The frequency of arthritis in Adamantiades-Behçet's disease in Greek patients.

BACKGROUND/OBJECTIVES: Musculoskeletal manifestations are frequent in Adamantiades-Behçet's disease (ABD) but only represent non-specific clinical findings. They have not been included in the two commonly used sets of classification criteria. The occurrence of musculoskeletal manifestations at ABD onset may even delay or obscure the diagnosis; therefore, detailed knowledge of the different musculoskeletal manifestations is essential. Our objective was to describe musculoskeletal signs and their clinical course in Greek ABD patients. METHODS: We conducted a retrospective cohort study, which included all patients with ABD, who had been examined in our Rheumatology Outpatient Division from 1995 to 2010. The study included 224 ABD patients (140 male, 84 female) that fulfilled the International Criteria for the diagnosis of BD. For statistical analysis, we have used chi-square and Fisher's exact tests. RESULTS: Arthritis as a presenting sign was seen in 10.2% of our patients. During the follow-up period, the frequency of arthritis was 58.4%. Monoarthritis was found in 32.8% and 22.6% of male and female patients, respectively (ns). During the follow-up period, polyarthritis was only occasionally observed in male patients (2.14%). Oligoarthritis was assessed in 20.0% and 41.6% of male and female patients, respectively (P < 0.001), and was the only significantly different manifestation between sexes. CONCLUSIONS: Musculoskeletal manifestations are common in ABD both at presentation and during the disease course. The most frequent sign is mooarthritis. Oligoarthritis was the only significantly different articular manifestation between sexes (more common in women) in our study group.

Toll-like receptors pathways implication in common autoimmune diseases and therapeutic perspectives.

Toll-like receptors (TLRs) are a category of receptors that recognize and activate their signaling pathways to defend against the pathogen factors. However, an alteration in the proper activation might occurr, resulting in the production of proinflammatory cytokines. This improper activation is the beginning of an autoimmune disease. The inhibition of the implicated receptors or their pathways may prevent the induction of autoimmunity. This paper describes in detail new therapeutic opportunities based on the alteration of the TLR activation for rheumatoid arthritis and systemic lupus erythematosus.

Cardiac sarcoidosis.

Sarcoidosis is a systemic disease which affects many organs, including the heart. Cardiac sarcoidosis has a reported incidence of about 25 % and carries a poor prognosis. It can occur in the form of conduction abnormalities, pericardial and valvular heart disease, congestive heart failure, arrhythmias and sudden cardiac death. The diagnosis of cardiac sarcoidosis is difficult, requiring a high index of suspicion and the use of electrocardiography, echocardiography, nuclear medicine imaging, myocardial biopsy and magnetic resonance imaging. Corticosteroids have been the cornerstone of treatment of cardiac sarcoidosis, but other immunosuppressives have also been used, along with standard heart failure therapy, antiarrhythmic medications, pacemakers and implantable defibrillators. Cardiac transplantation is an option for patients who do not respond to medical treatment. We briefly review the current armamentarium for the diagnosis and treatment of cardiac sarcoidosis.

Taxane Induced Cystoid Macular Edema: Case Report and Integrated Pathogenic Theory.

PURPOSE: To report a case of a 73-year-old man who presented with decreased visual acuity due to bilateral macular edema after paclitaxel administration for prostate cancer. METHODS: The ophthalmic evaluation consisted of medical and ocular history, Best Corrected Visual Acuity, slit-lamp biomicroscopy and Spectral-domain optical coherence tomography / Fluorescein Angiography. RESULTS: Optical Coherence Tomography and Fluorescein Angiography revealed silent cystoid macular edema. After consulting with the oncologist, the cessation of paclitaxel therapy was decided. The patient presented a gradual but steady resumption of the retinal edema, with complete restoration of normal retinal morphology and function within two months. The pathogenesis of the silent Cystoid Macular Edema (CME) is still unclear. Based on our case and a critical review of the previous observations and published data, we propose that the underlying cause of Taxane induced CME is the functional failure of Aquaporin mediated water transport at the level of retinal Intermediate and Deep capillary plexuses, and at lesser extent at the level of the Retinal Pigment Epithelium. CONCLUSION: Taxane induced silent CME should be attributed to the action of Taxanes on the microtubule guided aquaporin vesicles transport to the cell membrane. In our case of Taxane induced silent CME, withdrawal of the taxane was enough for complete recovery, and no additional treatment was needed.

