, intra-abdominal space r = 0.921 p <  1×10-59. The accuracy of the bedside US at the pleural, pericardial and abdominal level was respectively 98%, 93% and 96% (Cohen's kappa coefficient 0.966, 0.841 and 0.833). CONCLUSIONS: The present study showed a high prevalence of the intracavitary effusions and a high accuracy of the bedside US. The bedside US by a pocket-sized device is promising tool for its advantages of reproducibility and non-invasiveness of the device.

Lag3+ regulatory T lymphocytes in critical carotid artery stenosis.

The aim of this study was to evaluate CD25+ and Lag3+ T regulatory subpopulations in patients with critical carotid artery stenosis (CAS) and Stanford-A acute aortic dissection (AAD). CD25+ and Lag3+ were measured in 36 patients affected by CAS and 24 patients with Stanford type A AAD. Based on neurological symptoms, patients affected by CAS were further divided in 25 asymptomatic (CAS-A) and 11 symptomatic (CAS-S) subjects. Twenty-five patients with traditional cardiovascular risk factors (RF), matched for age and sex, were used as control group. Interleukin (IL)-10, IL-6 and transforming growth factor-ß-levels were also measured. CD25+ T cells were significantly increased in CAS-S versus CAS-A (p > 0.05), AAD (p > 0.05) and RF (p > 0.05). Moreover, a significant increase in Lag3+ Tregs was observed in CAS e CAS-S versus AAD (p < 0.05) and RF (p < 0.05), whereas no significant difference was observed between CAS-S and CAS-A. IL-6 was higher in AAD compared to the other groups. Patients with neurological symptoms display a peculiar expansion of CD25+ T cells, strongly confirming a relationship between ischemic brain damage and this regulatory subpopulation, whereas Lag3+ Tregs early distinguish CAS from AAD and probably exert protective actions against aortic wall rupture throughout their anti-inflammatory functions.

In vitro fertilization and autoimmunity: Evidence from an observational study.

INTRODUCTION: The aim of this study was to evaluate the prevalence of antiphospholipid antibodies (aPLs) in infertile women undergoing in vitro fertilization (IVF). METHOD OF STUDY: From January 2012 to December 2017, 520 consecutive clinical records of infertile women undergoing IVF were evaluated. Among them, 100 consecutive clinical records of patients with positive autoantibodies were selected. RESULTS: In 100/520 (19.23%) women, positive auto-antibodies were detected: 35/520 (6.73%) fulfilled classification criteria for a systemic disease. Positive aPLs were observed in 43 women (8.27%): 17/520 (3.27%) fulfilled diagnostic criteria for PAPS/APS, whereas patients with positive aPLs, who fulfilled diagnostic criteria for a systemic autoimmune disease other than APS were 18/520 (3.46%). LA and aCL were the main aPLs detected 53.49% and 44.19% respectively, whereas aB2GPI were found in 25.58%. CONCLUSIONS: we suggest that women with infertility may represent a subpopulation of patients with underhanded systemic autoimmune syndromes in which the main symptoms represented are obstetrical complications. We, therefore, recommend evaluating aPLs in all patients undergoing IVF with the aim of recognizing women at a higher risk of miscarriage or pregnancy morbidity.

Systemic lupus erythematosus and myelofibrosis: A case report and revision of literature.

Blood cytopenia represents one of the diagnostic criteria for systemic lupus erythematosus (SLE) and may occur as the first symptom of the disease. Antibody-mediated peripheral destruction of blood cells is the main cause of cytopenia observed in patients affected by SLE, however, inflammatory anemia, nutritional deficiencies, immunosuppressive therapy and, more rarely, myelofibrosis (MF) have also been documented. In the literature, 45 cases of autoimmune MF (AIMF) and SLE have been previously reported. Here the 46 th case of a 43-year-old female with a SLE and an underhand cytopenia, with a review of the literature.

Recurrent miscarriages in women not fulfilling classification criteria for antiphospholipid antibody syndrome.

Obstetric antiphospholipid antibody syndrome (APS), is well defined by classification criteria. It is well known that women with APS should receive prophylactic anticoagulation therapy with subcutaneous low weight heparin all throughout pregnancy and in the first 6 weeks postpartum. However, the optimal treatment for pregnant women having positive anti-phospholipid antibodies, but not fulfilling classification criteria for APS is still unclear. In this retrospective study we report pregnancy outcomes of 10 patients affected by recurrent miscarriages and positive anti-cardiolipin or aß2GP1 antibodies with titers ranging from 10 to 20 GPL/MPL demonstrated at least twice before pregnancy.

Granuloma annularis revealing Wegener's granulomatosis.

