Niki Tsifetaki

Subgroups of primary Sjögren’s syndrome. Sjögren’s syndrome in male and paediatric Greek patients

OBJECTIVES To describe the clinical and serological findings in male and paediatric Sjögren’s syndrome (SS) patients. PATIENTS AND METHODS Using the European criteria for the diagnosis of SS 12 male and 13 paediatric patients were identified and compared with those of 30 consecutive unselected adult female SS patients. RESULTS The mean (SD) age of paediatric patients was 9.4 (2.2) years, ranging from 6 to 14 years. Recurrent parotid gland enlargement was the initial clinical manifestation in the majority of the children with a statistical significance compared with male (p<0.01) and with female patients (p<0.0001). Sicca manifestations were the most common clinical symptoms in male and female patients at disease onset. The systemic manifestations were similar among the three groups except that men showed lower frequency of arthritis (p<0.05) and Raynaud’s phenomenon (p<0.05) compared with women. No differences were found among the immunological profile of children and female patients, while male patients had a lower frequency of antinuclear antibodies (p<0.025) and antibodies to Ro(SSA) nuclear antigens (p<0.025) compared with women. CONCLUSION Primary SS is rare in children and men in Greece. Recurrent parotid gland enlargement is the most common clinical finding at disease onset in children. Male patients seem to have less systemic manifestations and lower frequency of autoantibodies.

Anxiety and depressive symptoms and illness perceptions in psoriatic arthritis and associations with physical health-related quality of life.

Symptoms of psychological distress, including anxiety and depressive symptoms, and illness perceptions are important in determining outcome in patients with rheumatic disease. We aimed to compare psychological distress in psoriatic arthritis (PsA) and rheumatoid arthritis (RA) and to test whether the association between psychological variables and health‐related quality of life (HRQOL) was similar in the 2 forms of arthritis.

Diagnostic accuracy, internal consistency, and convergent validity of the Greek version of the patient health questionnaire 9 in diagnosing depression in rheumatologic disorders

The Patient Health Questionnaire 9 (PHQ‐9) was developed to screen for depressive disorders in community, primary care, and medical settings. We aimed to estimate its diagnostic accuracy, internal consistency, reliability, and convergent validity in diagnosing major depressive disorder (MDD) in Greek patients with rheumatologic disorders.

The role of psychological distress and personality variables in the disablement process in rheumatoid arthritis

Objective: To test whether psychological distress and personality variables mediate or moderate physical health-related quality of life (HRQOL) in rheumatoid arthritis (RA) patients. Methods: In 168 RA patients the following self-report instruments were administered: the Health Assessment Questionnaire (HAQ), the General Health Questionnaire (GHQ), the Defence Style Questionnaire (DSQ), the Hostility and Direction of Hostility Questionnaire (HDHQ), and the Sense of Coherence (SOC) scale. A total of 152 patients with several rheumatological disorders [56 with systemic sclerosis (SSc), 56 with systemic lupus erythematosus (SLE) and 40 with Sjögrens syndrome (SS)] served as disease controls. The outcome measure was the physical scale of the World Health Organization Quality of Life Instrument, Short Form (WHOQOL-BREF). We used hierarchical regression to determine whether our data were consistent with the disablement process model. Results: In RA patients, sense of coherence was associated with physical HRQOL but the relationship was mediated by psychological distress. Self-sacrificing defence style moderated the relationship between pain and physical HRQOL: pain was associated with impaired physical HRQOL only in patients with predominant self-sacrificing defence style. Although psychological distress and personality variables were also associated with physical HRQOL in the disease control group, the moderating effects of personality on physical HRQOL were unique to RA. Thus, in RA, psychological distress, functional disability, and the interaction term between pain and self-sacrificing defence style were independently associated with physical HRQOL. Conclusions: In RA patients, psychological distress mediated the association of personality variables with physical HRQOL but personality moderated the effects of pain on physical HRQOL and this could be relevant to psychological interventions.

Sicca syndrome in patients with sarcoidosis.

