Assessment of autonomic symptoms in diabetics: the Swedish version of the Autonomic Symptom Profile.

Objectives: Autonomic dysfunction (AD) is a complication of diabetes and may be associated with troubling symptoms and increased mortality. Commonly, AD is detected by objective physiological tests and only recently, a validated self-completed English questionnaire assessing AD symptoms, the Autonomic Symptom Profile (ASP), was developed. The aims of this study were to translate the ASP into Swedish and evaluate its reliability and validity.

Autonomic nervous symptoms in primary Sjogren's syndrome.

Objectives: Objective signs of autonomic dysfunction (AD) have been reported in patients with primary SS (pSS) while the presence of associated symptoms has not been systematically studied. Therefore, the aims of this study were (i) to assess the presence and severity of various AD symptoms in pSS patients and (ii) to relate AD symptoms to other clinical features of pSS.

Methods: Thirty-eight pSS patients and 200 population-based controls were studied for presence and severity of AD symptoms using the Autonomic Symptom Profile (ASP), a validated self-completed questionnaire evaluating various AD symptoms.

Diagnostic and prognostic significance of measuring antibodies to alpha-fodrin compared to anti-Ro-52, anti-Ro-60, and anti-La in primary Sjögren's syndrome.

Objective: To compare sensitivity and specificity of autoantibodies to alpha-fodrin with conventional anti-Ro and anti-La antibodies in patients with primary Sjögren's syndrome (pSS). Data on internal organ manifestations were correlated with presence of autoantibodies.

Methods: We collected clinical and laboratory data from 321 patients with pSS (Copenhagen criteria), of which 205 fulfilled the new American-European 2002 consensus criteria.

Dysphagia and dysmotility of the pharynx and oesophagus in patients with primary Sjögren's syndrome.

Objectives: To assess the prevalence of pharyngeal and oesophageal symptoms and dysmotility in patients with primary Sjögren's syndrome (pSS) and relate these to autonomic nervous function.

Methods: Twenty consecutive pSS patients, according to the American-European Consensus Criteria (AECC), and 30 age- and sex-matched controls from the Swedish general population registry were studied. All subjects completed a pharyngeal and oesophageal symptoms questionnaire and were examined by pharyngeal and oesophageal video radiography.

Autonomic and orthostatic dysfunction in primary Sjögren's syndrome.

Objective: Exocrine function always is and autonomic nervous function may be impaired in primary Sjögren's syndrome (pSS). Since autonomic nervous signaling is a prerequisite for exocrine secretion we wanted to assess autonomic nervous function in pSS and relate it to diagnostic measures of exocrine function.

Methods: Autonomic nervous function was determined in 46 patients with pSS using the deep breathing test [expiration/inspiration (E/I) ratio], orthostatic test [acceleration index (AI), orthostatic systolic and diastolic blood pressure response (lSBP ratio and lDBP ratio)], and finger skin blood flow test [vasoconstrictory (VAC) score].

Progress and regression within primary Sjögren's syndrome.

The purpose of this review is to give a modern view and an update of important areas in primary Sjögren's syndrome (SS), which may be the most common of the autoimmune systemic rheumatic diseases. Interest in aspects of primary SS including clinical manifestations, pathogenesis, aetiology, treatment, prognosis, etc has increased during the past three decades, the volume of scientific papers and the number of theses being the indicators. However, only a fraction of the money that is used for research into rheumatoid arthritis (RA) is used for SS, and the statement that SS is under-diagnosed, under-treated and under-researched will still be valid for several years to come.

Lymphoma and other malignancies in primary Sjögren's syndrome: a cohort study on cancer incidence and lymphoma predictors.

Objectives: To assess the risk of lymphoproliferative disease or other malignancy (standardised incidence ratios (SIRs)), in patients with primary Sjögren's syndrome according to the American-European Consensus Criteria (AECC), compared with patients with sicca syndrome (non-AECC) and the background population. To identify predictors of malignancy and describe lymphoma types and survival probabilities.

