Evolving phenotype of systemic lupus erythematosus in Caucasians: low incidence of lupus nephritis, high burden of neuropsychiatric disease and increased rates of late-onset lupus in the 'Attikon' cohort.

OBJECTIVE: This study aimed to analyse the phenotype of systemic lupus erythematosus (SLE) at first presentation and during follow-up in a newly established SLE cohort based at 'Attikon' University Hospital. The hospital combines primary, secondary and tertiary care for the region of Western Attica, Greece. METHODS: This study comprised a mixed prevalent and incident cohort of 555 Caucasian patients diagnosed with SLE according to American College of Rheumatology 1997 criteria and/or the Systemic Lupus Erythematosus International Collaborating Clinics (SLICC) 2012 criteria. Demographic and clinical characteristics, patterns of severity, treatments and SLICC damage index were recorded for each patient at the time of diagnosis and at last evaluation. RESULTS: The mean age at lupus diagnosis was 38.3 years (standard deviation = 15.6 years), with a median disease duration at last follow-up of two years (interquartile range 1-11). At initial presentation, the most common 'classification' manifestations were arthritis (73.3%), acute cutaneous lupus (65%) and unexplained fever (25%), while among symptoms not included in any criteria set, Raynaud's phenomenon (33%) was the most common. Kidney and neuropsychiatric involvement as presenting manifestations were present in 10.3% and 11.5% cases, respectively. Irreversible damage accrual was present in 17.8% within six months of disease diagnosis, attributed mainly to thrombotic and neuropsychiatric disease. At last evaluation, 202 (36.4%) patients had developed severe disease, of whom more than half were treated with pulse cyclophosphamide. CONCLUSION: In this cohort of Caucasian patients, lupus nephritis is not as common as in older cohorts, while neuropsychiatric disease is emerging as a major frontier in lupus prevention and care. These data may help to document changes in the natural history and treatment of SLE over time and may have implications for its early recognition and management.

The biology of normal bone remodelling.

During life, bone undergoes modelling and remodelling in order to grow or change shape. Bone modelling is the process by which bones change shape or size in response to physiologic influences or mechanical forces that are encountered by the skeleton, while bone remodelling takes place so that bone may maintain its strength and mineral homeostasis. During early childhood, both bone modelling (the formation and shaping of bone) and bone remodelling (the replacement or renewal of old bone) occur. The predominant process in childhood is bone modelling, while in adulthood bone remodelling predominates. The exception to this is after a fracture when we see massive increases in bone formation. During childhood and adolescence growth occurs in the bones longitudinally and radially, while in the growth plates it occurs longitudinally, thus promoting growth in size. Cartilage first proliferates in the epiphyseal and metaphyseal areas of long bones before undergoing mineralisation to form new bone.

No association of psoriasis with autoimmune thyroiditis.

BACKGROUND: Common autoimmune diseases tend to coexist in the same patients. Few studies have examined the possible association between autoimmune thyroiditis and psoriasis or psoriatic arthritis (PsA), with inconsistent results. OBJECTIVE: To investigate the prevalence of autoimmune thyroiditis in psoriatic patients with or without PsA, living in an iodine-sufficient area. METHODS: We studied prospectively, 114 psoriatic patients with disease duration of 5-38 years, 30 of them with PsA, and 286 age- and body mass index (BMI)-matched subjects without psoriasis or known thyroid disease or autoimmune disease. A detailed medical history was obtained from all participants and clinical examination and laboratory evaluation was performed. Psoriasis severity was assessed with Psoriasis Area and Severity Index (PASI). Autoimmune thyroiditis was defined by the presence of positive autoantibodies to thyroid peroxidase and/or thyroglobulin. RESULTS: There was no difference in the prevalence of autoimmune thyroiditis between psoriatic patients and controls (20.2% vs. 19.6%). The prevalence of autoimmune thyroiditis in male and female psoriatic patients was similar (9.6% and 10.5% respectively), in contrast to the increased, as expected, prevalence in female vs. male controls (14.7% vs. 4.9%, P < 0.01). Detected cases with hypothyroidism due to autoimmune thyroiditis were similar in psoriatic patients and controls (7.9% and 7.0% respectively). Autoimmune thyroiditis in psoriatic patients was not related with age of psoriasis onset, psoriasis duration, PASI score, PsA and obesity. CONCLUSION: These data support that psoriatic patients with or without PsA do not have an increased risk for autoimmune thyroiditis.

Prevalence of psoriatic arthritis and its correlates among patients with psoriasis in Greece: results from a large retrospective study.

