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Systemic lupus erythematosus (SLE) is a chronic autoimmune disease which may involve several organs or organ systems. SLE may also have hematological as well as joint, kidney, and central nervous system involvements. Hematological abnormalities such as anemia, leukopenia, thrombocytopenia, and autoimmune hemolytic anemia are the best-known and most common hematological findings detected in SLE patients which are included in the classification criteria for SLE. Autoimmune myelofibrosis (AIMF) refers to bone marrow (BM) fibrosis (myelofibrosis) that develops in an autoimmune setting. Myelofibrosis is not among the SLE classification criteria and it is also not well-known. Current reports in the literature on SLE-associated AIMF are mostly restricted to case reports or reviews of such case reports. The occurrence of BM fibrosis in SLE patients has been explored merely in few studies which concluded that myelofibrosis is a rare symptom of SLE. Herein, we propose the hypothesis that SLE-associated AIMF is not rare and, on the contrary, it can indeed be more frequent than what is known or expected.

Copyright of Journal of Turkish Society for Rheumatology is the property of Galenos Yayinevi Tic. LTD. STI and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Osmangazi Journal of Medicine / Osmangazi Tip Dergisi is the property of Eskisehir Osmangazi University and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Osmangazi Journal of Medicine / Osmangazi Tip Dergisi is the property of Eskisehir Osmangazi University and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Thromboses in hepatic and portal veins are the most common problems affecting the gastro-hepatic system in antiphospholipid syndrome (APS), but hepatic artery thrombosis has been rarely reported especially during pregnancy or the postpartum period. We have reported a 26-year-old female patient with a previous diagnosis of systemic lupus erythematosus and APS manifested by fever and severe abdominal pain. Based on clinical and laboratory findings, diagnosis of liver abscess was initially considered. The final diagnosis was confirmed as liver infarction due to APS based on liver biopsy. After treatment with intravenous immunoglobulin, anticoagulation, plasmapheresis, and steroid, the clinical and laboratory improvement were observed. Here, our aim is to attract attention that hepatic necrosis may mimic liver abscess in terms of clinical symptoms and laboratory findings. [ABSTRACT FROM AUTHOR]

In the article, the authors present the case of a 31-year-old man with Behçet's syndrome (BS) who was rushed to a clinic due to chest tightness, pleuritic chest pain, and dyspnoea to discuss reactivation of autoimmune disorders in BS patients receiving immunosuppressive therapy following SARS-CoV-2 messenger RNA (mRNA) vaccination. The patient was treated with drugs like ciclosporin for panuveitis for two years.

Copyright of Journal of Turkish Society for Rheumatology is the property of Galenos Yayinevi Tic. LTD. STI and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Objective: Idiopathic inflammatory myopathies (IIM) are a group of connective tissue diseases that are characterized with proximal muscle inflammation and immune mediated muscle damage. Compared to the general population, both malignancy and mortality rates are found to be higher in IIM patients. The aim of our study was to define the predictive factors for mortality and to evaluate the types and frequencies of concomitant malignities in IIM patients. Methods: A retrospective evaluation was conducted in 53 patients who were diagnosed with IIM using 1975 Bohan-Peter criteria in university hospital rheumatology department between January 1998 and January 2020. The clinical and laboratory findings, malignancy status and prognostic factors for mortality were analyzed for the patients. Results: Among the 53 patients (32 women, 21 men), the mean age was 55.24±16.12 years, the mean age of myopathy diagnosis was 44.45±17.62 years. When the subgroups of myositis were analyzed, 27 patients (50.9%) had dermatomyositis and 23 patients (43.4%) had polymyositis. The most frequent clinical finding was fatigue with 77.2%. Steroid usage rate was 98.1% and azathioprine usage rate was 34% among patients. Malignancy frequency was 13% (n=7) (2 nasopharyngeal cancer, 2 colon cancer, 1 non-small cell lung cancer, 1 breast cancer and 1 pancreas cancer) and the age of patients with malignancy and myositis was significantly higher (p=0.03, p=0.02 respectively). When the mortality frequency was evaluated by different parameters, it was found that gender, disease duration and malignity preexisting myositis were statistically significant parameters. The most frequent mortality cause was malignancy. Cumulative survival rate in our cohort was 80.75% in 5 years and 72.68% in 10 years. Conclusion: As a result, the frequencies of mortality and malignancy in our patients with IIM were generally similar to those in the literature. The most important factors affecting life expectancy and mortality were advanced age at diagnosis and the presence of malignancy. [ABSTRACT FROM AUTHOR]

