Biomedical subjects

F Ingegnoli

Publications and source records attributed to F Ingegnoli.

10 recordsLinked to original sources

PubMed2006

Primary antiphospholipid syndrome associated with small aorta syndrome: a case report.

It has been widely accepted that the antiphospholipid syndrome (APS) is an autoimmune hypercoagulability syndrome in which a variety of venous and arterial thrombotic events may occur. Peripheral obliterating arterial disease characterized by aortoiliac steno-occlusion occurring in young women, is reported in the literature under the name of Small Aorta Syndrome (SAS). Although it remains unclear whether SAS represents a separate entity, the small size of the distal aorta increases the risk for aortoiliac occlusive disease. A 41-year old white woman was admitted with acute digital ischemia of the left foot. She had positive lupus anticoagulant and IgG anti-cardiolipin antibodies (61 UI/mL), but antinuclear antibodies and anti-ds-DNA antibodies were negative. She previously had two deep venous thromboses of the legs and, despite the oral anticoagulant therapy, pulmonary embolism occurred. Shortly thereafter, abdominal angio-magnetic resonance imaging suggested that the infra-renal aorta was narrowed more than 50%, without thrombotic occlusion of the terminal aorta and common iliac arteries. These findings were compatible with the features of SAS. There were no atherosclerotic changes in the artery wall and no other prediposing risk factors such as smoking, oral contraceptive or hyperlipidemia. After adequate anticoagulation and intravenous prostacyclin treatment the patient's symptoms and the ischemic lesions improved markedly. To our knowledge this is the first report of the association of SAS and primary APS. The occurrence of SAS in patients with APS may dramatically increase the risk of trombothic events.

Adult↗

PubMed2004

Paget's disease and cochlear implantation.

Paget's disease of bone is a common disorder of unresolved etiology characterized by excessive bone resorption followed by excessive bone formation. If the skull is affected this may result in hearing loss and eventually develop into profound deafness. To date, no cases of cochlear implantation in patients with Paget's disease have been reported. The authors present a case of radiographically confirmed Paget's disease of the skull in a 77-year-old man with a 20-year history of progressive bilateral sensorineural hearing loss who underwent cochlear implantation. A successful insertion of the Nucleus 24 Contour electrode array was achieved without surgical and postoperative complications. At the 10 months' postoperative evaluation, the patient had gained useful open-set speech perception. In quiet conditions, his performance scores on the word and sentence recognition tests were 100 and 98 per cent, respectively. In the presence of noise (at +10 dB. signal-to-noise ratio), his performance scores on the word and sentence recognition tests were 96 and 94 per cent, respectively.

Aged↗

PubMed2002

Stromal cell-derived factor 1 (CXCL12) induces monocyte migration into human synovium transplanted onto SCID Mice.

OBJECTIVE: The mechanisms by which monocyte/macrophage cells migrate to the joint involve a series of integrated adhesion and signaling events in which chemokines and their receptors are strongly implicated. This study was undertaken to investigate the hypothesis that stromal cell-derived factor 1 (SDF-1), a CXC chemokine (CXCL12), plays a critical role in monocyte/macrophage localization to synovium. METHODS: SDF-1 and CXC receptor 4 (CXCR4) expression in rheumatoid arthritis (RA) and osteoarthritis synovium and graft SDF-1, tumor necrosis factor alpha (TNF alpha), and human and murine vascular markers were examined by immunohistochemistry and double-immunofluorescence. The functional capacity of SDF-1 to modulate monocyte migration into joints was investigated by examining the localization of pro-myelomonocytic U937 cells into synovial tissue transplanted into SCID mice. SDF-1, TNF alpha, or saline was injected into graft sites and response determined by the number of fluorescently labeled U937 cells (injected intravenously) detected in grafts by ultraviolet microscopy. RESULTS: SDF-1 and CXCR4 were highly expressed in CD68+ cells in the RA synovium. SDF-1 induced U937 cell migration in vitro and in vivo in a dose-dependent manner and, in vivo, SDF-1 was more effective than TNF alpha. In contrast to TNF alpha, SDF-1 did not induce intracellular adhesion molecule 1 in transplant microvasculature. Furthermore, intragraft injection of SDF-1 did not up-regulate TNF alpha, or vice versa. CONCLUSION: This study demonstrates, for the first time, that SDF-1 is functional in vivo when injected into synovial grafts. In addition, SDF-1 is more potent than TNF alpha, and its mechanisms of action appear to be autonomous. Therefore, SDF-1 may be an important TNF-independent molecule involved in the migration to and retention of inflammatory effector cells in the joint.