Skin manifestations are often associated with systemic autoimmune diseases (SAD). Some SAD, such as systemic lupus erythematosus, psoriatic arthritis and scleroderma display pathognomonic dermatological features, whereas other systemic diseases such as sarcoidosis, vasculitis and rheumatoid arthritis can present with non-specific skin manifestations that range from erythema nodosum to necrotic lesions. Here we report the case of a 25-year-old man with uveitis, polyarthrirtis, pulmonary involvement, nephrotic syndrome, cutaneous granuloma and pneumonia by E. coli.

Hypercalcaemia in systemic lupus erythematosus.

Hypercalcaemia is found in more than 90% of the cases of primitive hyperparathyroidism and malignancies. Rarely, D hypervitaminosis, sarcoidosis, other granulomatous diseases, some drugs, and endocrine diseases may be responsible. Nine patients with systemic lupus erythematosus (SLE) and hypercalcaemia, without evidence of primary hyperparathyroidism, have been previously described. Here we report the 10th patient with SLE and hypercalcaemia, along with a brief review of the literature.

Renal limited Wegener's granulomatosis.

Differential diagnosis in patients presenting with fever of unknown origin (FUO) is often difficult because infectious diseases, neoplasms, infective endocarditis or systemic autoimmune diseases may all be responsible for the condition. Furthermore, vasculitis may generate typical, atypical or limited syndromes depending on the extent of vascular involvement. Here, we report the case of a 73-year-old man with FUO and renal failure due to a rare variant of Wegener's granulomatosis, limited to the kidneys.

[Splenic infarction after warfarin discontinuation during atrial fibrillation]. / Infarto splenico: rara complicanza tromboembolica di fi brillazione atriale.

We describe a case of patient with splenic infarction, admitted to our department for sudden abdominal pain and fever after discontinuation of anticoagulant therapy for atrial fibrillation, complicating a dilated myocardiopathy and mechanical prosthetic valve. Diagnosis of splenic infarction was made by enhanced-contrast computed tomography, while ultrasounds and radiography were negative. Anticoagulant therapy, gold-standard treatment, was followed by fast clinical improvement. Moreover, splenic infarction should be considered in all cases of acute or chronic pain in left hypochondrium and especially in patients with emboligenous cardiopathies or atrial fibrillation, the most common arrhythmia source of peripheral embolism in clinical practice.

Response to anti-tumour necrosis factor alpha blockade is associated with reduction of carotid intima-media thickness in patients with active rheumatoid arthritis.

OBJECTIVES: To determine whether tumour necrosis factor (TNF)-alpha blockers may reduce carotid intima-media thickness (cIMT) in patients with active rheumatoid arthritis (RA) steadily responsive to such therapy. METHODS: From 287 consecutive RA patients attending our out-patient clinic and diagnosed on the basis of the American College of Rheumatology (ACR) criteria, 49 without traditional cardiovascular risk factors and meeting the requirements for TNF-alpha blockers therapy were selected. Among them, 39 actually started TNF-alpha blockers, but only 30, who reached at least a response on the ACR 20% improvement criteria at 14 weeks, maintained during the whole year of treatment, were finally considered (group A). The remaining 10/49, homogeneous for age, sex, traditional cardiovascular risk factors, socioeconomic status, disease activity and duration, who did not consent to TNF-alpha-blocker administration, were used as controls (group B). Disease activity score in 44 joints (DAS44), erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) were evaluated before starting the study, and 3, 6, 12 months thereafter; cIMT was measured by ultrasound before and 12 months thereafter only. RESULTS: Patients in group A showed a very significant cIMT reduction (P < 0.0001 and P < 0.0001, on the right and left side, respectively), preceded by an early and lasting significant decrease in DAS44, ESR and CRP. Moreover, a significant correlation was found between cIMT and DAS44 (r = 0.435, P < 0.05). CONCLUSIONS: These results demonstrate that TNF-alpha blockade is associated with cIMT reduction in RA patients steadily responsive to therapy, probably by lowering inflammation.

Influenza vaccine administration in patients with systemic lupus erythematosus and rheumatoid arthritis. Safety and immunogenicity.

OBJECTIVE: To evaluate immunological safety and immunogenicity of influenza vaccine administration in patients with systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA). PATIENTS AND METHODS: Twenty-four patients with low and/or stable disease activity 14 with SLE (mean age 43.42+/-12.18 years; 13 women) and 10 with RA (mean age 51+/-14.57 years; 9 women), diagnosed on the basis of the American College of Rheumatology criteria, have been immunized with trivalent split influenza vaccine without adjuvant. Further 24 non-vaccinated patients, 14 with SLE and 10 with RA, and 10 vaccinated healthy subjects, all age- and sex-matched, were used as controls. The patients underwent clinical and laboratory (specific anti-influenzavirus antibodies, auto-antibodies, peripheral blood lymphocyte subpopulations) evaluation before and 30 days after vaccination; auto-antibodies were also assessed at 90 days and disease activity at 90 and 180 days. RESULTS: The specific antibody response towards the three used antigens (A/New Caledonia/20/99, A/Moscow/10/99, and B/Shangdong/7/97) significantly increased in both patients and healthy controls, without any significant difference between them. No significant difference could instead be observed on the clinical activity, auto-antibodies, and peripheral blood lymphocyte subpopulations before and after vaccination, and between patients and controls. CONCLUSIONS: Trivalent split influenza vaccine without adjuvant seems to be safe and immunogenic in patients with SLE and RA, provided that only patients with low and/or stable disease activity are selected.

