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Background: Behçet's disease is a systemic vasculitis affecting both arteries and veins, as well as causing recurrent inflammatory multiorgan disease. Vascular involvement is associated with increased mortality and morbidity. Matrix metalloproteinases are released at sites of inflammation and degrade various components of the extracellular matrix. Increased levels of metalloproteinase-9 and metalloproteinase-2 have been previously reported in Behçet's disease. Methods: In this cross-sectional study, metalloproteinase-2 and metalloproteinase-3 serum levels were investigated in 103 patients with Behçet's disease and 69 healthy controls, using Invitrogen immunoassay human metalloproteinase-2 and metalloproteinase-3 ELISA kits. Results: Serum metalloproteinase-2 and metalloproteinase-3 levels were significantly higher in the Behçet's disease group compared to healthy controls. Besides, serum metalloproteinase-3 levels were significantly higher in subgroups of Behçet's disease with aneurysmal vascular involvement and with neurological involvement. However, metalloproteinase-2 and metalloproteinase-3 serum levels did not show a positive correlation with disease activity. Conclusion: Metalloproteinase-2 and -3 may contribute to the complex pathogenesis of Behçet's disease. More importantly, the detection of very high serum levels of metalloproteinase-3 may predict the formation of an aneurysm, or possibly the presence of neurological involvement in Behçet's disease and may lead the clinician to make an earlier diagnosis of these complications in young male patients with high risk. [ABSTRACT FROM AUTHOR]

Objectives: Vogt-Koyanagi-Harada Disease (VKHD) is a systemic autoimmune disorder characterized by granulomatous panuveitis. Inflammatory rheumatic diseases (IRDs) are among the differential diagnosis of VKHD. However, current knowledge on the rheumatological aspects of VKHD is still limited. We aimed to investigate the prevalence of rheumatic conditions in VKHD patients. Methods: VKHD patients were included in the study and they were reviewed in terms of the presence of any rheumatological manifestations. Results: There were 18 patients with a female preponderance (83.3%, female). Inflammatory type of peripheral joint pain (11%) and sicca symptoms (33%) were the most common rheumatological findings. The frequency of spondyloarthritis-related features such as inflammatory back pain and HLA-B27 rate was not increased. None of the patients had radiographic sacroiliitis. Anti-nuclear antibody was positive in high titres nearly in 30% of the patients and three patients had antibodies against extractable nuclear antigens. Nailfold capillaroscopy was abnormal in about one-third of the patients. Pathergy test was negative in all cohorts. While angiotensin-converting enzyme was elevated in nearly 20% of the patients, there were no abnormalities on chest X-rays. Conclusion: VKHD shares some features with IRDs. The common features were mostly suggestive of connective tissue disease rather than SpA or rheumatoid arthritis. [ABSTRACT FROM AUTHOR]

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Background: This study aimed to examine fetomaternal outcomes in pregnant women in a large Turkish Takayasu arteritis (TAK) cohort and to evaluate the effects of pregnancy on the disease in those patients. Methods: This is a cohort study involving 296 pregnancies of 112 TAK patients from 8 tertiary rheumatology centers in Turkey. Pregnancies were divided into 2 groups as pre‐d (before disease onset) and post‐d (after disease onset). In addition, post‐d pregnancies were further divided into 2 subgroups according to fetomaternal complications (FMC) development status. Finally, patients were grouped into those with and without a history of pregnancy after disease onset. Results: In post‐d pregnancies, rates of worsening hypertension, new‐onset hypertension, and preeclampsia were higher than in pre‐d pregnancies (0.9% vs 16%, P <.001, 0.5% vs 5.3%, P =.012, and 0% vs 4%, P =.013, respectively). Patients with FMC were more likely to have renal artery involvement (65% vs 21%, P =.003). The patients who had post‐d were younger, had longer disease duration, and had more relapses number than other patients (P <.001, P =.028, P =.016, respectively). Vasculitis Damage Index (VDI) results were similar in patients with or without post‐d pregnancies. Conclusion: Pregnancies after disease onset were found to be associated with HT and preeclampsia/eclampsia. HT‐related FMCs are increased in TAK, and patients with renal artery involvement are at higher risk. The number of relapses increases in patients who become pregnant after disease onset, but pregnancy was not an independent risk factor for relapse. Pregnancy after the onset of disease had no negative effect on VDI. [ABSTRACT FROM AUTHOR]

