Evaluation of Ocular Surface and Meibomian Glands in Patients With Scleroderma.

PURPOSE: To evaluate the prevalence of dry eye and meibomian gland dysfunction in patients with scleroderma. METHODS: A total of 32 patients with scleroderma (study group) and 31 healthy subjects (control group) were enrolled. Besides routine ophthalmologic evaluation, Schirmer 1 test, tear break-up time, tear osmolarity, ocular surface staining with fluorescein (Oxford score), and Ocular Surface Disease Index (OSDI) score, meibomian gland dysfunction evaluation (foamy tears, telangiectasia of the eyelid, eyelid contour abnormalities, and meibomian plugs), and meibography were performed. RESULTS: Sixty-four eyes of 32 patients with scleroderma and 62 eyes of 31 healthy individuals were evaluated. Mean ages of the study group was 48.34 ± 9.73 years (21-62 years) and of the control group was 45.84 ± 4.42 years (38-54 years) (P = 0.067). Mean duration of systemic sclerosis or scleroderma in study group was 9.78 ± 7.40 years (1-30 years). Mean tear break-up time was shorter in study group than that in the control group (P < 0.0005). No statistical differences were detected for osmolarity, OSDI score, and Schirmer 1 values between groups (P = 0.051, P = 0.053, and P = 0.358, respectively). The prevalence of grade 1 and higher Oxford score was higher in the study group (P < 0.0005). Upper meiboscores of grade 1 and higher were found to be more common in the study group than those in the control group (P = 0.036). The presence of foamy tears and telangiectasia of the eyelids were significantly higher in the study group (P = 0.002 and P = 0.002, respectively). OSDI score was the only significantly correlated data with disease duration (Spearman ρ coefficient = 0.396, P = 0.001). CONCLUSIONS: Evaporative type dry eye is more common in patients with scleroderma than the healthy population.

Cigarette smoking in primary Sjögren's syndrome: positive association only with ANA positivity.

Smoking is well known to contribute to the pathogenesis and severity of some systemic autoimmune rheumatic diseases and especially to the production of certain autoantibodies. Primary Sjögren's syndrome (pSS) is an autoimmune disease, affecting primarily the exocrine glands. It may also cause extraglandular involvement in some cases. In this study, we aimed to determine the frequency of smoking habits in our cohort of pSS patients and to investigate whether the frequencies of autoantibody positivity and extraglandular involvement were significantly different between patients with and without smoking. In this cross-sectional study, 207 patients with pSS (F/M 203/4), fulfilling the United States-European Consensus Criteria, and 602 healthy controls (F/M 534/68) were included. Patients and controls were classified into five groups: never smokers, current smokers, former smokers; ever smokers, and passive smokers. The χ(2) and Kruskal-Wallis tests were used for statistical analysis; a p value of less than 0.05 was accepted as statistically significant. While the frequency of current smokers was significantly lower in the pSS group compared with the healthy controls (11.6 vs 22.3%), the frequencies of former smokers (30.4 vs 11.8%), ever smokers (42.0 vs 34.1%), and passive smokers (47.3 vs 37.5%) were significantly higher in the pSS group compared with the healthy controls. In pSS patients, only antinuclear antibody (ANA) positivity was significantly associated with smoking habits, while there was no significant association with other autoantibodies or with the presence of extraglandular involvement. We found that in pSS patients smoking was significantly associated only with ANA positivity. Unlike the deleterious effects of smoking upon disease severity and anti-cyclic citrullinated protein (CCP) antibody production in rheumatoid arthritis, we could not find any association of smoking with extraglandular involvement and/or anti-Ro/anti-La antibody positivity in pSS. These results are indeed in line with the limited number of previous studies reported in the literature. Further studies with higher numbers of pSS patients are required to confirm the seemingly negative association of smoking with pSS.

Corneal hysteresis, corneal resistance factor, and intraocular pressure measurement in patients with scleroderma using the reichert ocular response analyzer.

PURPOSE: The Reichert ocular response analyzer (ORA) measures corneal biomechanical properties in vivo by monitoring and analyzing the corneal behavior when its structure is submitted to a force induced by an air jet. This study was designed to examine corneal biomechanical properties and intraocular pressure in patients with systemic sclerosis (SSc) and to compare with control eyes. PATIENTS AND METHODS: ORA measurements were performed on the right eyes of 29 patients with SSc (group 1) and 29 healthy people who served as the control group (group 2). Corneal hysteresis, corneal resistance factor (CRF), and intraocular pressure [Goldmann correlated (IOPg) and corneal compensated] were recorded with ORA. RESULTS: Mean age of patients with SSc and control groups were 51.7 +/- 11.1 and 50.3 +/- 10.8 years, respectively. Mean (+/-SD) of the corneal hysteresis and CRF readings were 9.8 +/- 1.7 versus 9.5 +/- 1.2 mm Hg (P > 0.05) and 10.0 +/- 1.5 versus 9.2 +/- 1.4 mm Hg (P < 0.05), in groups 1 and 2, respectively. Mean (+/-SD) of the IOPg and intraocular pressure corneal-compensated recordings were 15.9 +/- 2.5 versus 14.1 +/- 2.4 mm Hg (P < 0.05) and 16.9 +/- 3.2 versus 15.6 +/- 2.9 mm Hg (P > 0.05), in groups 1 and 2, respectively. Statistical analysis revealed significant differences for CRF and IOPg between the study groups. CONCLUSIONS: The mean CRF and IOPg values of patients with SSc were higher when compared with normal controls. According to the results of our study, one can conclude that corneal biomechanical properties would be changed in patients with SSc and this can be determined by CRF.