Abstract Out of 134, 12 sarcoidosis patients with symptoms of mucosal dryness as the first clinical manifestation were identified and compared with 30 consecutive unselected Sjögrens syndrome (SS) patients. Sicca manifestations were similar among the two groups, while parotid gland enlargement (PGE) was more frequently found in sarcoidosis patients (P<0.05). Patients with sarcoidosis had mainly pulmonary (P<0.001) and skin involvement (P<0.05), while SS patients presented more frequently with Raynauds phenomenon (P<0.05). Autoantibody profile was more often found in SS patients compared to sarcoidosis (P<0.0025). The histopathological findings of minor salivary gland biopsy (MSGB) revealed noncaseating granulomas (NCG) in 58% of patients with sarcoidosis, while in SS, MSGB showed focal sialadenitis in the majority of the patients. Transbronchial lung biopsy (TBLB), which was performed in 10 sarcoidosis patients, revealed the presence of NCG in all patients. In patients with sarcoidosis and sicca symptoms as the presenting syndrome, PGE is a useful clinical finding. Searching for pulmonary involvement is a determining factor to differentiate sarcoidosis from SS. The absence of autoantibodies is another useful tool for the diagnosis of sarcoidosis. Finally, MSGB is very helpful to discriminate between sarcoidosis and SS and when MSGB is not specific, then TBLB is valuable to confirm the diagnosis.

Subclinical atherosclerosis in scleroderma patients

Objective: To investigate subclinical atherosclerosis in patients with systemic sclerosis (SSc). Methods: Sixty-six patients with SSc who met the American College of Rheumatology criteria for the disease were included. The serum levels of total cholesterol (TC), triglycerides, high density lipoprotein cholesterol (HDL-C), and low density lipoprotein cholesterol (LDL-C) were determined in all patients. Carotid artery intima–media thickness (IMT) and carotid plaques were measured. Patients with a history of atherosclerosis, hypertension, smokers, or patients suffering from conditions that affect the lipid profile, such as diabetes mellitus, hypothyroidism, liver or kidney diseases, Cushings syndrome, obesity, and a history of familial dyslipidaemia, were excluded. Patients receiving medication affecting lipid metabolism were also excluded from the study. Fifty-one age- and sex-matched non-smoking volunteers were used as controls. Results: Sixty patients were investigated. Six were excluded. Of these, two were smokers, two had diabetes mellitus, one hypothyroidism, and one had hypertension treated with diuretics. Patients with SSc exhibited mild dyslipidaemia expressed mainly by low serum levels of HDL-C and high TC (p < 0.001 and p < 0.021, respectively) compared to controls. In addition, the atherogenic ratio LDL-C/HDL-C was significantly higher among SSc patients (p < 0.0001). Common carotid artery IMTs were higher in SSc compared to controls (0.77 ± 0.2 vs. 0.59 ± 0.14, p < 0.0001). No correlation between IMTs and any SSc features were found. Logistic regression analysis showed an independent association of scleroderma with IMTs and TC. Conclusion: The scleroderma patients exhibited an atherogenic lipid profile and subclinical atherosclerosis and have an increased risk for cardiovascular events.

Coping with health-stressors and defence styles associated with health-related quality of life in patients with systemic lupus erythematosus

This study aimed to assess the association of coping with health-stressors and defence styles with health-related quality of life (HRQOL) in systemic lupus erythematosus (SLE). In 56 SLE patients we assessed disease activity (SLEDAI), functional limitations (HAQ), psychological distress (SCL-90-R), defence styles (Defence Style Questionnaire), hostility (HDHQ), coping with health-stressors (Sense of Coherence scale) and HRQOL (WHOQOL-BREF). Two hundred and eight rheumatologic patients (168 with rheumatoid arthritis [RA] and 40 with primary Sjögren’s syndrome [SS]) served as disease controls. SLE patients’ HRQOL was similar to that of patients with RA and primary SS after adjusting for demographic and disease variables. Psychological distress was significantly associated with most aspects of HRQOL, but sense of coherence mediated the relationship of psychological distress with Physical HRQOL; this mediation effect was unique to SLE, as mediation analyses showed. Maladaptive action defence style was also significantly associated with Environment HRQOL independently of psychological distress (p < 0.024). These findings indicate that, apart from the early assessment and treatment of psychological distress, clinicians and consultation–liaison psychiatrists should bear in mind the SLE patients’ psychological resources and coping capacities to deal with the stress of the disease, since such traits, although usually underestimated, are strongly independently associated with HRQOL.

