Relationship between the occurrence of anti-SSA, anti-SSB autoantibodies and HLA class II alleles from the aspect of in vitro inhibitory effect of glucocorticosteroid on the antibody-dependent cellular cytotoxicity in patients with primary Sjögren's syndrome.

Though at present there is no evidence-based algorithm for the treatment of primary Sjögren's syndrome, it is generally accepted that glucocorticosteroid (GS) therapy must be introduced in cases with severe systemic manifestations. As the side-effects of the GSs are well known, it would be useful to know in advance how the patients will respond to this type of treatment. For this reason we measured the in vitro steroid sensitivity of 29 SS patients using inhibition of antibody dependent cellular cytotoxicity (ADCC) test by methylprednisolone compared to that of 28 controls. SS patients proved to be significantly less sensitive to GSs than controls (inhibition of ADCC reaction: 42.4 vs 53.1%; p < 0.01). This was especially true in SS patients with anti-SSA and/or SSB autoantibody positivity and with HLA-DR2 and/or -DR3 alleles. Comparing the results of the in vitro GS sensitivity and the clinical effectiveness of the previously applied corticosteroid therapy it seems that steroid inhibition of ADCC reaction has a predictive value in determination of in vivo sensitivity to GSs. However, in patients with decreased in vitro GS sensitivity a more expressed in vivo steroid sensitivity cannot be excluded.

The place of magnetic resonance and ultrasonographic examinations of the parotid gland in the diagnosis and follow-up of primary Sjögren's syndrome.

OBJECTIVE: The aim was to determine the place of magnetic resonance imaging (MRI) and ultrasonographic (US) examination in the diagnosis and follow-up of Sjögren's syndrome (SS). METHODS: Parotid MRI and US examinations were carried out on 44 primary SS patients and 52 controls of similar age. RESULTS: The most important structural changes in SS were different degrees of parenchymal inhomogeneity, which could be detected by both methods, and were found more frequently in the SS patients than in the controls (MRI: 95.4 vs 17. 3%; US: 88.6 vs 7.7%; P<0.001). There was good agreement between the MRI and US findings both in the SS cases (93.2%) and in the controls (86.5%). In one SS patient who developed parotid lymphoma, the US examination showed a hypoechoic 'cobblestones'-like inhomogeneous internal pattern which was coupled with an almost homogeneous MRI pattern. CONCLUSIONS: MRI appears unnecessary as a routine method in the diagnosis of SS; US examination is suitable both for the diagnosis and follow-up of SS. The above combination of the seemingly contradictory US and MRI findings is highly characteristic of lymphoma which has developed in the course of the disease.

Impaired microvascular response to cholinergic stimuli in primary Sjögren's syndrome.

OBJECTIVE: Signs of a parasympathetic dysfunction have been revealed in primary Sjögren's syndrome (SS). Its role in the pathogenesis and the clinical picture of the disease is not clear. To investigate the responsiveness of SS patients to a cholinergic agonist, a model was used involving examination of the cutaneous microcirculation. The microvascular response to the administration of carbachol was measured, a muscarinic cholinergic agonist. METHODS: Twenty two SS patients and 12 controls were examined. Carbachol and 0.9% saline solution were administered intracutaneously into the forearm skin at two distinct places. Skin blood flow (SBF) in the injected areas was measured continuously before and for 10 minutes after the injections by means of a laser Doppler perfusion monitor. The increase in SBF in response to carbachol (dSBF), reflecting vasodilatation, was calculated by a formula including the baseline and the maximum SBF values after the injections of carbachol and saline solution. RESULTS: The vasodilatation was significantly lower in SS patients than in the controls (mean dSBF: 2.1 (range: 1.0-4.5) versus 3.3 (range: 1.7-7.6), p=0.02). With non-responder patients defined as those in whom a smaller response was observed than in any of the controls, 11 of the 22 SS patients proved to be non-responders to carbachol. Comparisons of demographic, clinical and laboratory characteristics and HLA class II genotypes between responder and non-responder SS patients did not show any significant differences. CONCLUSIONS: A diminished or absent response to carbachol indicates a cholinergic dysfunction in SS patients. A disturbance in the neurotransmission at a receptorial or postreceptorial level is hypothesised. Unresponsiveness to cholinergic stimuli may contribute to exocrine insufficiency.