To compare the treatment outcomes of TNF inhibitors and tocilizumab (TCZ) in patients with Takayasu arteritis. Takayasu arteritis patients who were refractory to conventional immunosuppressive (IS) drugs and received biologic treatment were included in this multicenter retrospective cohort study. Clinical, laboratory and imaging data during follow-up were recorded. Remission, glucocorticoid (GC) sparing effect, drug survival was compared between TNF inhibitor and TCZ treatments. Also, a subgroup matched comparison was performed between groups. One hundred and eleven (F/M: 98/13) patients were enrolled. A total of 173 biologic treatment courses (77 infliximab, 49 TCZ, 33 adalimumab, 9 certolizumab, 3 rituximab, 1 ustekinumab and 1 anakinra) were given. Tocilizumab was chosen in 23 patients and TNF inhibitors were chosen in 88 patients as first-line biologic agent. Complete/partial remission rates between TCZ and TNF inhibitors were similar at 3rd month and at the end of the follow-up. GC dose decrease (≤4 mg) or discontinuation of GCs was achieved in a similar rate in both groups (TNF inhibitors vs TCZ: 78% vs 59%, p = 0.125). Drug survival rate was 56% in TNF inhibitors and 57% in TCZ group (p = 0.22). The use of concomitant conventional ISs did not affect the drug survival (HR =0.78, 95% CI =0.42–1.43, p = 0.42). The match analysis showed similar results between groups in terms of relapse, decrease in GC dose, surgery need and mortality. The efficacy and safety outcomes and drug survival rates seem to be similar for TNF inhibitors and tocilizumab in patients with Takayasu arteritis. [Display omitted] [ABSTRACT FROM AUTHOR]

Giriş: Paraneoplastik sendromlar, tümör dokusu tarafından üretilen hormonlar ve sitokinlerin veya tümör dokusuna karşı gelişmiş olan hücresel ve hümoral immün yanıtın bir sonucu olarak birincil maligniteden uzak bölgelerde görülebilen semptom ve bulgular olarak tanımlanabilir. Kanser ve artrit genel popülasyonda yaygın görülen durumlardır. Artrit başlangıcı ve malignite arasındaki zamansal ilişki veya tümör tedavisi sonrası eklem semptomlarının remisyonunun gözlenmesi paraneoplastik bir süreci düşündürebilir. Bu olguda simetrik oligoaritle başvuru sonrası hepatoselüler karsinom (HSK) tanısı alan bir olguyu sunmaktayız. Olgu: Yirmi dokuz yaşında kadın hasta 3 aydır ayak bileklerinde ağrı ve şişlik yakınması ile başvurdu. Sabahları metatarsal bölgede ağrı ve üzerine basamama yakınması da vardı. Diğer eklemleri ile ilgili aktif yakınması bulunmamaktaydı. Fizik muayenede her iki ayak bileğinde şişlik ve ağrı saptandı. On yıldır insülin bağımlı diyabeti mevcut. Kız kardeşine hepatoselüler karsinoma bağlı transplantasyon yapılmış. Hastanın ALT: 54 U/L (0-35), AST: 54 U/L (0-35) GGT: 618 U/L (0-38), alkalen fosfataz: 466 U/L (30-120), C-reaktif protein değeri 9 mg/L, romatoid faktörü (RF) 15 (0-30 IU/mL), anti-CCP (anti-sitruline protein antikorları): 32 (0-20 IU/mL), ANA (anti-nükleer antikor) negatif, brusella tüp aglütinasyonu negatif olarak saptandı. Akciğer grafisi olağan saptandı. Hepatobiliyer ultrasonografide en büyüğü 1,5 cm olan çok sayıda hipoekoik nodül gösterildi. Dinamik üst batın manyetik rezonans incelemesinde lezyonlar hepatoselüler karsinom ile uyumlu saptandı. Tümör FDG-PET görüntülemesinde karaciğer dışında aktif odak veya metastaz saptanmadı. Hasta gastroenteroloji takiplerinde karaciğer nakil sırasına alındı. Ayak bileği artritlerinde 4 mg/gün metilprednizolon ve ibubrufen 1,600 mg/gün ile semptomatik olarak kısmi rahatlama gösterdi. Sonuç: Paraneoplastik artritle ilişkili en sık görülen solid tümörler akciğer ve meme adenokarsinomları olduğu bildirilmiştir. Parenoplastik artritte %27,2 RF, %19 ANA, %10,7 anti-CCP pozitif olarak görülebilir. Tipik olmayan bölgelerde ve beklenmeyen bulguların eşlik ettiği artritte paraneoplastik sendrom da akılda tutulmalıdır. [ABSTRACT FROM AUTHOR]

