Necati Çakir

The prevalences of some rheumatic diseases in western Turkey: Havsa study

To study the prevalence major rheumatic diseases in western Turkey. This survey was conducted in Havsa which have a total population of 18,771. Physicians and interns visited every household, interviewed face to face a questionnaire about the symptoms of rheumatic disorders. The individuals replied positively to any question were examined at the nearest health center. Those have no objective findings related to any rheumatic diseases were excluded. People could not be clinically diagnosed were asked to come to the hospital for further evaluation. A total 17,835 of 18,771 residents participated. We estimated the prevalence of Behçet’s Disease (BD) as 0.019%; ankylosing spondylitis: 0.120%; rheumatoid arthritis: 0.321%; knee osteoarthritis (OA): 5.351%; hand OA: 1.110%; hand and knee OA: 1.958%; total OA: 8.420%; primary Raynaud’s: 1.192%; psoriasis: 0.424 %; psoriatic arthritis: 0.050%; rheumatic fever: 0.318%; rheumatic heart disease: 0.200%; inflammatory bowel disease: 0.023%; lupus: 0.059%; gout: 0.018%; systemic sclerosis: 0.022%; juvenile rheumatoid arthritis: 0.032%; temporal arteritis: 0.020%, and familial Mediterranean fever (FMF) as 0.006%. Figures were adjusted for age-sex of the general Turkish population. The prevalence’s of BD and FMF are considerably lower in Havsa as compared to other regions in Turkey.

Increased platelet activation markers in rheumatoid arthritis: Are they related with subclinical atherosclerosis?

Atherosclerotic cardiovascular mortality is increased in rheumatoid arthritis (RA) patients. We evaluated the association of inflammatory response with platelet, endothelial, coagulation activation parameters; and subclinical atherosclerosis in RA patients. We included 27 RA patients (21 female; six male) and 19 healthy subjects (14 female; five male). Disease activity score (DAS28) in RA patients was calculated; and patients were divided into two groups as active and inactive. Flow cytometry was used to determine platelet CD62P expression, platelet microparticles (PMP), platelet-monocyte (PMC) and platelet-neutrophil complexes (PNC). Plasma E-selectin, thrombin-antithrombin (TAT) complex, and serum sCD40L levels were determined by ELISA. The intima-media thickness (IMT) of carotid arteries was determined by B-mode ultrasonography. In RA patients, platelet CD62P expression (p < 0.001), PMC (p = 0.037) and sCD40L (p < 0.001) levels were increased when compared to the control group. PNC (p = 0.07) and TAT levels (p = 0.1) were non-significantly higher, and PMP level (p = 0.075) was nonsignificantly lower in RA patients. Soluble E-selectin level was significantly higher in the active RA group than in the inactive RA group (p = 0.009). There was no correlation between carotid IMT and activity markers, the evaluated parameters (p > 0.05).The increase in markers of active platelets, CD62P and sCD40L, and PMC levels might be associated with the increased cardiovascular mortality in RA. Nevertheless, none of these parameters were associated with carotid IMT: this suggests that one cross-sectional value might not be a good marker for atherosclerosis.

A patient with ankylosing spondylitis who presented with chronic necrotising aspergillosis : Report on one case and review of the literature

Upper lobe fibrobullous disease is a well-known finding in advanced stages of ankylosing spondylitis (AS). In this report, we present a 57-year-old male patient who was diagnosed with a right apical cavitary lesion after coming to us with the complaint of haemoptysis. The patient underwent upper lobe segmentectomy and an aspergilloma was detected. Histologic findings were in favour of necrotising Aspergillus pneumonia. It was interesting that the patient had not been diagnosed with AS before and presented initially with chronic necrotising Aspergillus pneumonia. In the literature, there are recently published series of pulmonary high-resolution computed tomography (HRCT) in AS which claim that parenchymal abnormalities are quite frequent. Although the clinical significance of these abnormalities is not known with certainty, it has been reported that they might be seen even in early-stage patients. It is suggested that the pulmonary involvement in AS might be affected by mechanical factors related to limitation of motion of the thoracic cage and also by parenchymal inflammation. Here, we review the series of pulmonary HRCT in AS patients.

Might platelet-leucocyte complexes be playing a role in major vascular involvement of Behçet's disease? A comparative study.

We determined platelet–leucocyte complexes, which play roles in the thrombosis–inflammation relationship, in Behçets disease patients with and without major vascular involvement (MVI) and in healthy controls. We included 36 Behçets disease patients (22 male, 14 female, mean age: 34.4 ± 8.3 years) and 20 healthy individuals (14 male, six female, mean age: 31.8 ± 4.4 years). Whole blood count, CRP and ESR were determined in both groups. Clinical data about the patients were obtained from medical charts. Individuals with hypertension, diabetes, coronary artery disease, and smokers were excluded. Behçets disease patients with MVI were taken as a separate group (8 male, 5 female, mean age: 37 ± 8 years). MVI was defined as the presence of pulmonary arterial aneurysm, deep venous thrombosis, vena cava inferior or superior thrombosis, or venous sinus thrombosis. Flow cytometry was used to determine platelet–monocyte complexes (PMC), platelet–neutrophil complexes (PNC), basal and adenosine diphosphate (ADP)-stimulated platelet CD62P expression. Behçets disease patients with MVI had significantly higher PNC than Behçets disease patients without MVI and healthy controls (P values = 0.01). PMC levels in Behçets disease patients with MVI were significantly higher than in healthy controls (P = 0.01). The groups were similar in basal and ADP-stimulated platelet CD62P expression (P values >0.05). Basal and ADP-stimulated CD62P expression, PMC and PNC were not significantly different between active Behçets disease versus inactive Behçets disease patients. The evaluated parameters were similar in Behçets disease patients with and without uveitis, and pathergy-positive and pathergy-negative groups. Our results might suggest that the formation of PMC and PNC might play a role in thrombosis and MVI of Behçets disease.