Efficacy of SLZ and MTX (alone or combination) on the treatment of active sacroiliitis in early AS.

Sacroiliitis is an important sign of spondylarthritis (SpA) of which the prototype disease is ankylosing spondylitis. The radiographic changes required for diagnosing AS occur as late as 8-11 years after the onset of clinical symptoms. Nonsteroid anti-inflammatory drugs (NSAIDs) have been the main treatment for spondylitis of AS. For patients refractory or intolerant to NSAIDs, disease-modifying antirheumatic drugs (DMARDs) have been used as a second-line approach. Sulphasalazine (SLZ) is known as the best DMARD in treatment of peripheral arthritis; also methotrexate (MTX) is currently one of the most widely used DMARDs. But there was no objective information about inflammation of sacroiliac joints during treatment with these DMARDS that are the first places of the beginning point of SpA. For this purpose, in this study, the effect of SLZ and MTX, which are used alone and combination in 6 months, on treatment of active sacroiliitis, which is shown by dynamic magnetic resonance and acute phase reactants in laboratory has been investigated. 55 patients (F:M = 34:21) with active sacroiliitis [mean age = 37.05 + 13.03 year (n = 55)] were evaluated and determined by dynamic magnetic resonance imaging in this study. The better response in the SLZ treatment group than the other two groups has been obtained. Nevertheless, those changes were not statistically found different. In conclusion, the ratio of treatment of active sacroiliitis, especially early period, with SLZ as a DMARD is better than MTX or MTX + SLZ, but this difference is not statistically significant. A prospective study of the treatment of active sacroiliitis by DMARDs may be more illustrative.

Radiological incomplete thymus involution in systemic sclerosis.

OBJECTIVE: Thymus plays a crucial role in immune system homeostasis, and thymic abnormalities have been previously reported in many autoimmune diseases, including SSc. The aim of this study is to evaluate the frequency of radiological thymus abnormalities in SSc patients and its relationship with various clinical and laboratory features. METHODS: Sixty-three female SSc patients (diffuse/limited: 49/14), all having pulmonary high-resolution CT (HRCT) scans, taken previously for evaluating lung involvement were included. At the time of the scans, mean age and disease duration of the patients were 50.1 +/- 8.5 and 10.2 +/- 7.8 years, respectively. As the control group, 45 age-matched female patients, having normal pulmonary HRCT scans taken previously for evaluating non-specific symptoms, were included. RESULTS: Frequency of incomplete thymus involution was significantly higher in SSc patients (12/63; 19%) compared with the control group (2/45; 4.4%; P = 0.022). In SSc patients with pulmonary fibrosis, incomplete thymus involution was significantly lower (3/38; 7.9%) than those without pulmonary fibrosis (9/25; 36%; P = 0.007). CONCLUSION: The present study shows significantly higher frequency of radiological incomplete thymus involution in SSc compared with normal controls. Furthermore, less common occurrence of pulmonary fibrosis in SSc patients with incomplete thymus involution deserves attention. These findings may have some implications regarding the possible role of thymic abnormalities at least in some patients with SSc.

Hypothermia during the infusion of cryopreserved autologous peripheral stem cell causes electrocardiographical changes: report of two cases.

Currently, autologous peripheral stem cell transplantation used as a therapeutic modality in the treatment of various hematological malignancies is gaining more popularity day by day. In this method, the patient's own peripheral stem cells are collected by a proper method and stored at -80 degrees C until they are reinfused into the patient after being rewarmed in water bath at 37 degrees C. A number of complications have been reported related to reinfusion of the cryopreserved cells into the patient. These may include noncardiovascular complications such as nausea, vomiting, flushing, abdominal pain, chest discomfort, and headache, as well as cardiovascular complications like arrhythmias, hypotension, and hypertension. Hypothermia related to rapid infusion has been reported as the main factor underlying the cardiovascular complications. Electrocardiographic findings of hypothermia include sinusal bradycardia, prolonged QT and PR intervals, widened QRS complexes, and J wave, which is a ECG abnormality characterized by supraventricular and ventricular arrhythmias. We here present two cases of giant J wave caused by hypothermia during infusion of cryopreserved autologous peripheral stem cell that is detected by ECG and regressed after infusion ceased.