Fahrettin Oksel

Ankylosing spondylitis-related secondary amyloidosis responded well to etanercept: a report of three patients

Secondary (AA) amyloidosis is one of the most significant complications of ankylosing spondylitis (AS) that frequently leads to proteinuria and renal dysfunction. Anti-tumor necrosis factor alpha (anti-TNF) agents are promising in inducing clinical remission by suppressing systemic inflammation in AA amyloidosis. We report three cases with AS-related AA amyloidosis that responded well to etanercept therapy. Despite treatment with disease modifying anti-rheumatic drugs, all three patients had active AS, marked proteinuria, impaired renal function, and low serum albumin level. During 1-year treatment with etanercept, all patients experienced gradual improvement in all of these parameters.

TWO SIBLINGS WITH JUVENILE HYALINE FIBROMATOSIS : CASE REPORTS AND REVIEW OF THE LITERATURE

Abstracts: In this paper, we describe two siblings with Juvenile Hyaline Fibromatosis (JHF) who were diagnosed at the age of 34 and 29 years respectively. JHF is a very congenital disease, mainly diagnosed in the first few years of life, with less than 40 published cases in literature. All the main clinical features of this syndrome, which may be summarised as multiple subcutaneous tumours, marked gingival hypertrophy, flexion contractures and osteolytic lesions were present in both of these cases. Clinical, radiological and histological differential diagnosis of JHF were made. Recent information about histopathology, treatment and prognosis of JHF was also reviewed.

Detection of Microembolic Signals in Patients with Neuropsychiatric Lupus erythematosus

The pathogenesis of central nervous system involvement in systemic lupus erythematosus (SLE) is not completely understood. In this study, we investigated the association of microembolic signals (MES) with a variety of neuropsychiatric SLE manifestations and compared our results with those from SLE patients without neuropsychiatric lupus and normal controls. Fifty-three patients with SLE (45 females and 8 males), all fulfilling the revised classification criteria for SLE, and 50 control subjects (44 females and 6 males) were enrolled in this study. All SLE patients were assessed by neuropsychological examination, including various neuropsychiatric tests. Twenty-five patients with SLE were found to have at least one of the neuropsychiatric syndromes defined by The American College of Rheumatology. The mean MES count in patients with neuropsychiatric lupus was significantly higher than those without (5.4 ± 1.1 vs. 0.3 ± 0.8/h; p < 0.005). We found a positive correlation between higher mean MES counts and the presence of neuropsychiatric syndromes in SLE. The mean MES count in the whole group of SLE patients was also significantly higher than that in healthy controls. The mean MES count of SLE patients with antiphospholipid (aPL) antibody positivity was significantly higher than those without aPL antibodies (3.6 ± 1.6 vs. 0.8 ± 0.1/h; p < 0.005). In conclusion, the association of MES with neuropsychiatric lupus may support the possible contribution of MES to the complex pathophysiology of this syndrome. More importantly, detection of MES on transcranial Doppler monitoring might suggest a high risk of involvement of the central nervous system in SLE, and could be used as a diagnostic tool.

Platelet-activating factor and P-selectin activities in thrombotic and nonthrombotic Behçet's patients

ObjectiveThe aim of this study was to compare plasma Platelet-activating factor (PAF) and P-selectin (CD62P) activities in Behçet’s disease patients with and without thrombosis.MethodsIn this cross-sectional and descriptive study, 30 consecutive Behçet’s patients were included, 15 of them with venous thrombosis. All patients were also divided into two subgroups according to the presence or absence of clinical activity. Plasma PAF levels, basal and Ca++ ionophore (A23187)-induced leukocyte (cellular) PAF activities, and platelet-rich plasma ΔCD62P activity (the mean fluorescent density difference between CD62P phycoerythrin-positive and -negative stains) were evaluated.ResultsIn the thrombotic group, plasma PAF (P=0.001), basal leukocyte PAF (P=0.017), induced leukocyte PAF (P=0.024), and ΔCD62P (P=0.023) levels were significantly higher than in the nonthrombotic group. In the whole group of Behçet’s patients, there was a positive correlation between plasma PAF and ΔCD62P levels (r=0.533, P=0.002). When we compared clinically active and inactive patients with respect to the above parameters, there was no significant difference, irrespective of thrombosis. Plasma PAF (P=0.001), basal leukocyte PAF (P=0.004), and ΔCD62P (P=0.038) levels were significantly higher in the presence of both clinical activity and thrombosis than of clinical activity alone.ConclusionPlatelet-activating factor and CD62P may contribute to endothelial injury and thrombosis development in Behçet’s disease. These two parameters seem related to the presence of thrombosis rather than clinical activity.

