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Biomedical subjects

R Priori

Publications and source records attributed to R Priori.

At least 37 records · Page 2Linked to original sources

Sjögren's syndrome presenting as ischemic stroke.

BACKGROUND: We describe a young woman who presented with minor stroke as a first clinical symptom of Sjögren's syndrome (SS) in the absence of well-known risk factors for cerebrovascular disease. CASE DESCRIPTION: The medical history included recurrent miscarriages and sun rashes, which directed the diagnosis toward immunologic disorders such as systemic lupus erythematosus and antiphospholipid antibody syndrome, which are often associated with stroke. Only complete laboratory testing, including SSB antibody studies, and ophthalmologic and salivary gland evaluation revealed the correct diagnosis. CONCLUSIONS: Sjögren's syndrome should be considered among the causes of stroke, especially in a young female patient.

Adult↗

Magnetic resonance imaging of the parotid glands and lip biopsy in the evaluation of xerostomia in Sjögren's syndrome.

Magnetic Resonance Imaging (MRI) of the parotid glands was performed in 23 patients with dry mouth. Each patient underwent lip salivary gland (LSG) biopsy and complete clinical and immunological assessment. MRI showed a quite specific nodular pattern in the parotid glands of patients with Sjögren's syndrome (SS), especially those with severe histologic abnormalities in LSG. However no significant correlation could be detected between MRI score and both LSG biopsy class and immunological abnormalities. MRI of the parotid glands can be regarded as a useful noninvasive procedure with high positive predictive value for the evaluation of the salivary component in SS.

Adult↗

Reflex seizures as initial manifestations of systemic lupus erythematosus in childhood.

We report a 10-year-old girl with reflex seizures characterized by visual and acoustic hallucinations, induced by visual and acoustic stimulations. The EEG showed atypical spike-wave discharges on the left temporo-occipital area, markedly activated by visual and acoustic stimuli (intermittent light, pattern-reversal stimulation and monoaural right pure tone). The patient was treated with carbamazepine and seizure stopped within 7 months. After 10 months from the start of the therapy she developed signs of systemic lupus erythematosus.

Acoustic Stimulation↗

Pulmonary arterial hypertension responsive to immunosuppressive therapy in systemic lupus erythematosus.

Two female patients with recent diagnosis of systemic lupus erythematosus (SLE) are reported. Pulmonary arterial hypertension was diagnosed by Doppler echocardiography. Immunosuppressive therapy was started at the time of diagnosis of SLE. After 2 months of therapy, Doppler echocardiography was repeated and the estimated pulmonary artery systolic pressure was substantially decreased from 78 to 42 mmHg and from 67 to 42 mmHg, respectively, along with significant improvement of the clinical conditions.

Adult↗

Markers of vascular injury in Behçet's disease associated with retinal vasculitis.

The pathogenesis of vascular damage in Behçet's disease (BD) is still unknown. We investigated anticardiolipin antibodies (AC) in a genetically homogeneous group of Italian patients with BD and retinal vasculitis. In a subset, we examined the level of factor VIII-related antigen and antiendothelial cell antibodies (AECA). We found a high prevalence of AC; AECA were not found in most of our patients. Only three patients had raised levels of factor VIII-related antigen, all of whom had systemic involvement. We believe BD may be considered as a vasculitis of unknown origin in which several and different pathogenetic factors may play a role.

Adolescent↗

Cardiac involvement in Behçet's disease.

To assess the prevalence and the extent of cardiac involvement in patients with Behçet's disease and to investigate the possible causes that may predispose to this involvement, 30 patients affected by Behçet's disease and 30 normal control subjects were submitted to M-mode, two-dimensional, and Doppler echocardiographic evaluation. Moreover, antinuclear and anticardiolipin autoantibodies were determined in the sera of both patients and control subjects. Finally, HLA-B51 positivity was assessed in the patients and in a historical control group. Mitral valve prolapse was observed in 50% and proximal aorta dilatation in 30% of the patients. There was a significant difference in the rate of these abnormalities in comparison with the control group. Left ventricular function parameters were similar between the two groups. The positivity rate of antinuclear and anticardiolipin autoantibodies was very low (7%), without differences between the groups. HLA-B51 was detected in 82.7% of the patients versus 21.7% in the control group (p < 0.00001). In conclusion, this study demonstrates a high rate of cardiac abnormalities in patients with Behçet's disease.

Adult↗

The lactoferrin tear test in the diagnosis of Sjögren's syndrome.