Aged↗

PubMed2002

[Role of chemokines in the pathogenesis of chronic synovitis during rheumatoid arthritis].

Chemokines play a central role in the pathogenesis of rheumatoid arthritis (RA) synovitis which is characterised by new blood vessel formation, thickening of the lining layer and infiltration of immune cells. The inflammatory infiltrate is generated by a series of events which include the recruitment of leukocytes from the blood stream into the tissue, their local retention and activation to effector cells. All these processes are finely regulated by the interplay of different cell adhesion molecules (CAMs) and chemoattractant factors called chemokines (CK). CK are a superfamily of small proteins that play a crucial role in immune and inflammatory reactions. These chemoattractant cytokines share structural similarities including four conserved cysteine residues which form disulphide bonds in the tertiary structure of the proteins. CK mediate their effects by binding specific receptors (CK-R) characterised by a domain structure which spans the cell membrane seven times and signal through heterotrimeric GPT-binding proteins. Activation of the CK network results in an amplification of the inflammatory cascade and consequently in the progressive destruction of RA joints. The recognition of the central role of CK in inflammation has paved the way to the development of new agents capable of interfering with CK and CK-R. This review will focus in particular on the role of CK in regulating leukocyte trafficking in RA joints.

Arthritis, Rheumatoid↗

PubMedDate not supplied

[Role of cell migration in the pathogenesis of rheumatoid arthritis: in vivo studies in SCID mice transplanted with human synovial membrane].

OBJECTIVE: Adhesion mechanisms play a central role in the recruitment of leukocytes which characteristically infiltrate rheumatoid synovium. Therefore, we adapted an animal model, in which human rheumatoid synovium was transplanted into severe combined immunodeficient (SCID) mice, to study the effects of Tumor Necrosis Factor-alpha (TNF-alpha) in modulatine leukocyte migration and to investigate the chemotactic potential of Stromal Derived Factor-1 alpha (SDF-1 alpha). MATERIALS AND METHODS: Human synovium samples, obtained from patients undergoing joint replacement, were divided into two parts. One was analysed by immunohistology and the other was implanted subcutaneously into SCID mice under general anaesthesia. Four weeks post-transplantation, grafts were injected with optimal dose of SDF-1, TNF-alpha or saline (negative control). At the same time, animals were injected iv with fluorescently labelled cells. 48 hours later mice were sacrificed and grafts removed for cryo-hystology. The number of cells migrating to the grafts was determined by UV-microscopy and the results expressed as cells per high power field. RESULTS AND CONCLUSIONS: In these studies we provide the evidence that: 1) the animal model, in which human tissues are grafted into SCID mice, can be used to study cell migration under controlled experimental conditions; 2) direct intragraft injection of TNF-alpha increases lymphocytes migration and up-regulates the expression of human adhesion molecules (CAMs) and 3) SDF-1 alphainjected intragraft increases the migration of the pro-myelo-monocytic U937 cells to synovial transplants, even more efficiently than TNF-alpha, but without modifications of CAMs' expression.

Animals↗

PubMedDate not supplied

[Case report: rheumatoid arthritis and large granular lymphocytes syndrome].