The article discusses a study which investigated the presence of elevated procalcitonin (PCT) level in adult-onset Still's disease (AOSD) patients in the absence of infections. Screeinng were done on patients with AOSD between 2010 and 2021. It was observed that PCT level declined when immunosuppression was administered and that there was a link between serum ferritin and PCT levels.

Objective: In this study, our primary aim was to determine the order frequency of antinuclear antibody (ANA) test according to special departments and its results in a university-based hospital. The second aim was to determine the most common cause of ANA test orders and to highlight the importance of "choosing wisely". Methods: The frequency and results of ANA tests ordered for various reasons by clinicians between January 2014 and August 2016 were retrospectively screened by using ANA test code. ANA test was evaluated by indirect immunofluorescence test (IIF) method and reported as+1 positive, +2 positive, +3 positive, +4 positive according to fluorescent grade. Results: 11,407 of ANA test results were taken into consideration. The first five department requiring the most ANA test was neurology, dermatology, rheumatology, pediatric nephrology and hematology. The rate of ANA positivity according to departments including neurology, dermatology, rheumatology and hematology was 14.9%, 15.4%, 32.6% and 14.1%, respectively. It was determined that the rate of +4 positivity of ANA test was the most in patients ordered by rheumatology department. (135/262=%51.5) (p<0.0001). The most reasons for ANA test according to ICD codes were headache, cerebrovascular events and multiple sclerosis for neurology department; urticaria, psoriasis and dermatitis for dermatology; systemic lupus erythematosus (SLE), arthritis, arthralgia, and fibromyalgia for rheumatology; anemia and thrombocytopenia for hematology. Conclusion: Although neurology was the department that most frequently ordered ANA test in daily clinical practice in our hospital, negativity rate of ANA test was also high in neurology department. The rate of high positivity of ANA test was found by rheumatology department. As a result, in terms of "choosing wisely, when and in which patients ANA test be required must be well known. Awareness must be raised through information sharing on ANA test in order to avoid inappropriate ANA test request. [ABSTRACT FROM AUTHOR]

