Familial mediterranean fever and glomerulonrphritis: a case report

Ailevi Akdeniz Ateşi ateş ve seröz zarların iltihabı ile ortaya çıkan tekrarlayan ataklar ile karakterize, otozomal resesif bir hastalıktır. En sık Musevi, Arap, Türk ve Ermenilerde görülür. Ailesel Akdeniz ateşinin en önemli ve prognozu belirleyen komplikasyonu böbrek tutulumudur. En sık böbrek tutulumu nedeni AA tipi amiloidozdur. Kolşisinin etkin ve yaygın kullanımı amiloidoz sıklığını eskiye nazaran azaltmıştır. Ülkemizde amiloidoz sıklığı kolşisin kullanmayanlarda %20-25 civarında olduğu bildirilmiştir. Ailevi Akdeniz Ateşi seyri sırasında nadir olarak glomerulonefrit gibi amiloidoz dışında böbrek patolojileri de görülebilmektedir. Burada Ailesel Akdeniz ateşi ve Membranaproliferatif glomerolonefrit birlikteliği görülen bir olgu sunuyoruz.Familial mediterranean fever is characterized by recurrent attacks of fever and polyserositis. Familial mediterranean fever is an autosomal recessive disease. Familial mediterranean fever is commonly seen in Jewish, Arab, Turkish and Armenian. The most important and prognosis-determining complication is kidney involvement. The most common cause of kidney involvement is AA type amyloidosis. Effective ad common use of colchicine decreased amyloidosis frequency when compared to before. It has been reported that frequency of amyloidosis in patients who don't use colchicine is around 20-25% in our country. During the course of familial mediterranean fever, renal pathologies such as glomerulonephritis can be seen other than amyloidosis. Here we present a patient with familial mediterranean fever and membranoproliferative glomerulonephritis

Diagnosis of systemic lupus erythematosus in a patient with generalized lymphadenopathy: a case report

Lenfadenopati (LAP), lenf dügümünün boyut ve karakterindeki anormallik olarak tanımlanır. Lenfadenopati neoplastik veya inflamatuar hücrelerin lenf nodunda çogalması veya lenf nodunu invazyonu sonucu olusabilir. Lenfadenopati genis bir hastalık tablosu sonucunda gelisebilir. Yaygın lenfadenopati sebepleri arasında enfeksiyonlar, otoimmun hastalıklar, malignensiler, histiyositozlar, depo hastalıkları, hiperplaziler ve ilaç etkilesimleri sayılabilir. Lenfadenopati etyolojisinde kollajen doku hastalıkları önemli yer tutmaktadır. En sık lenfadenopatiye neden olan otoimmun hastalıklar romatoid artrit, sistemik lupus eritematozis (SLE) ve Sjögren sendromudur. Bu olguda yaygın lenfadenopati nedeni ile arastırılan ve SLE tanısı alan bir olguyu sunduk. Lenfadenopati nedeniyle tetkik edilen hastalarda kollajen doku hastalıklarını ve özellikle de SLE'u göz önünde bulundurmamız gerektigini bu yazıda vurgulamak istedik.Lymphadenopathy (LAP) is defined as the abnormality in the size and character of lymph node. Lymphadenopathy emerges due to increase of inflammatory cells in lymph node or invasion of these cells in the lymph node. Lymphadenopathy may occur in any age group, in symptomatic or asymptomatic patients, and in a single site or at multiple sites. Causes of generalized lymphadenopathy include infections, autoimmune diseases, malignancies, histiocytoses, storage diseases, benign hyperplasia, and drug reactions. In LAP etiology, collagen tissue disorders hold an important place. The most common autoimmune causes of LAPare rheumatoid arthritis, systemic lupus erythematosus (SLE) and Sjogren syndrome. In this case, we presented a case which was investigated for generalized lymphadenopathy and diagnosed with SLE. SLE diagnosis should be considered in patients researched for lymphadenopathy etiology