Felty's syndrome (FS) is a rare complication (less than 1%) of rheumatoid arthritis (RA), with the clinical feature of splenomegaly and neutropenia. Approximately 10-40% of FS patients have an expansion of peripheral blood large granular lymphocytes (LGL). This cell population mainly consists of two subsets: cytotoxic T cells (CD8+, CD57+) and natural killer cells (CD3-,CD8-,CD56+). It has been hypothesised that LGL expansion could be responsible for neutropenia by suppressing neutrophil precursors in the bone marrow, but various mechanisms have been proposed to explain this association. We report a case of a 60-year-old woman with rheumatoid factor positive RA who developed LGL expansion responsible for splenomegaly, but without neutropenia. In conclusion, LGL expansion is an uncommon complication of RA and may be responsible for both FS and clinical pictures resembling FS.

Felty Syndrome↗

PubMedDate not supplied

[Anti-chromatin antibodies in juvenile rheumatoid arthritis].

OBJECTIVE: to evaluate the prevalence and clinical significance of anti-chromatin antibodies (Abs) in juvenile rheumatoid arthritis (JRA). METHODS: IgG anti-chromatin Abs were detected by an enzyme-linked immunosorbent assay (ELISA), in sera of 94 children with JRA (10 children with systemic, 38 with polyarticular and 46 with oligoarticular disease onset). As control group, 33 age- and-sex-matched healthy children (HC) were also examined. RESULTS: Abs to chromatin were detected in 24/94 (25.5%) of children suffering from JRA. Particularly, the higher prevalence of anti-chromatin Abs has been found in children with oligoarticular (30,4%) and polyarticular (23.7%) onset JRA. In these groups Abs titers were significantly higher compared to systemic JRA and HC (p=0.003). Anti-chromatin Abs were observed more frequently in patients with oligoarticular disease and chronic uveitis (21.7%). Furthermore, higher levels of anti-chromatin Abs has been found in all the patients treated with anti-TNF-alpha therapy (p< 0.0001). CONCLUSIONS: our results confirm previous data about the prevalence of anti-chromatin Abs in JRA. These Abs were significantly higher in the group of patients with oligoarticular onset with past or present history of ocular involvement and in the group with polyarticular JRA treated with biologic therapy. A long-term follow-up study could be useful to evaluate the potential utility of these autoantibodies.

Arthritis, Rheumatoid↗

PubMedDate not supplied

Autoantibodies to chromatin: prevalence and clinical significance in juvenile rheumatoid arthritis.

OBJECTIVE: To determine the prevalence of anti-chromatin antibodies (Abs) in juvenile rheumatoid arthritis (JRA) and to assess any association between the presence of anti-chromatin Abs and clinical subsets of the disease. METHODS: IgG anti-chromatin Abs and anti-extractable nuclear antigens (ENA) Abs were detected by an enzyme-linked immunosorbent assay (ELISA), and antinuclear Abs (ANA) by indirect immunofluorescence in sera of 89 children with JRA. Ten children with systemic, 32 with polyarticular and 47 with pauciarticular disease onset (uveitis occurred in 17/47 children) were studied. As a control group, 12 sera of patients suffering from idiopathic uveitis and 31 age- and-sex-matched healthy children (HC) were examined. RESULTS: Abs to chromatin were detected in 14/47 (29.8%) of children suffering from pauciarticular onset JRA and in this group the higher prevalence of anti-chromatin Abs has been found in children with chronic uveitis (p = 0.002). Anti-chromatin positivity was observed in 2/10 (20%) of systemic and in 3/32 (9.3%) of polyarticular onset JRA. Furthermore, none of the patients with idiopathic uveitis and HC had Abs to chromatin. anti-chromatin Abs titers remained relatively stable over a 6-month control period. CONCLUSION: Our results confirm previous data about the presence of circulating anti-chromatin Abs in juvenile arthritis. Interestingly, anti-chromatin Abs were significantly higher in the group of patients with pauciarticular onset with past or present history of uveitis, than in patients without ocular involvement. A long-term follow-up study could be useful to demonstrate the potential utility of these autoantibodies in diagnosing, classifying and treating children affected.