The frequency of sicca symptoms and Sjögren's syndrome in patients with systemic sclerosis.

The objectives are to detect the frequency of sicca symptoms and Sjögrens syndrome (SS) in patients with systemic sclerosis (SSc) based on the diagnostic criteria of the American–European Consensus Group (AECG) and to evaluate demographic, clinical and serologic characteristics.

SERUM PROLACTIN LEVELS IN BEHCET'S SYNDROME

Abstract: Since prolactin (PRL) has been implicated as playing a role in the pathogenesis of certain autoimmune diseases and since Behçet’s Syndrome (BS) is a unique systemic vasculitis, we investigated serum PRL levels in patients with BS. We found that mean PRL levels in patients with clinically active BS, were not significantly higher than patients with clinically inactive BS and healthy controls. This finding may be regarded as evidence that a contribution of hyperprolactinemia to the aetiopathogenesis of BS seems unlikely.

Transcranial Doppler detection of microembolic signals in patients with Behçet’s disease

Abstract We evaluated the prevalence of microembolic signals (MES) in patients with Behçet’s disease (BD). We also attempted to determine the frequency of MES in BD patients with or without neurological involvement. This study enrolled 55 patients fulfilling the diagnostic criteria of International Study Group for BD. Bilateral transcranial Doppler ultrasound of the middle cerebral arteries was performed. MES were identified based on the criteria of International Consensus group on Microembolus Detection. Patients with BD were divided into two groups in respect of the presence of neurological involvement (n = 10) or not (n = 45), and counts of MES in the two were compared with each other and with normal subjects. We found MES in 16 patients (29%) with BD. The frequency was higher in patients with neurological involvement than in those without (80% vs. 17%, P < 0.001). In patients with neurological involvement there was a positive correlation in regression analysis between the prevalence of MES and disease duration (P = 0.025). There was a significantly higher prevalence of MES in BD patients than in control subjects. The frequency of MES was higher in patients with neurological involvement than in those without. TCD detection of MES may allow the recognition of subset of patients at high risk for the appearance of neurological involvement.

Coexistence of systemic lupus erythematosus, Hashimoto’s thyroiditis and IgA nephropathy in the same patient

Autoimmune disorders encompass a wide spectrum of diseases that progress with several clinical findings. They can be organ-specific (such as Hashimoto’s thyroiditis) or they can involve multiple organs (such as SLE). The common characteristic of all these disorders is the production of different autoantibodies against various autoantigens along with inflammation. IgA nephropathy is the most common non-lupus glomerulopathy. It rarely coexists with SLE and has never been reported to coexist with Hashimoto’s thyroiditis. In this case report, a female patient diagnosed with SLE and Hashimoto’s thyroiditis and whose renal biopsy revealed IgA nephropathy is presented.

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.

Cyclosporine A-induced neck fibrosis in a patient with adult-onset Still’s disease

Cyclosporine A (CsA) is an immunosuppressive agent used for the prevention of graft rejection during organ and bone marrow transplantation. CsA is also used for the treatment of various inflammatory rheumatic diseases. Although different side effect profiles have been reported, nephrotoxicity, renal vascular damage, hypertension, and gingival hypertrophy are among the most commonly encountered side effects. The development of massive fibrosis in the neck associated with CsA in a 30-year-old male patient with Still’s disease is presented herein. Significant regression was observed after the discontinuation of CsA.