Methods: A linked register study using information from the Malmö Primary SS Register, Swedish Cancer Register, and Cause-of-Death Register for calculation of SIRs was carried out.

Proposed core set of outcome measures in patients with primary Sjögren's syndrome: 5 year follow up.

Objective: To clarify the spontaneous course of important disease manifestations (a core set of outcome measures) over a period of 5 years in patients with primary Sjögren's syndrome (SS), and to analyze predictors of unfavorable outcome. To test the usefulness of the recently proposed core set of outcome measures.

Methods: A cohort of patients with primary SS according to the American-European consensus criteria (AECC) (n = 58) was followed over a period of 5 years.

Clinical manifestations correlated to the prevalence of autoantibodies in a large (n=321) cohort of patients with primary Sjögren's syndrome: a comparison of patients initially diagnosed according to the Copenhagen classification criteria with the American-European consensus criteria.

In this study we imposed the recently described American-European consensus criteria for primary Sjögren's syndrome (pSS) on a large cohort of patients originally classified according to the Copenhagen set of criteria. Of the 321 patients fulfilling the Copenhagen criteria, 205 conformed to the Consensus criteria. When comparing clinical manifestations and laboratory findings between the two groups defined by different standards we found only small variations.

Immunohistochemistry of the B-cell component in lower lip salivary glands of Sjögren's syndrome and healthy subjects.

Serial sections of lower lip salivary gland (LSG) biopsies were examined by immunohistochemistry, using a battery of B- and partly T-related antibodies (CD5, CD20, CD21, CD27, CD38, CD45RO, CD79a, Bcl-2 and Bcl-6) in different groups of subjects: healthy controls and clinically verified smoking or nonsmoking cases of primary Sjögren's syndrome (SS). The purpose was to characterize the B-cell pattern of the lymphocytic foci and of the tiny perivascular infiltrates preceding the development of foci. Hyperplastic tonsil was used as stain control.

Late neonatal lupus erythematosus onset in a child born of a mother with primary Sjögren's syndrome.

Background: The neonatal lupus syndrome can be present as congenital heart block (CHB) or as neonatal lupus erythematosus (NLE), both seldom passively acquired autoimmune diseases. CHB starts around week 20 of pregnancy and is a lifelong event, whereas NLE is self limiting and usually starts at the 6th week of the child's age-the maximum recorded up to week 20.

Case Report: An asymptomatic mother with primary Sjogren's syndrome and anti-SSA/Ro52, anti-SSA/Ro60, and anti-SSB/La autoantibodies is described who, at gestational week 23 during her first pregnancy, was diagnosed as having a male fetus with CHB due to third degree atrioventricular block.

Enhanced DNA-dependent protein kinase activity in Sjögren's syndrome B cells.

Objective: To examine the stress response, including the role of DNA-dependent protein kinase (DNA-PK), in B cells from Sjögren's syndrome (SS) patients.

Methods: B-cell lines were exposed to gamma radiation and then postincubated to allow inducible stress functions to develop. The magnitude of the DNA damage response was monitored with respect to DNA-PK phosphorylation of a p53 peptide, defence protein levels (Ku, DNA-PK catalytic subunit, ATM, p21 and p53) and flow cytometric determination of cell cycle phases and apoptosis.

CD4+ T-lymphocytopenia--a frequent finding in anti-SSA antibody seropositive patients with primary Sjögren's syndrome.

Objective: Case reports have described an association between idiopathic CD4+ T-lymphocytopenia (ICL) and non-Hodgkin's malignant lymphoma (NHML), and both entities have an increased prevalence in patients with primary Sjögren's syndrome (SS). We investigated lymphocyte subset counts in patients with primary SS to determine if presence of different autoantibodies is associated with ICL and hence may represent an increased risk for development of NHML.