OBJECTIVES: To evaluate the prevalence and its clinical characteristics of psoriatic arthritis (PsA) in a specialized psoriasis clinic of a University Hospital. METHODS: In this retrospective study, 278 patients with psoriasis were evaluated between 2011 and 2013. RESULTS: The study included 278 patients with psoriasis: 144 (52%) were male and 134 (48%) female. Their median age was 51.41 with median psoriasis presenting age of 34.52 years. Referring to the type of psoriasis, 86% presented with plaque psoriasis, 5% guttate, 2% palms and soles, 2% inverse, 1% pustular and 4% with psoriasis of more than one type. Nail disease appeared in 121 patients (43.5%) and scalp disease in 175 (63%). Of these patients, 85 (30%) had PsA, whereas 51% of patients with PsA had psoriatic nail disease. With reference to the PsA type, 43 (51%) patients presented with polyarthritis, 10 (12%) with oligoarthritis, 7 (8%) with axial arthritis, whereas the rest 25 of them (31%) had PsA of more than one type. The subgroup of patients with PsA had significantly higher rates of comorbidities including arterial hypertension, diabetes and hypercholesterolaemia compared to non-PsA patients with 41% vs. 17% (P = 0.001), 20% vs. 8% (P = 0.021) and 41% vs. 19% (P = 0.004), respectively. CONCLUSION: The prevalence of PsA among patients with psoriasis was relatively higher in Greece compared to other ethnic-based studies. Comorbidities related to life expectancy were more frequent. As there is a high percentage of undiagnosed cases with active arthritis among patients with psoriasis, dermatologists should be aware of PsA clinical signs in order to recognize it earlier and provide successful treatment.

Association of soluble apoptotic markers with impaired left ventricular deformation in patients with rheumatoid arthritis. Effects of inhibition of interleukin-1 activity by anakinra.

Myocardial function is impaired in rheumatoid arthritis (RA). Inhibition of interleukin (IL)-1 activity reduces experimental myocardial infarction by limiting apoptosis. We investigated whether a) soluble apoptotic markers are related with impaired left ventricular (LV) performance and b) treatment with anakinra, an IL-1 receptor antagonist, reduces apoptotic markers leading to improved LV performance in RA. We studied 46 RA patients. In an acute, double-blind cross-over trial, 23 patients were randomised to a single injection of anakinra or placebo and after 48 hours (h) to the alternative treatment. In a chronic trial, 23 patients who received anakinra for 30 days were compared with 23 patients who received prednisolone. At baseline, 3 h and 30 days after treatment, we measured circulating IL-1ß, tumour necrosis factor (TNF)-α, Fas, Fas-ligand and caspase-9 to assess apoptosis. At baseline and 30 days after treatment, we assessed LV longitudinal strain, strain rate and E/Em ratio using 2D-speckle tracking and tissue Doppler echocardiography. At baseline, increased apoptotic markers were related with reduced LongSRS and increased E/Em (p<0.05). After 3 h and 30 days of anakinra, there was a reduction in Fas (median 481 vs. 364 vs. 301 pg/ml), Fas-ligand (median 289 vs. 221 vs. 190 pg/ml), caspase-9 (median 1.90 vs. 1.40 vs. 1.07 ng/ml), TNF-α and IL-1ß (p<0.05 for all comparisons). E/Em, LongS and LongSRS were improved after anakinra (p<0.01) and their percent changes were related with the corresponding changes of Fas and caspase-9 (p<0.05). No changes of the examined parameters were observed after prednisolone. In conclusion, inhibition of IL-1 activity by anakinra reduces apoptotic markers leading to improved LV performance in RA.

Lowering interleukin-1 activity with anakinra improves myocardial deformation in rheumatoid arthritis.

OBJECTIVE: Inhibition of interleukin-1 activity improves nitro-oxidative stress, endothelial and coronary function. The authors investigated (a) the association of nitro-oxidative stress and endothelial function with myocardial deformation, (b) the effects of anakinra, an interleukin-1a receptor antagonist on myocardial deformation in patients with rheumatoid arthritis (RA). METHODS: The authors compared 46 RA patients to 23 normal controls. 23 patients received anakinra (150 mg subcutaneously once daily) and 23 patients a 5-mg increase of prednisolone dose for 30 days. At baseline and post-treatment this study assessed (a) the left ventricular (LV) longitudinal, circumferential and radial strain and strain rate, using speckle tracking echocardiography, (b) the coronary flow reserve (CFR), (c) the flow-mediated endothelial-dependent dilation of the brachial artery (FMD) and (d) nitrotyrosine (NT) and malondialdehyde blood levels. RESULTS: Patients had impaired baseline myocardial deformation indices compared to controls (p<0.05). CFR and NT levels were related to longitudinal strain, systolic and diastolic strain rate, circumferential strain and systolic strain rate (p<0.05). FMD was related to longitudinal and circumferential diastolic strain rate (p<0.01). Compared to baseline, anakinra-treated patients increased the longitudinal strain (-17.8% (3.7%) vs -22.1% (3.5%)), systolic (-1.02 (0.23) l/s vs -1.25 (0.23) l/s) and diastolic (0.96 (0.37) l/s vs 1.20 (0.39) l/s) longitudinal strain rate, circumferential strain and strain rate (p<0.05 for all comparisons). No significant changes were observed among prednisolone-treated patients CONCLUSIONS: Myocardial deformation is impaired in RA patients and is related to nitro-oxidative stress and endothelial dysfunction. Chronic inhibition of IL-1 improves LV deformation in parallel with endothelial function and nitro-oxidative stress.