PURPOSE: To assess the sensitivity and specificity of the lactoferrin tear test (LTT) in the diagnosis of keratoconjunctivitis sicca due to Sjögren's syndrome (SS), comparing it with the other lacrimal tests and with immunological tests. METHODS: 25 patients suffering from SS (24 women and 1 man, median age 51.5 years, s.d. 16.3); control group: 20 patients with various kinds of conjunctivitis without dry eye. Tests in both groups: Schirmer I (ST), BUT, ferning test, lactoferrin immunoassay, fluorescein and Rose Bengal staining. Immunological tests: serum titers of anti-nuclear (ANA), anti-DNA, anti-ENA (SS-A, SS-B, RNP, FR) antibodies. In the patients with SS, labial salivary gland biopsy was also performed. RESULTS: LTT had a specificity of 95% and a sensitivity of 72%, compared to 85% and 64% for the Schirmer I test. The ferning test has the highest sensitivity (92%), and none of the cases positive to BUT was negative to the ferning test. The combination of LTT and ferning test gave a value of 78% compared to 70% with ST. The correlations between positive LTT and positive ANA, SS-A and labial biopsy were respectively 83%, 67% and 80%, as against 67%, 50% and 65% for the Schirmer I test. CONCLUSIONS: In our study, LTT showed very high specificity, good sensitivity particularly when combined with qualitative tear tests, and a good correlation with the immunological and bioptic tests for SS. Since it is easy to perform, in our opinion LTT can be included in the diagnostic routine for keratoconjunctivitis sicca in SS.

Antibodies, Antinuclear↗

Five-year follow-up of 165 Italian patients with undifferentiated connective tissue diseases.

OBJECTIVE: To study those conditions with a proven or hypothesised immunologic pathogenesis and denominated under a working definition of undifferentiated connective tissue diseases (UCTD). METHODS: A multicentre prospective study was organised involving 10 tertiary referral centers of internal medicine in Italy, with the aim of describing the natural history of UCTD and the prevalence of its different clinical and immunological manifestations. RESULTS: After a five-year follow-up period, data on 165 patients were available for analysis. UCTDs occur mainly in females in their fourth decade of life. Articular and mucocutaneous features and Raynaud's phenomenon represent the most common findings. Nevertheless, we also detected a relatively high incidence of permanent major organ damage. Regarding the immunologic parameters, we documented some conflicting results in the correlation between serologic abnormalities and clinical features. In 10 patients UCTD evolved to a major disease, generally systemic lupus erythematosus or Sjögren's syndrome. CONCLUSION: A low rate of evolution to a defined autoimmune disease, the limited use of steroid or immunosuppressive therapy, and a favourable course in the majority of cases are the main characteristics of patients with UCTDs.

Adolescent↗

Thalidomide: focus on its employment in rheumatologic diseases.

Thalidomide is an immunomodulatory agent; although its mechanisms of action are not fully understood, many authors have described its anti-inflammatory and immunosuppressive properties. More interestingly, thalidomide has shown the ability to suppress tumor necrosis factor alpha (TNF alpha) production and to modify the expression of TNF alpha induced adhesion molecules on endothelial cells and on human leukocytes. Thalidomide has been used in several diseases (i.e. dermatological, autoimmune, gastrointestinal). In this review we focus specifically on the use of this drug in disorders with rheumatological features such as lupus erythematosus, rheumatoid arthritis and Still's disease, ankylosing spondylitis, and Behçet's disease. Despite its well known side effects, first of all peripheral nerve involvement and teratogenesis, which can be avoided by following strict guidelines, thalidomide could represent an alternative drug in some rheumatological conditions, particularly in patients who show resistance, contraindication or toxicity with other conventional treatments.

Abnormalities, Drug-Induced↗

Wrist joint involvement in systemic lupus erythematosus. An ultrasonographic study.

OBJECTIVE: To define joint alterations in the wrists of patients with systemic lupus erythematosus (SLE) by ultrasonography (US). METHODS: Fifty-two wrists of 26 SLE patients and 30 wrists of 15 healthy controls were evaluated using US by two different experienced operators, blinded to the clinical data. A 14 MHz linear probe was used. Power Doppler (PD) was applied to evaluate the presence of synovial neoangiogenesis as a parameter of active local synovitis. The findings were correlated to the clinical evaluation, serological systemic disease activity parameters (ESR, C3 levels) and the SLE-disease activity score (SLEDAI). Statistical analysis was performed by the EPISTAT program. RESULTS: Signs of synovitis were found in 22 wrists (42.3%). Synovial proliferation was present in 10 joints (19.2%), PD positivity in 5 (9.6%) and joint effusion in 13 (25%). Erosions were present in both wrists (3.8%) of one patient. Signs of tenosynovitis of one or more tendons were shown in 23 cases (44.2%). Ganglia were found in 2 joints (3.8%). Changes of the median nerve, joint dislocations, tendons' ruptures, cysts and nodules were never detected. In 14 wrists (26.9%) no alterations were found. There was no correlation between sonographic findings and clinical, laboratory and indexes signs of disease activity. In the control group the only alteration found was tenosynovitis in 1 joint (p < 0.0001). CONCLUSION: US proved to be an useful technique to detect wrist joint alterations in SLE. These findings may help the physician to modulate treatment strategies and to perform a low cost monitoring of joint disease activity.

Adult↗