G. Pokorny

Cardiac manifestations in primary Sjögren's syndrome.

OBJECTIVE: To determine cardiac manifestations in primary Sjögrens 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.

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

OBJECTIVE Signs of a parasympathetic dysfunction have been revealed in primary Sjögrens 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.

Antineutrophil cytoplasmic antibodies in patients with systemic lupus erythematosus

Summary  We determined the prevalence of antineutrophil cytoplasmic antibodies (ANCAs) in patients with systemic lupus erythematosus (SLE) and evaluated the correlation between ANCA positivity and clinical features. Forty‐one patients with SLE and two control groups were examined. One of the control groups consisted of 15 patients with systemic vasculitis, and the other of 12 healthy blood donors. A quantitative enzyme‐linked immunosorbent assay technique was used to measure the serum cytoplasmic ANCA (cANCA) and perinuclear ANCA (pANCA) levels. cANCA positivity was found in three patient samples, and pANCA positivity in 10 SLE patients. The occurrence and titres of both ANCA types in SLE patients were similar to those in healthy controls and significantly lower than those in patients with systemic vasculitis. The clinical picture and antibody profile were similar in ANCA‐positive and ANCA‐negative SLE patients. We conclude that measurement of ANCAs does not provide any additional diagnostic or prognostic data in SLE.

Types of atrophic gastritis in patients with primary Sjögren's syndrome.

Histological examination of the gastric mucosa was performed in 44 patients with primary Sjögrens syndrome with extraglandular symptoms (mean age 51.9, range 22-76). Biopsy specimens were taken from each of three separate regions: the antrum, the corpus, and the transitional zone between the antrum and the corpus. The incidence of chronic atrophic gastritis was considerably higher in patients with Sjögrens syndrome than in the controls. In the young patients with Sjögrens syndrome atrophic lesions were more common both in the antrum and in the corpus than in the control group. In middle aged patients, however, only the antrum, and in the elderly only the corpus, was much more commonly affected than in the controls. All three types of chronic atrophic gastritis occurred in patients with Sjögrens syndrome. Decreased gastric acid secretion was associated mainly with atrophic gastritis of types A and AB, whereas hypergastrinaemia occurred almost exclusively in gastritis of type A.

HLA class II allele polymorphism in Hungarian patients with systemic lupus erythematosus

Recently, a comprehensive study was published on HLA class II DNA typing in a large cohort of European patients with SLE.1 Independently of this collaborative study, we have examined similar DRB1, DQA1, and DQB1 allele polymorphisms and clinical features in Hungarian patients with SLE. Fifty patients with SLE (48 female; mean age at the time of the examinations 41 years (range 21–76)) and 50 healthy blood donors matched for age and sex with the controls were examined. Genotyping of HLA-DRB1 alleles was carried out with the Dynal RELI SSO HLA-DRB kit, and the DRB1*15/16 subtyping by the method of Ota et al .2 DQA1 determination was performed by the method of Ota et al .3 The DQB1 …

Association between autoimmune pancreatitis and systemic autoimmune diseases

AIM To investigate the association between autoimmune pancreatitis (AIP) and systemic autoimmune diseases (SAIDs) by measurement of serum immunoglobulin G4 (IgG4). METHODS The serum level of IgG4 was measured in 61 patients with SAIDs of different types who had not yet participated in glucocorticosteroid treatment. Patients with an elevated IgG4 level were examined by abdominal ultrasonography (US) and, in some cases, by computer tomography (CT). RESULTS Elevated serum IgG4 levels (919 ± 996 mg/L) were detected in 17 (28%) of the 61 SAID patients. 10 patients had Sjögrens syndrome (SS) (IgG4: 590 ± 232 mg/L), 2 of them in association with Hashimotos thyroiditis, and 7 patients (IgG4: 1388 ± 985.5 mg/L) had systemic lupus erythematosus (SLE). The IgG4 level in the SLE patients and that in patients with SS were not significantly different from that in AIP patients (783 ± 522 mg/L). Abdominal US and CT did not reveal any characteristic features of AIP among the SAID patients with an elevated IgG4 level. CONCLUSION The serum IgG4 level may be elevated in SAIDs without the presence of AIP. The determination of serum IgG4 does not seem to be suitable for the differentiation between IgG4-related diseases and SAIDs.

Neuroimmune interactions in Sjögren's syndrome: relationship of exocrine gland dysfunction with autoantibodies to muscarinic acetylcholine receptor-3 and mental health status parameters.

Objectives: Antimuscarinic acetylcholine receptor-3 (m3AChR) autoantibodies have been described in primary Sjögren’s syndrome (pSS). The aim of this study was to compare various methods for their detection and to assess the contributions of anti-m3AChR and other immunological and psychosocial factors to the pathomechanism of secondary SS (sSS). Methods: Sixty-five rheumatoid arthritis (RA) patients, 103 systemic lupus erythematosus (SLE) patients, 76 pSS patients and 50 controls were compared. Three immunodominant epitopes of m3AChR were synthesized and used in ELISA. Two extracellular epitopes were also prepared in fusion with glutathione-S-transferase and one in conjugation with bovine serum albumin. Mental health status was assessed with the 36-item Short-Form Health Survey and Functional Assessment of Chronic Illness Therapy fatigue scale. Correlations were evaluated between glandular function and anti-m3AChR positivities and specificities, features of SLE and RA, and mental health parameters. Results: Fourteen RA and 27 SLE patients had sSS. The autoantibody levels to all epitopes of m3AChR were significantly higher in pSS and SLE patients than in the controls. The fusion protein forms discriminated RA from pSS and SLE; furthermore, the YNIP fusion protein also distinguished pSS from SLE. The prevalence and the mean levels of all autoantibodies did not differ statistically between sicca and non-sicca SLE or RA patients. Glandular dysfunction correlated with higher age in SLE and RA and an impaired health-related quality of life in SLE. Conclusions: The second and third extracellular loops of m3AChR are antigenic in pSS. Immunoassays with antigens as fusion peptides demonstrate the best performance. Sicca SLE patients have worse mental health status. Anti-m3AChR antibodies represent a peculiar example of neuroimmune interactions.

Fatal cases of disseminated nocardiosis: Challenges to physicians and clinical microbiologists - Case report

Despite the development in the identification of Nocardia spp., common challenges exist in the laboratory diagnosis and management of nocardiosis. We report two cases of disseminated nocardiosis in a patient with hematologic disorder and in a patient with systemic lupus erythematosus, where the cooperation between various specialists was essential to set up the adequate diagnosis of disseminated nocardiosis.