Retroperitoneal fibrozis, tipik olarak abdominal aort ve ana iliak arter çevresinde kronik enflamatuvar hücre infiltrasyonu ve fibrotik lezyonlar ile karakterize, üreterleri de tutan ve obstrüktif üropatiye neden olan nispeten nadir bir hastalıktır. Genellikle idiyopatiktir, ancak bazı ilaçların kullanımına, malign hastalıklara, enfeksiyonlara ve cerrahiye sekonder olarak da gelişebilir. Hastalığın idiyopatik formu, olguların üçte ikisinden fazlasını oluşturur. İdiyopatik retroperitoneal fibrozisin ilk tanımı, 1905'te üreter obstrüksiyonuna neden olan geniş bir retroperitoneal fibrotik sürecin cerrahi tedavisini bildiren Fransız ürolog Albarran tarafından yapılmıştır. Retroperitoneal fibrozisin epidemiyolojik özellikleri iyi belirlenmemiştir ve yalnızca idiyopatik formla ilgili veriler mevcuttur. Finlandiya'da yapılan bir çalışmaya göre idiyopatik retroperitoneal fibrozisin 100.000 kişi-yılında 0,1 insidansına ve 100.000 kişi başına 1,38 prevalansa sahip olduğuna dikkat çekilmiştir. Erkekler kadınlardan 2 ila 3 kat daha sık etkilenir. Ortalama başvuru yaşı 50-60'tır. Patogenez: İdiyopatik retroperitoneal fibrozisin, retroperitoneal fibrozis ile birlikte kronik periaortitin bir alt grubu olduğu kabul edilmektedir. Kronik periaortitin, abdominal aortun aterosklerotik plaklarındaki antijenlere karşı lokal bir enflamatuvar reaksiyondan kaynaklandığı düşünülmüştür. İdiyopatik retroperitoneal fibrozisin patogenezi muhtemelen çok faktörlüdür. Asbeste mesleki maruziyet, sigara gibi çevresel faktörler idiyopatik retroperitoneal fibrozis gelişme riskini önemli ölçüde artırır. Klinik Bulgular: İdiyopatik ve sekonder retroperitoneal fibrozisin klinik belirtileri sıklıkla örtüşür, bu nedenle iki hastalık grubu arasındaki ayırıcı tanıda klinik yararlı değildir. Retroperitoneal fibrozisin başlangıcındaki klinik belirti ve semptomlar spesifik değildir. Yaygın semptomlar arasında bel, karın veya yan ağrısı ve halsizlik, ateş, iştahsızlık ve kilo kaybı gibi yapısal semptomlar bulunur. Skrotal şişlik, varikosel veya hidrosel sık görülür. Bazen hematüri, poliüri ve üriner enfeksiyonlar rapor edilir ve ilerlemiş bilateral üreteral obstrüksiyonu olan hastalarda oligoanüri ve üremi ile ilgili semptomlar gelişebilir. Fizik muayene genellikle anlamlı değildir. Abdominal veya lomber hassasiyet olabilir. Özellikle aort dilate olduğunda, periumblikal bir üfürüm duyulabilir. Mevcut klinik belirtilerin spesifik olmayan doğası ve fiziksel bulguların azlığı nedeniyle, semptomların başlangıcı ile tanı arasında sıklıkla önemli bir gecikme vardır. Olguların %80-100'ünde üreter tutulumu bildirilmektedir. Laboratuvar bulguları: Laboratuvar bulguları retroperitoneal fibrozise özgü değildir. Rutin laboratuvar testlerinin sonuçları enflamatuvar hastalık ile uyumludur. Eritrosit sedimentasyon hızı (ESH) ve C-reaktif protein (CRP) gibi akut faz reaktanları hastaların %80-100'ünde yüksektir. Bu laboratuvar testleri genellikle hastalığın klinik seyrini izlemek için kullanılır, ancak hastalık aktivitesini her zaman güvenilir bir şekilde yansıtmazlar. Azotemi genellikle üreteral obstrüksiyonun derecesine bağlıdır. Hem kronik enflamasyonun hem de mevcut olduğunda böbrek yetmezliğinin katkıda bulunabileceği hafif ila orta derecede anemi görülür. Bu test sonuçları, retroperitoneal fibrozisin idiyopatik ve sekonder formlarında ortaya çıkabilir. Otoimmün hastalık için yapılan testlerden, antinükleer antikorlar (ANA) idiyopatik retroperitoneal fibrozisli hastaların %60'ında tespit edilir. Romatoid faktör ve dsDNA, ekstrakte edilebilir nükleer antijen (ENA) ve anti nötrofilik sitoplazmik antikorlar (ANCA) pozitif saptanabilir. Retroperitoneal fibrozisin sekonder formlarını tanımlayabilecek laboratuvar testleri yoktur. Tanıyı doğrulamak ve hastalığı ayırt etmek için biyopsi ve histopatolojik değerlendirme gerekli olabilir. Bilgisayarlı tomografi (CT), manyetik rezonans (MRI) görüntüleme ve pozitron emisyon tomografisi (PET-CT) hastalığın tanı ve takibinde tercih edilen yöntemlerdir. Tanı: Retroperitoneal fibrozis için standart tanı kriterleri yoktur. Görüntüleme kullanımı hakkında bir uzlaşı yoktur. Tanıya ulaşmak için hem görüntüleme hem de klinik sunumu dikkate alan bütüncül bir yaklaşıma ihtiyaç vardır. İdiyopatik retroperitoneal fibrozisin tipik CT/MRI bulguları, sekonder retroperitoneal fibrozisin (örneğin; malignite) aksine anevrizmal olmayan distal abdominal aortu anterolateral olarak çevreleyen ancak önemli ölçüde yer değiştirmeyen tek/birleşmiş bir kitle şeklindedir. ESH ve CRP gibi yüksek düzeylerde akut faz reaktanlarının varlığı, tanıyı doğrulamaya yardımcı olabilir. Retroperitoneal dokunun histopatolojik incelemesi, kitle atipik lokalizasyon (örneğin; pelvik, peripankreatik) gösterdiğinde veya diğer klinik veya laboratuvar bulguları altta yatan malign hastalık veya enfeksiyonların varlığını düşündürdüğünde gereklidir. Tedavi ve seyir Retroperitoneal fibroziste tedavi hedefi, remisyonu sağlamak, yani obstrüktif üropatiyi veya diğer kompresif komplikasyonları önlemek veya çözmek ve semptomları azaltmaktır. Retroperitoneal fibrozisin farmakolojik tedavisi birkaç randomize çalışmada değerlendirilmiştir ve esas olarak gözlemsel çalışmalara dayanmaktadır. Steroid tedavisi, tedavinin altın standardı olmaya devam etmektedir. Şimdiye kadar hiçbir randomize çalışma, immünosüpresif ilaçların etkinliğini ve toksisitesini steroidlerle karşılaştırmamıştır. Mikofenolat mofetil, siklofosfamid, azatioprin, metotreksat ve takrolimus gibi diğer immünosüpresif ilaçların etkinliği de bildirilmiştir. Ek olarak, bir anti-CD20 antikoru olan rituksimab, IgG4 ilişkili retroperitoneal fibrozis tedavisi için umut vericidir. Ayrıca steroidi tolere edemeyen veya ciddi steroid yan etkisi gelişen veya steroid monoterapisi ile yanıt alınamayan hastalarda tamoksifen de önerilebilmektedir. Retroperitoneal fibrozisin sekonder formlarının tedavisi ise mümkünse nedene dayalı bir yaklaşım gerektirir. Retroperitoneal fibrozisli hastalar tedavinin başlangıcından sonra, genellikle subjektif semptomlar, ESR ve CRP düzeyleri ile düzenli olarak izlenmelidir. Hastaların %50'sinde nüks görülebilir ve bu nedenle tekrar immünosüpresif tedaviler gerekebilir. Üreter obstrüksiyonunu gidermek için tıbbi tedavilere ek olarak girişimsel prosedürler (esas olarak üreteral stentleme) gerekirken, cerrahi üreteroliz genellikle dirençli olgulara uygulanabilir. [ABSTRACT FROM AUTHOR]