Antibodies, Antinuclear↗

PubMedDate not supplied

[Raynaud's phenomenon].

Raynaud's phenomenon (RP) is a vasospastic disorder characterized by episodic color changes of blanching, cyanosis, and hyperemia in response to cold and/or emotional stress. Although most typically noted in the fingers, the circulation of the toes, ears, nose and tongue is also frequently affected. Population studies have shown that RP in adults is more common in women than men, with prevalence estimates ranging from 4% to 30%. Geographic variations in the prevalence reflect differences in climate. RP may be a primary or a secondary process. LeRoy and Medsger suggested criteria for primary RP: symmetric attacks, the absence of tissue necrosis, ulceration or gangrene, the absence of a secondary cause, negative antinuclear antibodies, normal nailfold capillaroscopy and a normal erythrocyte sedimentation rate. Secondary RP is characterized by an age of onset of more than 30 years, painful and asymmetric attacks, ischemic skin lesions, positive autoantibodies, capillaroscopic abnormalities and/or clinical features suggestive of connective tissue diseases (CTDs). Among the CTDs, systemic sclerosis has the highest frequency of RP. Finding a cause for RP requires a knowledge of the patient's occupational, smoking, drug history, physical examination, nailfold capillaroscopy, routine laboratory tests and autoantibodies. Furthermore, RP should be distinguished from acrocyanosis, a condition characterized by continuous cyanosis of the hands or feet that is aggravated by cold temperature. The most important instruction to the patient is abstinence from any smoking, offending drugs should be discontinued, and abrupt changes in temperature. If these measures are inadequate, calcium-channel blockers are the most widely used (nifedipine 30 mg up to 90 mg daily). Alternatively, sympatholytic agent (prazosin), angiotensin II -receptor type I antagonist (losartan), selective sertonin-reuptake inhibitor (fluoxetine) may be useful. In the severe cases the role of prostaglandins is well established, but standard therapeutic protocols are not jet available.

Adolescent↗

PubMedDate not supplied

Capillaroscopic observations in childhood rheumatic diseases and healthy controls.

OBJECTIVE: To describe, by using video nailfold capillaroscopy (NFC), microvascular abnormalities in children with rheumatic diseases and to evaluate the capillary changes over a follow up period. METHODS: 118 children suffering from rheumatic diseases: 55 juvenile idiopathic arthritis (JIA), 7 mixed connective tissue disease (MCTD), 6 primary Raynaud's phenomenon (PRP), 34 systemic lupus erythematosus (SLE), 8 juvenile systemic sclerosis (JSSc) and 8 juvenile dermatomyositis (JDM) were included in the study. Patients with major capillaries abnormalities or scleroderma pattern were followed up for at least 12 months. 70 age- and sex-matched healthy controls (HC) were also examined. RESULTS: In HC there was a significant correlation between age and capillary length (p = 0.001). JIA patients showed capillary number, size, shape and arrangement similar to HC. Minor abnormalities were frequently observed. The percentage of major abnormalities were significantly increased compared to HC in MCTD (p = 0.008), SLE (p = 0.0002) and JDM patients (p < 0.0001). 5/8 of JSSc had a scleroderma pattern from the onset of the disease. The serial observations in connective tissue diseases also showed that the evolution of capillaroscopic pattern was not unidirectional. In fact, in some nailfolds there was an increase in capillary loss and in avascular areas, whereas sometimes it remained stable on repeated examination. CONCLUSION: NFC can be used as a simple, inexpensive, non-invasive method to evaluate the microvascular abnormalities in childhood rheumatic conditions, and it may be useful in early recognition and monitoring scleroderma spectrum disorders.

Adult↗