Isotretinoin-induced spondyloarthropathy-related symptoms: A prospective study

Objective. Acne vulgaris is a chronic inflammatory disease involving the pilosebaceous unit of the skin. Isotretinoin is a systemic retinoid that is often used as an effective treatment option for severe and treatment-resistant acne. Isotretinoin may also cause rheumatologic symptoms. The aim of this prospective observational study was to present followup results regarding the rheumatologic symptoms of patients who received systemic therapy for the treatment of acne (isotretinoin and tetracycline). Methods. For inclusion in the study, all consecutive patients with acne who were aged > 18 years were evaluated by the same dermatologist. The first 42 consecutive patients were included in the isotretinoin group, and after matching for age and sex, 32 consecutive patients were included in the tetracycline group. Isotretinoin treatment was planned as an average dose of 30 mg daily and a total dose of 120-150 mg/kg for 4-6 months. The patients were administered a dose of 1 g/day of tetracycline as 2 equal doses for 3 months. Results. Forty-two patients diagnosed with acne vulgaris were treated with isotretinoin 20.6 ± 4.4 (male/female: 17/22), and 32 patients were treated with tetracycline 20.6 ± 2.7 (male/female: 8/24). There was no significant difference between the 2 groups with respect to age and sex. Unilateral Achilles enthesopathy developed in 3 patients, whereas both Achilles enthesopathy and unilateral sacroiliitis developed in 1 patient. Inflammatory back pain developed in 6 patients in the isotretinoin group. Conclusion. To our knowledge, this was the first prospective observational study that assessed the rheumatologic symptoms of isotretinoin treatment. The spondyloarthropathy findings were identified in 23.1% of the patients who used isotretinoin

Analysis of the common genetic component of large-vessel vasculitides through a meta- Immunochip strategy

Giant cell arteritis (GCA) and Takayasu's arteritis (TAK) are major forms of large-vessel vasculitis (LVV) that share clinical features. To evaluate their genetic similarities, we analysed Immunochip genotyping data from 1,434 LVV patients and 3,814 unaffected controls. Genetic pleiotropy was also estimated. The HLA region harboured the main disease-specific associations. GCA was mostly associated with class II genes (HLA-DRB1/HLA-DQA1) whereas TAK was mostly associated with class I genes (HLA-B/MICA). Both the statistical significance and effect size of the HLA signals were considerably reduced in the cross-disease meta-analysis in comparison with the analysis of GCA and TAK separately. Consequently, no significant genetic correlation between these two diseases was observed when HLA variants were tested. Outside the HLA region, only one polymorphism located nearby the IL12B gene surpassed the study-wide significance threshold in the meta-analysis of the discovery datasets (rs755374, P?=?7.54E-07; ORGCA?=?1.19, ORTAK?=?1.50). This marker was confirmed as novel GCA risk factor using four additional cohorts (PGCA?=?5.52E-04, ORGCA?=?1.16). Taken together, our results provide evidence of strong genetic differences between GCA and TAK in the HLA. Outside this region, common susceptibility factors were suggested, especially within the IL12B locus

Distinction of hypertriglyceridemic waist phenotype from simple abdominal obesity: interaction with sex hormone-binding globulin levels to confer high coronary risk

CAN, GUNAY/0000-0001-5815-6700WOS: 000396796800015PubMed: 27846754Objective: The associations of total testosterone (TT) and sex hormone-binding globulin (SHBG) with the hypertriglyceridemic waist (HtgW) phenotype and coronary heart disease (CHD) risk have scarcely been examined. We explored such cardiometabolic risk mediations in middle-aged adults. Methods: Participants (n = 1924) in a population-based study were studied by forming categories consisting of abdominal obesity, hypertriglyceridemia, both (HtgW), or none ('healthy'). Cardiometabolic risk was prospectively analyzed (mean follow-up 5.7 years). Results: With reference to the healthy group, SHBG values in HtgW were significantly lower, alike serum HDL-cholesterol. ApolipoproteinB-containing lipoproteins, fasting glucose and complement C3 levels, inverse to lipoprotein[Lp](a) especially in female participants with HtgW phenotype compared with those in the 'healthy' category, suggested the operation of aggregation to Lp(a). Multivariable Cox regression analysis in a model comprising age, waist circumference and systolic blood pressure showed significant protection by SHBG against incident diabetes which tended to be so with TT in men. Sex hormones were not associated with risk of incident CHD or MetS. In another multivariable model, compared to the ` healthy' and the hypertriglyceridemia categories, dichotomized high and, in females, low SHBG values within the HtgW category, positively predicted CHD at significant over 2-fold relative risks. Conclusion: HtgW phenotype distinguishes itself from the (virtually neutral) simple abdominal obesity in independently conferring high CHD risk when elevated or reduced SHBG levels interact. Underlying operation of Lp(a) aggregation is suggested.automotive firm TOFAS, Istanbul, TurkeyFinancial support for this study was provided by the automotive firm TOFAS, Istanbul, Turkey

Apparently "low" serum asymmetric dimethylarginine is associated with fasting glucose and tends toward association with type-2 diabetes

Objective: We investigated the association of serum asymmetric dimethylarginine (ADMA) with metabolic syndrome (MetS), type-2 diabetes and coronary heart disease (CHD) in the general population

Apparently “low” serum asymmetric dimethylarginine is associated with fasting glucose and tends toward association with type-2 diabetes

Objective: We investigated the association of serum asymmetric dimethylarginine (ADMA) with metabolic syndrome (MetS), type-2 diabetes and coronary heart disease (CHD) in the general population