Objective: Systemic lupus erythematosus (SLE) may affect a number of systems, with the hematological system being one of the most common. Our aim is to determine the existence of cytopenia at diagnosis or during follow-up of our SLE patients as well as the associated factors. Material and Methods: A cohort of SLE patients that had been followed-up in the Department of Rheumotology from 1998 to 2015 was retrospectively assessed. Clinical and laboratory findings about the patients were recorded. Results: Out of 221 patients composing the cohort, cytopenia was already present in 83.3% (n=184) at the time of diagnosis. Anemia was detected in 56.1% (n=124), leukopenia in 28.9% (n=64), lymphopenia in 76% (n=168), neutropenia in 4.5% (n=10), and thrombocytopenia in 17.2% (n=38) of patients. The proportion of patients with cumulative cytopenia was 90% (n=199). Cumulative cytopenia was disease-related in 83.4% (n=166) and medication-related in 16.6% (n=33) of the patients. In cases of drug-induced cytopenia, azathioprine was the most frequently prescribed drug. In patients with cytopenia at the time of diagnosis, erythrocyte sedimentation rates (ESR) were higher, C3 and C4 hypocomplementemia was more prevalent, and they were positive for anti-dsDNA at a greater proportion (p<0.001, p=0.015, p=0.028, and p=0.019, respectively). Moreover, photosensitivity, renal involvement, and antiphospholipid syndrome (APS) were detected more frequently in patients with cytopenia at the time of diagnosis. There was no difference between the two patient sets in terms of other organ involvement (p>0.05). Conclusion: The most common hematological disorders in SLE patients are lymphopenia and anemia, and patients must be further examined for APS and renal involvement if they suffer cytopenia. [ABSTRACT FROM AUTHOR]