Olgu: Otuz iki yaşında erkek hasta her iki bacakta kaşıntılı, kızarık lezyonlar nedeniyle kliniğimize başvurdu. Kastamonu doğumlu, köyde çiftçilikle uğraşan hastanın şikayetleri 3 gündür mevcuttu; birlikte yaşadığı yakınlarında ve kendisinde şüpheli böcek ısırığı olduğu öğrenildi. İki yıl önce oral aft, genital ülser ve artrit ile Behçet hastalığı tanısı alan hasta kolşisin 1 mg/gün kullanmaktaydı. Muayenesinde bacaklarda daha sık olmak üzere gövdede birkaç adet kaşıntılı sıralı şekilde ortaları ekskoriye edilmiş papüller saptandı (Resim 1). Hastanın tetkiklerinde CRP: 20 mg/L, ESH: 30 mm/h, hemogram, karaciğer ve böbrek fonksiyonları olağan olarak sonuçlandı. HLAB51 pozitif, ANA, ENA profil, ANCA testleri, komplemanlar ve AFAS antikorları negatif olarak geldi. Deri lezyonlarından punch biyopsi yapıldı. Hastada ön planda böcek ısırığına bağlı paterji reaksiyonu düşünüldü. Hastaya 16 mg/gün metilprednizolon başlandı. İki hafta sonra kontrole gelen hastanın şikayetlerinde büyük oranda gerileme olduğu görüldü (Resim 1). Tetkiklerinde AFR olağan olarak sonuçlandı. Deri biyopsisinde eozinofillerin eşlik ettiği yüzeyel ve derin perivasküler dermatit tablosu, dermal hipersensitivite reaksiyonu gözlendi; yapılan seri kesitlerde aktif vaskülit ile uyumlu damar değişiklikleri izlenmedi, morfolojik bulgular klinik ön tanıdaki pikür (böcek ısırığı) ile bağdaşmaktadır şekinde raporlandı. İzlemde steroid tedavisi doz azaltım şeması altında azaltılarak kesildi. Sonuç: Paterji reaksiyonu, intradermal pikür ile ortaya çıkan eritem, papül ve püstül şeklinde deri hiperreaktivitesidir. Böcek ısırığı Behçet hastalarında doku bütünlüğünü bozarak abartılı enflamatuvar yanıt ve sonrasında anormal yara iyileşme sürecine neden olarak paterji reaksiyonuna yol açabilmektedir. Deri lezyonu ile başvuran Behçet hastalarında akılda tutulması gereken klinik tablolardan biridir. [ABSTRACT FROM AUTHOR]

The article describes the case of a 56-year-old man who presented with a 3-month history of cough, chest pain and dyspnea. Topics covered include the medical history of the patient, the findings of the laboratory and pulmonary tests which led to the diagnosis of pulmonary apical fibrosis as the initial symptom ofankylosing spondylitis, and patient outcome following treatment.

The patient, a previously healthy 25-year-old woman, presented with a 3 month history of new-onset headaches, polyuria, and polydipsia. There was no infection, trauma, or drug intake reported prior to symptoms. Physical examination findings were unremarkable; results of laboratory testing were consistent with increased levels of markers of inflammation along with hypernatremia, high serum osmolality, and low urine osmolality. Water deprivation test findings were suggestive of central diabetes.

Introduction: We aimed to investigate the effects of the coronavirus disease 2019 (COVID-19) pandemic on the course and treatment of patients with inflammatory rheumatic musculoskeletal disease (iRMD) using biologic or targeted synthetic disease modifying and rheumatic drugs (b/tsDMARDs). Methodology: The study was carried out in two stages: in the first stage we investigated the delay of b/tsDMARD treatment in the first 3 months of the pandemic; in the second stage, we investigated all patients who decided to continue treatment after interruption in the 12-month period. Results: A total of 521 patients were included in the study. The iRMD diagnosis was listed as spondyloarthritis (SpA) (54.3%), rheumatoid arthritis (RA) (25.7%), psoriatic arthritis (PsA) (8.4%), vasculitis (6.1%), and others (5.4%). Concurrent use of hydroxychloroquine (hazard ratio [HR] = 1.49), iv bDMARD use (HR = 1.34), and a history of discontinuation of drug in the first 3 months of the pandemic (HR = 1.19) were determined as factors that reduced 12-month drug retention rates. The use of glucocorticoid (HR = 3.81) and having a diagnosis of interstitial lung disease/chronic obstructive lung disease (HR = 4.96) were found to increase the risk of being infected by SARS coronavirus 2 (SARS-CoV-2). Conclusions: It was shown that approximately 1/5 of iRMD patients using b/tsDMARDs delayed their treatment due to the fear of COVID-19 in the first three months of the pandemic process. However, with good communication with the patients, b/tsDMARD treatment was restarted and the 12-month drug retention status was quite high. [ABSTRACT FROM AUTHOR]