Methods: A total of 80 patients with primary SS according to the American-European Consensus Classification Criteria (AECC) and 37 non-AECC sicca patients were studied for presence of different autoantibodies, and lymphocyte subsets were investigated by flow cytometry.

Mortality and causes of death in primary Sjögren's syndrome: a prospective cohort study.

Objective: This study was undertaken to analyze standardized mortality ratios (SMRs) and causes and predictors of death in primary Sjögren's syndrome (SS) diagnosed according to 3 different classification criteria sets (the Copenhagen criteria, the European criteria, and the American-European consensus criteria (AECC).

Methods: A linked registry study using information from the Malmö Primary SS Registry combined with the Swedish Cause-of-Death Registry was performed, and SMRs were calculated. Kaplan-Meier survival curves and log rank tests were used to compare survival probability between subgroups of patients with primary SS.

Physical capacity in women with primary Sjögren's syndrome: a controlled study.

Objective: To examine physical capacity (aerobic capacity, joint mobility, muscle function, and standing balance) in women with primary Sjögren's syndrome (primary SS) and to examine the correlation of aerobic capacity with fatigue, functional disability, and mental aspects (anxiety and depression).

Methods: Fifty-one women who fulfilled the European Community criteria for primary SS and who had anti-SSA/SSB antibodies or a positive lip biopsy were compared with 51 age-matched controls. Physical capacity, fatigue, functional disability, anxiety, and depression were investigated by means of questionnaires and clinical examinations.

Fibrosis regression induced by intravenous gammaglobulin treatment.

Objectives: To review case histories of patients in whom fibrosis played a significant role in the pathogenesis of their disease, and to determine whether intravenous gammaglobulin (IVIg) contributed to the regression of their fibrotic condition.

Methods: Eight patients with excess fibrotic reaction in the course of diverse diseases were analysed; a tendency that reverted with different IVIg treatment options. Myelofibrosis was predominant in three patients (a patient with a myeloproliferative syndrome, one with systemic lupus erythematosus, and one with Sjögren's syndrome).

Gammalinolenic acid treatment of fatigue associated with primary Sjögren's syndrome.

Objective: To evaluate the efficacy of the essential omega-6 fatty acid Gammalinolenic acid (GLA) on fatigue associated with primary Sjögren's syndrome.

Methods: Ninety patients with primary Sjögren's syndrome (with or without signs of autoimmunity) entered a 6-month double blind placebo-controlled randomised trial with high dose GLA (extracted from Evening Primrose Oil) or corn oil. The primary outcome parameter was fatigue; secondary endpoints were eye dryness, mouth dryness, muscle and joint pain.

Enhanced DNA damage-induced p53 peptide phosphorylation and cell-cycle arrest in Sjögren's syndrome cells.

Background: Cells from primary Sjögren's syndrome (SS) patients have been reported to show alterations in DNA repair and p53 expression. The DNA-dependent protein kinase (DNA-PK) autoantigen may be involved in both of these alterations in relation to cellular DNA damage responses. We conducted this study of cell-cycle kinetics and p53 to find additional evidence for an abnormal stress response role in the pathogenesis of SS.

Surface activity of tear fluid in patients with primary Sjögren's syndrome.

Rupture of the preocular tear film leads to formation of a dry spot on the cornea with ocular irritation and symptoms of dry eye. One of the factors determining the stability of the tear film is its surface activity. The purpose of this study was to examine the surface activity of tear fluid from patients with Sjögren's syndrome.

Exposure of HEp-2 cells to stress conditions influences antinuclear antibody reactivity.

This study of stress-related antinuclear antibody (ANA) reactivity was undertaken with the objective of improving clinical ANA testing. ANA was determined by parallel enzyme-linked immunosorbent assays of crude nuclear protein antigen extracted from HEp-2 cells either grown under optimal conditions (providing nonstress ANA antigen) or exposed to stress (providing stress ANA antigen). The stress stimuli used were gamma radiation (causing DNA damage) and a hypertonic environment (causing apoptosis).