Gender and age differences in systemic lupus erythematosus. A study of 489 Greek patients with a review of the literature.

We investigated whether gender and age influence the clinical course and outcome in systemic lupus erythematosus (SLE) patients. Thus, we analyzed the clinical and laboratory data of 489 SLE patients at presentation and during follow-up. In addition, disease activity score (using the European Consensus Lupus Activity Measure, ECLAM) and organ damage index (using the Systemic Lupus International Collaboration Clinics/American College of Rheumatology Damage Index, DI) were measured. Furthermore, data from both sexes were analyzed according to the following age groups: < 55 years(younger group) and > 55 years (older group). There were 68 men and 421 women, giving a ratio of 1:7. We found no differences in the mean age, mean age at diagnosis, disease duration as well as duration of follow-up between men and women. Young men presented more frequently with serositis and discoid lesions, while women presented with Raynaud's phenomenon (RP) and malar rash. Regarding the laboratory findings, young women presented more often with anti-Ro(SSA) and anti-La(SSB) antibodies, while increased levels of erythrocyte sedimentation rate (ESR) were found in old women. During follow-up, men had serositis and renal disease more frequently, while the women's group were found to complain of RP, photosensitivity and mucosal ulcers more frequently, especially in young women.Anemia, leukopenia, thrombocytopenia and elevated levels of ESR were also found more frequently in young women during follow-up. However, there were no significant differences concerning ECLAM and DI scores between the two gender groups. Using multiple logistic regression analysis, a statistically significant association of malar rash, discoid lesions, serositis, RP, anti-Ro(SSA)/ La(SSB) and increased ESR with sex was found independently of age, while only malar rash showed a statistically significant association with age independently of sex. Thus, we conclude that gender influences the clinical expresion of the disease independently of age, while both gender and age do not affect the overall damage score.

Clinical course and outcome of early rheumatoid arthritis.

We studied whether patients with seropositivity in early rheumatoid arthritis (RA) comprise a different clinical group than those with seronegativity. Four hundred seventeen patients with early RA according to the American College of Rheumatology criteria (disease duration less than 1 year) were retrospectively studied by analysis of demographic, clinical, laboratory, radiological, and therapeutic disease characteristics from the time of diagnosis until the end of the study period (1981 1999) using a data base. There were 248 seropositive patients and 169 seronegative patients with RA. No statistically significant differences were seen between the two groups before commencement of the study period in relation to age of disease onset, male:female ratio, and disease duration. However, seropositive patients showed longer medical follow-up. In addition, at disease onset, seropositive RA patients presented more frequently with symmetrical polyarthritis and small joint involvement than seronegative patients. The seropositive group also had more tender and swollen joints, weaker grip strength, and higher erythrocyte sedimentation and C-reactive protein rates during the follow-up period. In contrast, the seronegative group had less severe radiological findings and greater functional ability at the end of the study. In Greek patients with early RA, rheumatoid factor seems to be a predictor of more severe disease activity.

Outcome of lupus pregnancy: a controlled study.

