[Isolated congenital heart block in undifferentiated connective tissue disease and in primary Sjögren's syndrome: a clinical study of 81 pregnancies in 41 patients]. / Il blocco cardiaco congenito nella connettivite indifferenziata e nella sindrome di Sjögren primitiva. Studio di 81 gravidanze in 41 pazienti.

OBJECTIVE: To study the incidence and the features of congenital heart block (CHB) in patients with undifferentiated connective tissue disease (UCTD) and primary Sjögren's syndrome (pSS). METHODS: We studied 81 pregnancies of 41 women attending the Outpatients' Clinic of the Rheumatology Unit of University Hospital of Padova from July 1989 to March 2004. Twenty five of these (61%) were affected with UCTD and 16 (39%) with pSS. Serologic inclusion criteria was anti-Ro/La positivity, assessed by counterimmunoelectrophoresis and ELISA. RESULTS: CHB was found in 2 out of the 46 (4.3%) pregnancies followed by our Staff and in 2 out of the 35 (5.7%) included in the retrospective part of the study. In 3 cases CHB was a 3rd degree block, causing pregnancy termination in 2. The only 2nd degree block was identified in one patient at the 22nd week of gestation and treated with dexamethasone and plasma-exchange. All of the women were positive to 52 kd and 60 kd Ro autoantibodies. CHB mothers had higher titer antibodies to 52 kd Ro protein than did the mothers with healthy infants (P = 0.026). Electrocardiographic abnormalities at birth were found in 3 out of 29 asymptomatic infants. One presented sinus bradycardia, the second abnormalities of ventricular repolarization, both regressed spontaneously, while the third ventricular extrasystoles which continue even now at 5 months. CONCLUSIONS: These results showed that in UCTD and pSS there is a higher incidence of CHB than that reported in Systemic Lupus Erythematosus. Electrocardiographic screening in all infants born to mothers with anti-Ro/La antibodies would seem an important measure to identify those with irreversible heart conduction abnormalities.

[Comparison of ultrasonography and sialography vs. magnetic resonance in the diagnosis of the primary Sjogren's syndrome]. / La risonanza magnetica nucleare nella diagnosi della sindrome di Sjögren primitiva. Confronto con le metodiche ecografica e scialografica.

To date there is no agreement as to which imaging technique is best for the evaluation of the oral component of primary Sjögren's Syndrome (SS). The purpose of the present study has, therefore, been to determine the reliability of Magnetic Resonance (MR) in the evaluation of salivary alterations in patients with SS. The study involved 23 patients suffering from SS according to the European criteria. All the patients underwent ultrasonography and MR of the major salivary glands, parotid sialography and biopsy of the minor salivary glands. The first control group was made up of 50 healthy subjects who underwent parotid ultrasonography. The second control group comprised 23 subjects who underwent MR of the head and neck for other non parotid pathology. The ultrasonography, MR and sialography images were evaluated by a single observer during a single session and scored from 0 to 4. In the SS patients ultrasonography was abnormal in all 23 cases (100%): 3 patients showed grade 1 alterations (13%); 5 grade 2 (21.7%); 9 grade 3 (39.1%); 6 grade 4 (26.1%). In the healthy controls, grade 0 was found in 36 subjects (72%) while the remaining 14 subjects revealed grade 1 alterations (28%). Using MR imaging only one of the SS patients showed grade 0 alterations (4.3%), 7 showed grade 1 alterations (30.4%), 9 grade 2 (39.1%), 5 grade 3 (21.7%) and only 1 grade 4 (4.3%). MR imaging sensitivity was 95.8% while specificity was 100%. For ultrasonography, considering grade 1 as non pathological, we found a sensitivity of 88.4% and specificity of 100%. The MR score for SS patients was compared to that obtained with sialography and ultrasonography. There was a good correlation between MR and sialography (r = 0.528, p = 0.010) while the correlation between MR and ultrasonography was not statistically significant. This study confirms that, of the diagnostic procedures available for evaluation of salivary gland involvement in SS, the most useful initial examination is ultrasonography. When there is some doubt or there are subtleties, MR is a valid alternative to classical sialography.

[Imaging in major salivary gland diseases] / Diagnostica per immagini nelle malattie delle ghiandole salivari maggiori.

Most of the salivary glands diseases are characterized only by a few distinct clinical patterns. Medical history and clinical examination are still considered of great relevance. However, in order to obtain a definite diagnosis, imaging techniques are required in most of the cases. Salivary glands ultrasonography (US) is the technique to be used as the first because US can easily differentiate calculosis, inflammatory diseases and tumors. Sonography is also frequently needed to perform needle aspiration or biopsy (FNAC). Sialography should be used essentially for assessing chronic sialoadenitis as well as Sjögren's syndrome. At present, Magnetic Resonance sialography should be preferred because of the greater sensibility in diagnosing inflammatory diseases of the salivary glands. It allows to evaluate both intraglandular oedema and nodules, so that incannulation of the salivary duct is not required. Computer Tomography (CT) and Magnetic Resonance imaging (MR) are useful when neoplasm are suspected, particularly if deep areas of the gland, which cannot be visualized by US, are involved. Sequential scintigraphy is currently employed for assessing the functional status of all the 4 major salivary glands and evaluating the chronic evolution of glandular damage.

[Primary Sjögren's syndrome. A longitudinal study of 68 patients]. / Sindrome di Sjögren primitiva. Studio longitudinale di 68 pazienti.

There are only few studies, regarding primary Sjögren's syndrome (pSS) long-term clinical course. Moreover, it has been often studied in a restricted number of patients, employing different recruitment and diagnostic criteria. During a 10 years follow-up, we longitudinally evaluate clinical course as well as severe complications and mortality rates in 68 patients with pSS, diagnosed according to the Fox's criteria. Patients were divided into 2 groups according to the autoantibodies pattern detected at the diagnosis: anti-Ro and/or La positive and anti-Ro La negative. Glandular manifestations of pSS were distinctively present in the majority of patients already at time of the diagnosis and serological findings remained typically constant during the whole follow-up. Increased IgG, IgA and ESR as well as low C4 serum levels were significantly prevalent in the Ro and/or La positive group. Finally, we did not found any significant increase in the mortality rate of pSS patients in comparison with the general population.