OCT-Angiography for monitoring and managing neovascular age-related macular degeneration.

PURPOSE: To evaluate the combined use of optical coherence tomography and angiography (OCT-A) for imaging choroidal neovascularization (CNV) secondary to neovascular age-related macular degeneration (nAMD). MATERIALS AND METHODS: This prospective observational study was conducted from May 2015 to April 2017. Included in the study were 54 patients (n = 63 eyes), all of whom had CNV secondary to nAMD and all of whom had been examined by OCT-A. Angioscans (3x3 and 6 × 6) and conventional B-scan OCT scans were obtained for all patients at baseline and at various times during the 24-month follow-up period. For diagnostic confirmation, conventional imaging methods fluorescein angiography (FA) and indocyanine green angiography (ICGA) were performed at baseline. A total of 13 patients (n = 15 eyes) underwent serial imaging during 34 follow-up visits. The main outcomes included (i) determination of OCT-A sensitivity for the detection of CNV (classic and occult) and (ii) the correlation between B-scan OCT and OCT-A vis-à-vis consecutive follow-up changes. RESULTS: At baseline, the detection rate (i.e., overall sensitivity) of OCT-A for detecting CNV was 64.4% (75.7 and 48.0% for classic and occult CNV, respectively), independent of prior treatment status. In terms of quality, 6 × 6 angioscans were superior to 3 × 3. Moreover, specific CNV morphologic patterns by B-scan OCT did not correlate with lesion composition. Correspondence between OCT-A and B-scan OCT was observed in only 53% of the cases. CONCLUSIONS: OCT-A may prove to be a valuable adjunctive diagnostic tool for the interpretation of CNV, as it not only reduces the need for invasive angiographic procedures but also facilitates the follow-up process.

Large pericardial cyst in an asymptomatic child: a case report.

We present a case report of a nine-year-old asymptomatic girl with a large cystic lesion of the left hemithorax, occupying almost 80% of the left hemithorax, first believed to be a parasitic cyst that was operated and proved to be of parietal pericardial origin. Mediastinal cysts and pericardial cysts in particular are mostly discovered accidentally and so was the case with our patient. The diagnosis included not only clinical examination but also the utilization of computed tomography and magnetic resonance imaging. The only treatment for pericardial cysts is surgical excision and patients recover fully with no complication.

Dysphagia as the presenting symptom of thyroid cancer.

A 72 year old man presented with progressive dysphagia. A poorly differentiated thyroid carcinoma occluding the esophagus was diagnosed. The trachea was initially unaffected. This mode of presentation for a thyroid carcinoma is very uncommon.

Raynaud's phenomenon is correlated with elevated systolic pulmonary arterial pressure in patients with systemic lupus erythematosus.

In patients with Systemic lupus erythematosus (SLE), Raynaud phenomenon (RP) is frequently present and associated with pulmonary hypertension (PHT). Elevated pulmonary artery systolic pressure (PASP) is an indicator of PHT and can be estimated noninvasively. We attempt to explore the significance of RP in SLE and to correlate it with clinical and serological parameters of the disease. The study population consisted of 34 patients (age, sex and disease duration matched) who fulfilled the revised SLE criteria of the American College of Rheumatology, and were categorized into two groups: Group 1 had patients having SLE and RP (2 males/15 females, mean age 45 +/- 18 years) and group 2 had patients with SLE but without RP (3 males/14 females, mean age 40 +/- 14 years. Detailed cardiac ultrasound was performed including measurement of PASP, while clinical and serological features of both groups were collected and correlated. Significant differences were shown in the presence of arterial hypertension (P < 0.05), arthralgias (P < 0.005), arthritis (P < 0.05), myalgias (P < 0.05), alopecia (P < 0.05) and PASP (P < 0.0001). No difference was observed among the cardiac ultrasound indices and the ejection fraction between the two groups. PASP was significantly correlated with RP, while no correlation was observed regarding the disease duration. In patients with SLE, the presence of RP was associated with elevation in PASP. Further investigation is needed to clarify the significance of this relation.