OBJECTIVE: The reciprocal relationship between systemic lupus erythematosus (SLE) and pregnancy was investigated in a controlled study. METHOD: The outcome of 47 pregnant SLE patients with 59 pregnancies was compared with that of 57 healthy control women and 59 pregnancies. The results were also compared with those of 59 non-pregnant control SLE patients. RESULTS: All pregnant SLE patients but one were in remission at the onset of pregnancy and were being treated with low doses of prednisone (< or = 10 mg/day, 26 patients), hydroxychloroquine (200 mg/day, eight patients) or azathioprine (100 mg/day, one patient). Sixty-one per cent of SLE pregnancies were delivered at term and 5% had premature deliveries. The rates of spontaneous abortion and total fetal loss were significantly higher in the mothers with SLE than in the control population (P: < 0.001 and P: < 0.01 respectively). None of the 39 neonates from SLE mothers had neonatal lupus, anti-Ro(SSA) or anti-La(SSB) antibodies. Eight out of 59 pregnancies of SLE mothers (13.5%) were characterized by disease exacerbation. Arthralgias or arthritis, fever and skin lesions were observed more frequently in the mothers with SLE than in the non-pregnant group (P: < 0.001). Renal involvement was found in three SLE patients during pregnancy and in three after delivery. CONCLUSIONS: Pregnant women with SLE are at high risk of fetal loss and spontaneous abortion. Pregnancy does not cause life-threatening manifestations of the disease. Thus, for a better outcome of lupus pregnancy, it is essential to control disease activity and to achieve clinical remission.

CT features of gastric lymphoma.

The gastrointestinal tract is the most frequent site of extranodal involvement by lymphoma and stomach is the most frequently involved. The incidence of gastric lymphoma appears to be rising as that of adenocarcinoma is declining. It produces a spectrum of radiologic appearances and its distinction from adenocarcinoma is difficult since their findings frequently overlap. The aim of this article is to present pictorially a spectrum of CT findings of the gastric lymphoma.

Prevention of chemotherapy-induced alopecia using an effective scalp cooling system.

Alopecia is a distressing side-effect of cancer treatment. Taxanes (TX), anthracyclines (ANR) and etoposide (ET) have been consistently associated with significant alopecia. We studied an effective scalp cooling system, the Penguin Cold Cap system, for the prevention of chemotherapy-induced alopecia in 70 patients receiving chemotherapy, including one of the following major alopecia-causing agents: Group A, TX-based regimes (without ANR); Group B, TX+ANR; Group C, ANR-based regimes (without TX); Group D, ET-based regimes. Protection from hair loss was achieved by maintaining scalp temperatures below 15 degrees C before, during and after chemotherapy by frequent changing of the caps. Assessment was carried out using a grading system from 0 to 4. Grades 0-2 were considered as satisfactory hair protection, whilst Grades 3-4 were considered failures. 57 patients were evaluable for assessment. An overall 81% protection was achieved. In groups C and D 11 of 12 patients (92%) had no alopecia, whilst 30 of 34 patients (88%) treated with taxanes had adequate hair protection. In Group B, 4 of 11 patients (36%) had adequate hair protection. The system was well tolerated and is a very effective method for protection from hair loss caused by TX, ANR and ET. Our results are comparable with and, in most cases, better than those reported in other studies using various alopecia preventive methods.

Treatment of fibromyalgia with tropisetron, a 5HT3 serotonin antagonist: a pilot study.

In this pilot study we investigated 10 women suffering from primary fibromyalgia. All patients received 5 mg of tropisetron in the evening, for a period of 4 weeks. Clinical disease variables included the measurement of a pain score, fatigue, sleep disturbances and measurement of the number of tender points. Five of our patients (50%) showed a statistical clinical improvement of all the above parameters starting after the first week of treatment. Two patients did not respond to the therapy and three discontinued the study because of side-effects. We conclude that administration of tropisetron in fibromyalgia patients could be useful in the management of this difficult and incurable syndrome.

Endobronchial metastases secondary to solid tumors: report of eight cases and review of the literature.

Endobronchial metastases (EBM) secondaries to extrapulmonary solid malignant tumors are rare. Breast, colon and renal adenocarcinomas are the most frequent tumors associated with EBM. Since 1990 we have treated eight patients with EBM secondary to renal adenocarcinoma (three cases), colon adenocarcinoma (two cases), gastric adenocarcinoma (one case), bladder carcinoma (one case) and basal cell carcinoma (one case). Endobronchial lesions were detected by bronchoscopy and their metastatic nature was confirmed histopathologically in all eight cases. We also conducted a review of EBM reporting studies published in English language. The median interval from the diagnosis of the primary tumour was 41 months. Symptoms and radiological findings were indistinguishable from those of primary lung cancer. Five patients were treated with external radiotherapy with symptomatic improvement while two patients had chemotherapy and one patient underwent surgical resection of the metastasis. Systemic treatment was used in six cases with no significant effect on EBM. Median survival after EBM diagnosis was 9 months with one patient surviving 3.5 years and two patients still alive at 1 year. In conclusion, EBM usually represent a late manifestation requiring differential diagnosis from a primary lung cancer. Local treatment may result in symptomatic improvement but prognosis is generally poor averaging 1-2 years in most series.