[Transbronchial catheterization: a method for outpatient angiographic examination]. / Transbrachialis katéterezés: lehetöség a járóbetegek angiográfiás vizsgálatára.

The authors report on their own experiences with a not unknown, but not widely used catheter technique. On the basis of 607 transbrachial angiographies, they have established the possibilities and occasional dangers of this method. They conclude that the transbrachial technique is suitable not only for aortography, but also for selective angiography and vascular interventions. In cases involving cooperative patients, this method can furnish a basis for the angiography of outpatients.

Laparoscopic deroofing of nonparasitic liver cysts. Comparison different methods.

Between 1982 and 1995 116 patients underwent operations because of NC of the liver, 18 of them were operated on by laparoscopic way. There were 94 male (mean age: 51.9 years) and 66 female (mean age: 54.6 years) patients. The mean size of the cysts were 57.4 mm in diameter (20-140 mm), In 94 cases they were solitary and in 11 they were multiple. In 31 cases we performed an enucleation, in 60 cases a fenestration and in one case a punction of the cysts. A liver resection had to be carried out in two cases. In 18 cases the fenestration of the cysts was performed by laparoscopy and in 10 cases cholecystectomy was simultaneously carried out. The most common complications were fever and wound suppuration. There was no mortality. Another 26 patients with NC have been treated with ultrasound guided puntion of the liver cysts. The cysts were solitary in twenty-three cases and multiple in three other cases. The number of the cysts were thirty-two. One case had to be operated on because of recurrence of the cyst. Mortality could not be observed. In the laparotomized group the average time of the hospitalisation was 14.3 days, and among the patients who were operated on by laparoscopic way 7.0 days. An intervention is indicated only in case of severe complaints or the growing of the lesion. Recently we prefer the ultrasound guided punction or the fenestration of the liver cysts by means of the laparoscopic way.

Parotid gland ultrasonography as a diagnostic tool in primary Sjögren's syndrome.

The diagnostic value of parotid gland ultrasonography (Acuson 128, 7 MHz transducer) was studied in 62 patients with primary Sjögren's syndrome (SS) and in 69 controls of similar age and sex distribution. Different degrees (mild, evident or gross) of parenchymal inhomogeneity (PIH) were the most important sonographic changes in SS; they occurred in 83.9% of the patients. The sonographic results (the presence or absence of PIH) were in accordance with the parotid sialographic and scintigraphic findings and the histology of the minor salivary glands in 87.3, 84.7 and 84.3% of the cases, respectively. Of the degrees of PIH, only evident and gross PIH are thought to be of true diagnostic value for SS. On the basis of the good agreement between the sonographic and sialographic results, consideration of the introduction of parotid sonography as an alternative to sialography is suggested in SS if the latter method cannot be performed.

Cardiac manifestations in primary Sjögren's syndrome.

OBJECTIVE: To determine cardiac manifestations in primary Sjögren's syndrome (SS). METHODS: Echocardiographic examination was undertaken in 64 patients (62 women, two men) with primary SS (54 definite (DSS) and 10 probable (PSS)) who had systemic symptoms. Twenty one healthy women volunteers of similar age acted as controls. RESULTS: Acute exudative pericarditis occurred in only one patient. An echogenic pericardium was demonstrated in 21 patients (19 DSS, two PSS) (33%) who had a previous symptom free pericarditis, but in none of the controls. Pulmonary pressure was significantly greater in the patients than in the controls (31 (SD 8) mm Hg compared with 24 (7) mm Hg), but there was no significant difference between the DSS and PSS groups. Left ventricular (LV) systolic function was similar in patients and controls. Twenty two patients (20 DSS, two PSS) and one control subject were excluded from LV diastolic function evaluation because of conditions likely to influence the parameters. Of the remaining 42 patients with SS (34 DSS, eight PSS), 21 (17 DSS, four PSS) had impaired diastolic function, confirmed by several diastolic parameters. LV diastolic dysfunction and echogenic pericardium occurred independently of each other, and there was no correlation between the occurrence of these silent cardiac abnormalities and the clinical and laboratory findings. CONCLUSIONS: Obvious cardiac involvement is rare in primary SS, but clinically silent manifestations (symptom free pericarditis and LV diastolic dysfunction) are common. The clinical and prognostic significance of these changes cannot yet be defined.