Objectives: Glucocorticoids (GC) are widely accepted as the standard first-line treatment for giant cell arteritis (GCA). However, relapse rates are reported up to 80% on GC-only protocol arms in controlled trials of tocilizumab and abatacept in 12-24 months. Herein, we aimed to assess the real-life relapse rates retrospectively in patients with GCA from Turkey., Methods: We assembled a retrospective cohort of patients with GCA diagnosed according to ACR 1990 criteria from tertiary rheumatology centres in Turkey. All clinical data were abstracted from medical records. Relapse was defined as any new manifestation or increased acutephase response leading to the change of the GC dose or use of a new therapeutic agent by the treating physician., Results: The study included 330 (F/M: 196/134) patients with GCA. The mean age at disease onset was 68.9±9 years. The most frequent symptom was headache. Polymyalgia rheumatica was also present in 81 (24.5%) patients. Elevation of acute phase reactants (ESR>50 mm/h or CRP>5 mg/l) was absent in 25 (7.6%) patients at diagnosis. Temporal artery biopsy was available in 241 (73%) patients, and 180 of them had positive histopathological findings for GCA. For remission induction, GC pulses (250-1000 methylprednisolone mg/3-7 days) were given to 69 (20.9%) patients, with further 0.5-1 mg/kg/day prednisolone continued in the whole group. Immunosuppressives as GC-sparing agents were used in 252 (76.4%) patients. During a follow-up of a median 26.5 (6-190) months, relapses occurred in 49 (18.8%) patients. No confounding factor was observed in relapse rates. GC treatment could be stopped in only 62 (23.8%) patients. Additionally, GC-related side effects developed in 64 (24.6%) patients, and 141 (66.2%) had at least one Vasculitis Damage Index (VDI) damage item present during follow-up., Conclusions: In this first multi-centre series of GCA from Turkey, we observed that only one-fifth of patients had relapses during a mean follow-up of 26 months, with 76.4% given a GC-sparing IS agent at diagnosis. At the end of follow-up, GC-related side effects developed in one-fourth of patients. Our results suggest that patients with GCA had a low relapse rate in real-life experience of a multi-centre retrospective Turkish registry, however with a significant presence of GC-associated side effects during follow-up.

Background: Behçet's disease is a systemic vasculitis affecting both arteries and veins, as well as caus- ing recurrent inflammatory multiorgan disease. Vascular involvement is associated with increased mortality and morbidity. Matrix metalloproteinases are released at sites of inflammation and degrade various components of the extracellular matrix. Increased levels of metalloproteinase-9 and metal- loproteinase-2 have been previously reported in Behçet's disease., Methods: In this cross-sectional study, metalloproteinase-2 and metalloproteinase-3 serum levels were investigated in 103 patients with Behçet's disease and 69 healthy controls, using Invitrogen immunoassay human metalloproteinase-2 and metalloproteinase-3 ELISA kits., Results: Serum metalloproteinase-2 and metalloproteinase-3 levels were significantly higher in the Behçet's disease group compared to healthy controls. Besides, serum metalloproteinase-3 levels were significantly higher in subgroups of Behçet's disease with aneurysmal vascular involvement and with neurological involvement. However, metalloproteinase-2 and metalloproteinase-3 serum levels did not show a positive correlation with disease activity., Conclusion: Metalloproteinase-2 and -3 may contribute to the complex pathogenesis of Behçet's dis- ease. More importantly, the detection of very high serum levels of metalloproteinase-3 may predict the formation of an aneurysm, or possibly the presence of neurological involvement in Behçet's dis- ease and may lead the clinician to make an earlier diagnosis of these complications in young male patients with high risk.