Bosentan for Digital Ulcers in Patients with Systemic Sclerosis: A Prospective 3-year Followup Study

To the Editor: Systemic sclerosis (SSc) is a complex autoimmune connective tissue disease characterized by cutaneous and visceral fibrosis and widespread vascular pathology1. Digital ulcers (DU) are a major clinical problem in SSc, occurring in about one-third of patients2. DU cause local pain and functional impairment and have a negative effect on quality of life for patients with SSc2. Therapeutic agents potentially used for management of DU include calcium channel blockers, α-adrenergic inhibitors, angiotensin II-converting enzyme inhibitors, prostacyclin analogs, phosphodiesterase-5 inhibitor, and others2. Bosentan is a specific orally active dual endothelin receptor antagonist that has been used for the treatment of pulmonary hypertension (PH) and recently for DU3–5. The objective of our study was to examine the effectiveness and safety of bosentan for healing DU in patients with SSc over the long term. For a cohort of 110 patients with SSc, 30 patients with DU were identified. All patients fulfilled the American College of Rheumatology criteria for SSc6 and all were refractory to calcium channel antagonists, angiotensin II inhibitors, or sildenafil. … Address reprint requests to Prof. Drosos. E-mail: adrosos{at}cc.uoi.gr

CNS Involvement in Primary Sjögren Syndrome: Assessment of Gray and White Matter Changes With MRI and Voxel-Based Morphometry

OBJECTIVE The purpose of this study was to evaluate with MRI the involvement of gray matter and white matter structures in patients with primary Sjögren syndrome. SUBJECTS AND METHODS Fifty-three patients with primary Sjögren syndrome, 18 age- and disease duration-matched patients with systemic sclerosis, and 35 age-matched control subjects were examined for differences in white matter hyperintensities (WMHIs) detected on FLAIR MR images. Differences in brain volume between patients with primary Sjögren syndrome and controls were studied by application of voxel-based morphometry to a 3D T1-weighted sequence. RESULTS WMHIs were observed in 38 of the 53 patients with primary Sjögren syndrome, six of 18 patients with systemic sclerosis, and 17 of 35 controls. The numbers of WMHIs 2 mm or larger and the number smaller than 2 mm were higher in patients with primary Sjögren syndrome than in controls (≥ 2 mm, p = 0.004; < 2 mm, p < 0.001). No significant difference was observed in the number of WMHIs in primary Sjögren syndrome patients and that in systemic sclerosis patients. After control for age, a positive relation was found between disease duration and total number of WMHIs (p = 0.037) and number of WMHIs 2 mm or larger (p = 0.023) in patients with primary Sjögren syndrome. In comparison with the controls, patients with primary Sjögren syndrome had decreased gray matter volume in the cortex, deep gray matter, and cerebellum. Associated loss of white matter volume was observed in areas corresponding to gray matter atrophy and in the corpus callosum (p < 0.05). CONCLUSION Patients with primary Sjögren syndrome have WMHIs and gray and white matter atrophy, probably related to cerebral vasculitis.

Comparison of health-related quality of life and associated psychological factors between younger and older patients with established rheumatic disorders

Objectives: To compare health-related quality of life (HRQOL) between younger and older patients with established rheumatic disorders and to assess the relative impact of a number of psychosocial parameters on HRQOL. Methods: In a cross-sectional study of 320 patients (245 < 65 and 75 ≥ 65 years old, response rate: 74.9%) with various rheumatic disorders (rheumatoid arthritis, 168; systemic lupus, 56; scleroderma, 56; and Sjogrens syndrome, 40) attending a follow-up clinic, HRQOL was assessed by the WHOQOL-BREF. Functional limitations (Health Assessment Questionnaire), psychological distress (Symptom Distress Checklist-90-R), defense mechanisms (Defense Style Questionnaire and Life Style Index), sense of coherence, and interpersonal difficulties (Inventory of Interpersonal Problems-40) were also assessed. Results: Older patients presented more impaired physical HRQOL (p = 0.018) and social relationships HRQOL (p = 0.041) independent of disease type, education, and pain. Functional limitations were more prominent in the older group (p = 0.030). Pain, functional limitations, and psychological distress were independently associated with physical HRQOL in both groups. Psychological distress was the only common independent correlate of social relationships HRQOL. Personality factors were significant correlates of physical and social relationships HRQOLs only in the younger group, while the impact of pain in physical HRQOL was greater for younger than older patients, as shown by a moderator analysis. Conclusion: Older patients with rheumatic diseases experience more impaired HRQOL than the younger ones, and the management and prevention of functional limitations and psychological distress should be a priority, since they are strongly associated with HRQOL. Pain also warrants attention in all age groups, but especially in younger patients. Personality factors impact on HRQOL in younger patients, and this might be relevant to psychological interventions.