Eker Doganavsargil

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.

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.

Pulmonary hypertension in rheumatoid arthritis.

Using Doppler echocardiography (DE), we measured pulmonary arterial systolic pressure (PASP) in rheumatoid arthritis (RA) patients without coexisting cardiopulmonary diseases. Accepting the normal upper limit of PASP as 30 mmHg, we found elevated PASP in 11 out of 40 (27.5%) RA patients, values being mostly 30–40 mmHg, indicating mild pulmonary hypertension (PHT). Although estimation of PASP by DE is not as reliable as cardiac catheterisation, it is possible that mild elevations in PASP may contribute to the high incidence of cardiovascular events not explained by traditional cardiac risk factors in patients with RA. Long‐term follow‐up will be obviously necessary to ascertain the impact of mild PHT on the prognosis and mortality rate of RA patients.

Manometric assessment of esophageal motility in patients with primary Sjögren’s syndrome

ObjectiveThe aim of this study was to assess the esophageal motility by manometry in patients with primary Sjögren’s syndrome.MethodsEsophageal manometry was carried out in 40 patients with primary Sjögren’s syndrome (SS), 15 with rheumatoid arthritis (RA), 15 with RA and secondary SS, and 21 healthy volunteers.ResultsWe found that the mean lower esophageal sphincter (LES) pressures measured by station pull-through and rapid pull-through techniques were significantly higher in primary SS patients than with healthy controls and RA patients with or without SS (P<0.05). Our study did not show any major differences when comparing the three patient groups (P>0.05). However, peristaltic contraction velocity was lower and peristaltic contraction duration significantly higher at the middle and lower thirds of the esophagus in primary SS patients than in healthy controls (P<0.05).ConclusionThe results of our study support the view that various esophageal motility disorders can be found in patients with primary SS which could be related to an increase in LES pressure. We also found no correlation of the esophageal abnormalities with other factors studied, suggesting that the cause of dysphagia is multifactorial in nature.

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.

The plasma levels of activated thrombin activatable fibrinolysis inhibitor and thrombomodulin in Behçet disease and their association with thrombosis.

OBJECTIVE To investigate the plasma levels of activated thrombin activatable fibrinolysis inhibitor (aTAFI) and thrombomodulin (TM) in Behçet disease (BD) and their relationship with thrombosis. METHODS Plasma aTAFI and TM levels were measured by ELISA in 89 patients with BD (18 having venous thrombosis) and in 86 healthy controls. RESULTS Compared with healthy controls, the BD group had significantly lower levels of aTAFI (13.49+/-8.88 microg/ml vs. 26.76+/-11.57 microg/ml, p<0.0001) and significantly higher levels of TM (3.26+/-1.85 ng/ml vs. 2.6+/-0.69 ng/ml, p=0.0003). Neither aTAFI, nor TM levels differed significantly between BD patients with and without thrombosis (p>0.05). Despite a tendency to positive correlation (r=0.37, p=0.0004) between plasma levels of aTAFI and TM in healthy controls, there was a tendency for negative correlation (r=-0.51, p<0.0001) between these two parameters in BD patients. CONCLUSION The plasma aTAFI and TM levels do not seem to be related with the presence of thrombosis observed in BD. Increased plasma TM levels in BD may simply reflect endothelial cell activation and dysfunction.

Increased excretions of glycosaminoglycans and heparan sulfate in lupus nephritis and rheumatoid arthritis

Urinary glycosaminoglycans (GAG) and heparan sulfate (HS) are considered to be markers of early renal involvement. This study was undertaken to demonstrate their excretion patterns in rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE) with and without arthritis. Serum creatinine and urinary GAG, HS, microalbumin, and creatinine measurements were made in 51 biopsy-proven lupus nephritis (LN) cases, 12 RA patients, and 21 healthy controls. Urinary GAG and HS levels were higher in the LN and RA groups than in controls. Heparan sulfate excretions and SLE disease activity index (SLEDAI) scores were no different between SLE patients with classes 1 and 2 (group A) and those with classes 3, 4, and 5 (group B) renal involvement. However, GAG and microalbumin excretions were significantly high in the latter. There were no differences in GAG and HS excretions between normoalbuminuric, microalbuminuric, and macroproteinuric SLE patients or between those with and without arthritis. In conclusion, urinary GAG and HS, being unrelated to the presence of arthritis, are independent markers of LN. Extrarenal causes or subclinical renal involvement may be responsible in RA due to their increased excretion in these patients.

Hypothalamus-Hypophysis-Thyroid Axis, Triidothyronine and Antithyroid Antibodies in Patients with Primary and Secondary Sjo¨gren’s Syndrome

Abstract: It has been well established that, anti-thyroglobulin antibodies (ATG) and anti-microsomal antibodies (AMC) may be present in various thyroid disorders and other systemic autoimmune diseases, including Sjo¨gren’s syndrome (SS). However, presence of circulating autoantibodies to thyroid hormones, i.e. both to triiodothyronine (T3) and tetraiodothyronine (T4), has not been studied extensively in SS. Autoantibodies to T3 and T4 are very important, because serum T3 and T4 levels may be detected spuriously higher or lower, due to the presence of these autoantibodies. Their presence should be suspected when measured serum thyroid hormone levels are not consistent with clinical status of the patient. SS is a slowly progressive, inflammatory autoimmune disease, affecting primarily the exocrine glands. Thyroid gland, being a target in some autoimmune diseases, is well known to be affected in SS as well. Keeping this possibility in mind, we investigated T3 autoantibody levels and thyroid gland involvement in patients with SS.  Twenty-six SS patients (F/M:22/4) with a mean age of 46,6 years, were recruited in this study.Twelve of them were accepted as primary SS (pSS), while others had secondary SS (sSS) (7 with rheumatoid arthritis (RA), 3 with systemic lupus erythematosus (SLE), 3 with progressive systemic sclerosis (PSS) and 1 with sarcoidosis). Thyroid function tests, including T3, T4, fT3, fT4, TSH, ATG, AMC, T3 antibody measurements, thyroid scintigraphy, thyroid ultrasonography and TRH stimulation tests were performed in all patients. We compared our results with those of the twenty healthy normal controls.  Serum ATG and/or AMC were detected in three patients with pSS (25%) and no patients with sSS. No significant difference could be shown in the other parameters, including T3 autoantibodies and thyroid function tests. TRH stimulation test was also normal, showing that the hypothalamus-hypophysis-thyroid axis was not affected in patients both with pSS and sSS.  In conclusion, we found that T3 autoantibody levels in pSS, were not significantly higher than sSS and normal controls.

An Unusual Presentation of Behçet’s Disease: Intestinal Perforation

Abstract: Behçet’s disease (BD), when first described in 1937, consisted of three symptoms: recurrent oral and genital ulcerations and iridocyclitis [1]. Today, it is known that BD is a multisystemic chronic vasculitic disorder which may involve both arteries and veins of all sizes, as well as the central nervous and gastrointestinal systems. The rate of gastrointestinal involvement of BD varies in different populations, being more common in Japan (50%–60%) and less common in the Mediterranean basin, including Turkey (0%–5%) [2,3]. We present a 34-year-old Turkish woman with BD who had ileal and colonic ulcerations complicated by perforation and gastrointestinal bleeding. Special emphasis was placed on the differential diagnosis between Crohn’s disease (CD) and BD with gastrointestinal involvement.