Raynaud's phenomenon and antiphospholipid antibodies in systemic lupus erythematosus: is there an association?

OBJECTIVE: To evaluate the association of IgG and IgM antibodies directed against different negatively charged phospholipids (that is, anticardiolipin (aCL), antiphosphatidylinositol, antiphosphatidylserine, and antiphosphatidic acid) and anti-beta(2)-glycoprotein I (abeta(2)GPI), with Raynaud's phenomenon in patients with systemic lupus erythematosus (SLE). METHODS: Ninety three patients with SLE (81 female), 40 with and 53 without Raynaud's phenomenon, were included in the study. IgG and IgM antiphospholipid antibodies and abeta(2)GPI were determined by enzyme linked immunosorbent assay (ELISA). RESULTS: Fifty patients (54%) were positive for IgG and/or IgM antibodies to one or more phospholipid antigens or to beta(2)GPI. The prevalence of all autoantibodies evaluated, either IgG or IgM, was higher in patients without than in those with Raynaud's phenomenon. A negative association was found between IgG aCL and Raynaud's phenomenon (p=0.038), whereas autoantibodies other than aCL were not significantly associated with Raynaud's phenomenon. CONCLUSION: Our results demonstrate no positive association between antiphospholipid antibodies and Raynaud's phenomenon in SLE and indicate that measurement of anti-negatively charged phospholipid antibodies other than aCL is not useful as a serological marker predictive for Raynaud's phenomenon.

Primary antiphospholipid syndrome and cerebral atrophy: a rare association?

Neurologic complications are common in patients with antiphospholipid syndrome. In this article, we report the case of a young woman with neurologic disorders, a history of hypertension and transient ischemic attacks, and cerebral atrophy associated with primary antiphospholipid syndrome (PAPS). Magnetic resonance imaging of the brain showed multiple ischemic lesions and remarkable atrophy of frontal and parietal lobes. Cerebral atrophy in patients with PAPS can be considered as a feature of this disease. The case is discussed on the basis of relevant past literature. Although there are few reports on neuroradiologic findings in patients with PAPS, cerebral atrophy has been described. Because PAPS is more frequently recognized today than in the past, this condition should be included in the differential diagnosis of cerebral atrophy, particularly in young patients.

The infrequent association of synchronous renal and colonic malignancies.

The coexistence of both kidney and colon primary malignancies is a rare condition. We report the case of a 75-year-old woman who presented with bilateral pulmonary nodules at chest X-ray and stratigraphy. Total-body CT scan showed multiple, apparently metastatic, bilateral pulmonary lesions, a diffusely dysomogeneous neoformation in the lower pole of the right kidney and a gross neoformation in the ascending colon. A right nephrectomy and a right hemicolectomy were performed and histology showed two primary neoplasms: clear cell renal carcinoma and undifferentiated adenocarcinoma of the colon.

Kidney vasculogenesis and angiogenesis: role of vascular endothelial growth factor.

Vascular Endothelial Growth Factor (VEGF) plays a crucial role in the establishment of the vascular tree pattern. New vessels can be formed by two different ways; in the development of kidney both vasculogenesis and angiogenesis participate to microvessel assembly. VEGF and its receptor (VEGF-R) are co-expressed during kidney organogenesis and stimulate renal blood vessels development, induce and maintain the fenestrated phenotype in endothelium and regulate vascular permeability. VEGF and many other growth factors participate to the development of embryonic glomerular microvasculature. We believe that therapeutical use of VEGF or anti-VEGF antibodies may be performed in the treatment of many disorders.

Vascular endothelial growth factor: a key mediator of neoangiogenesis. A review.

Vascular endothelial growth factor (VEGF) is a multifunctional cytokine that exerts in vivo a key role in physiological and pathological neoangiogenesis by stimulating endothelial cell proliferation and vessel hyperpermeability. VEGF exists as one of four different isoforms, respectively, VEGF 121, VEGF 165, VEGF 189, VEGF 206, and seems to be a crucial mediator of physiological neoangiogenesis during the embryonic development and the female cycle. VEGF also has a major role in the pathogenesis of many diseases including hypervascularized tumors, rheumatoid arthritis, cutaneous diseases and proliferative retinopathies. Anti-VEGF anti-bodies or VEGF agonists may represent a novel approach in the treatment of these diseases.