Primary Sjögrens syndrome presenting as neuromyelitis optica.

This report presents a patient with Devics neuromyelitis optica associated with primary Sjögrens syndrome. Her first attack was right-sided optic neuritis at age 10 years. Attacks involving both optic nerves and medulla spinalis were recorded during the ensuing years. The diagnosis of Sjögrens syndrome could not be made until the second decade because it was not suspected.

Hodgkin's lymphoma following treatment with etanercept in ankylosing spondylitis.

It has been well known that anti-TNF drugs might increase lymphoma risk in rheumatoid arthritis (RA), where the rate of lymphoma has already been increased. However, unlike RA, an increased rate of lymphoma has not been reported in ankylosing spondylitis (AS). Hereby, we present a case with AS developing Hodgkin's lymphoma (HL) following 6 months of etanercept treatment. Pathological analysis revealed mixed cellular type of HL. Although we report a single case, it should be kept in mind that anti-TNF drugs might cause lymphoma development not only in RA, but also in AS.

A comparison of four disability scales for Turkish patients with neck pain.

OBJECTIVE: The Neck Disability Index, the Northwick Park Pain Questionnaire, the Copenhagen Neck Functional Disability Scale and the Neck Pain and Disability Scale are widely used scales for assessing neck pain or disability. The aim of this study was to determine the most suitable scale for Turkish patients with neck pain. METHODS: All scales were translated into Turkish, administered to 102 patients with neck pain, then compared with regard to their construct validity, reliability, responsiveness, acceptability and usefulness. RESULTS: The scales were similar in their high validity, reliability standards and sensitivity to change, but differed in their acceptability and usefulness. The item about driving in the Neck Disability Index and the Northwick Park Pain Questionnaire was omitted by 69.6% of patients for reasons other than a neck problem. CONCLUSION: All scales were reliable, valid and sensitive instruments, with similar psychometric properties. The scale that most adequately reflects the patient should be chosen.

Laryngeal findings and voice quality in Sjögren's syndrome.

OBJECTIVE: The effect of Sjögren syndrome (SS) on perceptual ratings of the laryngeal findings using the Reflux Finding Score (RFS) and the Reflux Symptom Index (RSI) and the objective voice quality using Jitter (JITT), Pitch Period Perturbation Quotient (PPQ), Shimmer (Shim), Amplitude Perturbation Quotient (APQ) and Noise-to-Harmonic Ratio (NHR) was examined. METHODS: Seventy-seven patients with SS and seventy-seven healthy individuals for the control group were studied. The Reflux Symptom Index (RSI); nine-item outcomes instrument for assessment of symptoms in patients and the Reflux Finding Score (RFS), eight-item laryngoscopic-based scale for evaluation of laryngeal findings in patients were realised. The Multi Dimensional Voice Program (MDVP) was used for capturing and analysis of the voice samples. For comparison of all parameters of patients and control group subjects, the independent sample t-test was used. RESULTS: The difference of RSI and RFS between patients with SS and control subjects was statistically significant. The difference of voice quality parameters between patients with SS and control subjects expect NHR were statistically significant. CONCLUSION: There is a significant association between SS and a variety of laryngeal pathologies. The SS patients with reflux symptoms and voice problems must be examined by an ENT specialist and local laryngeal manifestations of SS can be treated symptomatically.

Telomerase activity in connective tissue diseases: elevated in rheumatoid arthritis, but markedly decreased in systemic sclerosis.

Telomerase is a reverse transcriptase enzyme contributing to the maintenance of the telomeric structure by adding telomere repeat sequences to chromosomal ends, thus compensating for its shortening. Telomerase activity which is common in cancers and human germ line tissue, may also be increased, although to a lesser extent, in systemic autoimmune diseases. We aimed to evaluate telomerase activity in a group of Turkish patients with various connective tissue diseases. In this cross sectional study, 19 patients with systemic sclerosis (SSc), 15 with systemic lupus erythematosus (SLE), 10 with rheumatoid arthritis (RA) and 14 with primary Sjögren's syndrome (pSjS) were studied. As the control group, 29 healthy subjects were also included. Human telomerase-specific reverse transcriptase (hTERT) was measured in peripheral blood lymphocytes, using online real-time reverse-transcriptase polymerase chain reaction (PCR). We also investigated if hTERT values in each patient group were correlated with clinical parameters and disease activity. Highest hTERT values were observed in RA group (21.24 +/- 28.54), followed by SLE (13.38 +/- 26.05) and pSjS (11.73 +/- 10.59) groups. Only hTERT values in RA group was significantly higher than the healthy control group (7.62 +/- 4.21) (p < 0.05). Interestingly, hTERT values in SSc were very low (2.09 +/- 3.18), even less than the healthy control group. In consistent with previous studies, telomerase activity was increased in SLE and RA. Very low telomerase activity in SSc group was rather surprising. Since existing telomerase data in SSc was limited and telomere shortening was previously reported in SSc, our finding of low telomerase activity in SSc group deserves relevant discussion and further studies.

Evaluation of t-PA, PAI-2, IL-1beta and PGE(2) in gingival crevicular fluid of rheumatoid arthritis patients with periodontal disease.

AIMS: This study was undertaken to compare periodontal conditions, gingival crevicular fluid (GCF) levels of tissue-type plasminogen activator (t-PA), its inhibitor plasminogen activator inhibitor-2 (PAI-2), interleukin-1beta (IL-1beta), prostaglandin E(2) (PGE(2)) in rheumatoid arthritis (RA) patients and control groups. METHODS: Twenty-three RA patients, 17 systemically healthy patients with periodontal disease (PD), and 17 systemically and periodontally healthy subjects were recruited. GCF samples were obtained from two single-rooted teeth. Full-mouth clinical periodontal measurements were recorded at six sites/tooth. GCF samples were analysed using relevant ELISA kits. Data were tested statistically by appropriate tests. RESULTS: Total amounts of t-PA, PAI-2 and PGE(2) in GCF samples of the healthy control group were significantly lower than the other groups (p<0.05). The RA group exhibited a higher total amount of t-PA in GCF samples than the PD group (p<0.05). PAI-2, IL-1beta and PGE(2) total amounts were similar in RA and PD groups (p>0.05). CONCLUSION: The coexistence of RA and periodontitis does not seem to affect clinical periodontal findings or systemic markers of RA. Similar inflammatory mediator levels in RA and PD groups, despite the long-term usage of corticosteroids, non-steroidal anti-inflammatory drugs, suggest that RA patients may have a propensity to overproduce these inflammatory mediators.

Ochronotic arthropathy: disappearance of alkaptonuria after liver transplantation for hepatitis B-related cirrhosis.

The deficiency of homogentisic acid oxidase, an enzyme that is mainly found in hepatocytes, is associated with alkaptonuria and ochronosis. We report a patient with clinical and radiologic findings of ochronotic arthropathy in whom alkaptonuria disappeared and the progressive course of the disease stopped after liver transplantation for hepatitis B-related cirrhosis.