Objective: Behçet's disease (BD) is a systemic vasculitis with a significantly varying clinical course following relapses and remissions, which may involve a number of organs such as the skin, joints, lungs, and blood vessels as well as systems such as the central nervous system and gastrointestinal system. Its prognosis is known to be worse in males. There are several studies in the literature on the clinical features and gender distribution of BD. The aim of the present study was to determine the clinical characteristics of BD and the presence of a relation with gender and to investigate the correlation of our results with the current literature. Material and Methods: We retrospectively reviewed 329 patient files. The demographic features of the patients, their symptoms and findings of BD, the results of pathergy tests, the presence of any individuals in the family with BD, and HLA-B51 antigen positivity were recorded. Results: The most frequent findings were oral aphtous ulcers (100%), genital ulcers (84%), papulopustular lesions (69.9%), and joint involvement (57.4%). Vascular involvement and ocular involvement were significantly higher in males, whereas joint involvement and headache were more common in females (p<0.001, p=0.014, p<0.001, and p<0.001, respectively). Contrary to the literature, we found that the disease had an earlier onset in individuals with a positive familial history of an oral aphthous ulcer or BD (p=0.03 and p=0.02, respectively) and that joint involvement was more common in patients with erythema nodosum (p=0.02). Conclusion: The clinical features of BD may vary between the genders. Variations exist in the results depending on the population size, the department where the study was conducted, the patient inclusion criteria, and the region where the patients live. [ABSTRACT FROM AUTHOR]

Objective: This study aimed to investigate the frequency in which familial Mediterranean fever (FMF) coexists with other diseases and determine whether Mediterranean fever (MEFV) gene mutations are involved in such coexistence. Material and Methods: In total, 142 consecutive patients with FMF investigated for MEFV mutation were enrolled in this study [Female: 87; Male: 55, mean age 32±12 years (11-62)]. All the patients were questioned for the presence of concurrent disorders, and the medical records of these patients were revised retrospectively. A previous diagnosis of inflammatory disorder other than FMF was considered true if it met the relevant criteria. MEFV mutations were divided into 2 groups, namely M694V and its subgroup (homozygous or heterozygous) (Group I) and others (Group II). Compound heterozygosity for M694V mutation was included in Group II to form a homogeneous group for Group I. Group I and Group II were compared according to phenotypical features. The presence of MEFV mutation was investigated in exons 2, 3, 5, and 10 by the multiplex-PCR reverse hybridization method. Results: Concomitant disorders were found in 17 of 73 patients with FMF (23%) in Group I and 5 of 56 patients (8.9%) in Group II (p=0.04). Concomitant disorders in Group I were as follows: 7 cases of amyloidosis, 2 cases of Behcet's disease (BD), 4 cases of ankylosing spondylitis (AS), 1 case of antiphospholipid syndrome, 1 case of Henoch-Schonlein purpura (HSP), 1 case of combination of psoriatic arthritis, HSP, and membranoproliferative glomerulonephritis, and 1 case of AS and amyloidosis. In Group II, the following disorders were found: 1 case of amyloidosis, 1 case of BD, 1 case of AS, 1 case of ulcerative colitis, and 1 case of vitiligo. Conclusion: The presence of M694V mutation may predispose patients with FMF to developing other inflammatory disorders. [ABSTRACT FROM AUTHOR]