Unilateral phrenic nerve paralysis in a patient with Wegener's granulomatosis

A 35-year-old male diagnosed with Wegeners granulomatosis (WG) in 1989 recently came to our hospital with the complaint of left lumbar pain. He was found to have left nephrolithiasis and left diaphragmatic elevation. Fluoroscopic study and electromyographic examination revealed findings compatible with unilateral phrenic paralysis. We could not detect any cause other than WG which could be responsible for the unilateral diaphragmatic paralysis. Although different types of lung involvement have been reported in WG, to our knowledge this is the first in which unilateral phrenic nerve paralysis and diaphragmatic elevation associated with WG have been diagnosed.

Cleidocranial dysplasia in a mother and her two children

Cleidocranial dysplasia (CCD) is an autosomal dominant disease characterized by skeletal abnormalities which is secondary to haploinsufficiency of the transcription factor Runx2 that plays a role in osteoblast differentiation. In this report, we present a female patient who came to our Rheumatology outpatient clinic with widespread pain, who was diagnosed with fibromyalgia (FM), and who was investigated because of her phenotypic features together with her two children; and consequently, diagnosed with CCD. The diagnosis of CCD was confirmed with genetic analysis. The patient whose alkaline phosphatase was low had no osteoporosis on DEXA. It is unclear whether CCD has or does not have a causal relationship with widespread pain.

A patient with Behçet’s disease who presented with simultaneous cardiac tamponade and central nervous system involvement

Central nervous system (CNS) involvement has been reported in 5–10% of patients with Behcet’s disease (BD) [1]. Cardiac involvement in BD is quite rare, and a few cases with valvular lesions, myocarditis, pericarditis have been defined [2]. Until now, pericardial tamponade in BD has been reported in only two patients [3, 4]. In this report we present a patient with BD who came to us with acute meningitis, developed pericardial tamponade and underwent pericardiocentesis. A 24-year-old man was hospitalized in our department with the complaints of headache, fever, nausea, vomiting, weakness and numbness of the left lower extremity for the last 3–4 days. He described fatigue, myalgia and intermittent fever during the last 6 months. His medical history revealed painful oral aphthae which had recurred once a month for the last 9 years, and painful scrotal lesions which healed with scarring and which had recurred 6–7 times during the last year. He denied any other illnesses or the use of any drugs. On physical examination his temperature was 39 oC; blood pressure, 120/80 mmHg; and pulse rate 108/min. He had one oral ulcer and seven scrotal scars. Cardiac auscultation revealed a pericardial friction rub. On neurologic examination the patient had nuchal rigidity and hypoesthesia, starting from T4 level and continuing all along the left lower extremity. Laboratory data were: ESR, 70 mm/h; CRP, 28.7 mg/dl (N:0–0.5); hemoglobin, 11.6 g/dl; leukocytes, 12 800/mm (with 79% neutrophils); platelets, 354 000/mm. Other biochemical values, urinalysis, C3, C4 and b2-microglobulin levels were normal. Antinuclear antibody, anti-DNA, RF, HBsAg, anti-HCV, anti-HIV, anti-CMV IgM, anti-HSV I, II IgM, VDRL, and Wright tests were negative. Chest X-ray revealed cardiomegaly, and ECG showed ST segment T-wave changes from V1 to V4. On cranial MRI T2-weighted axial images revealed right amygdalohippocampal hyperintensity. Short-time inversion recovery (STIR) images showed an additional hyperintense area on the right parietal lobe localized corticosubcortically (Fig. 1). Cranial MR venography and angiography were normal. Spinal MRI showed a localized hyperintense area on the posterior side of spinal cord at T4 level. Funduscopy revealed no uveitis or retinal vasculitis. On lumbar puncture the CSF was clear, with a high opening pressure. There was neutrophilic pleocytosis of the CSF; Gram staining was nonsignificant. In CSF protein was 50 mg/dl (N:20–40) and glucose was 40 mg/dl (serum: 83). As septic meningitis could not be excluded, the patient was administered ceftriaxone 2·2 g i.v. On the second day of admission he developed hypotension, tachycardia, chest pain which increased on lying down and decreased on bending forward, and the intensity of the pericardial friction rub decreased. He had a generalized tonic–clonic convulsion and antiepileptics were given. After the detection of a massive pericardial effusion, 700 ml of serosanguinous fluid was drained by pericardiocentesis. The patient’s vital signs and clinical status improved. The microscopic examination of the pericardial fluid showed abundant neutrophils; Gram and acid-fast staining were non-significant. Pericardial biopsy yielded a fibrinous pericarditis. His blood, CSF, pericardial fluid and urine cultures were sterile; the tuberculin skin test was negative, and both the pathergy and the monosodium urate crystal tests were positive. Therefore, the neurologic signs and pericarditis were linked to BD; dexamethasone 8 mg every 6 h were added and antibiotic therapy was N. Cakir (&) AE O. N. Pamuk Department of Rheumatology, Trakya Medical Faculty, University of Trakya, Edirne, Turkey E-mail: necaticakir@hotmail.com Tel.: +90-284-2357006