Explore the vast range of titles available.

ABSTRACT Objectives: This study compared the secukinumab treatment responses and adverse effects in psoriatic arthritis patients who received secukinumab as second-line with those that received secukinumab after two or more tumor necrosis factor-alpha (TNF-a) inhibitors. Treatment responses of the groups were measured and compared using the Bath Ankylosing Spondylitis Disease Activity Index (BASDAI) and Visual Analog Scale (VAS). Conclusion: Second-line secukinumab treatment resulted in a greater decline in BASDAI and VAS scores. [...]secukinumab achieved a significantly higher rate of remission when it was used as second-line therapy after one anti-TNF-a agent. Psoriatic arthritis (PsA) is a chronic inflammatory musculoskeletal disease that is seen in 10 to 40% of patients with psoriasis.1 Although the development of psoriasis precedes arthritis in most cases, approximately 15% of the patients develop psoriasis after presenting with arthritis.2 The treatment of PsA should focus on reducing arthritis and skin manifestations while preserving joint function and improving the quality of life.

[LANGUAGE=”English”]Erdheim-Chester disease (ECD) is a rare multisystem disease characterized by the proliferation of non-Langerhans histiocytes. It is characterized by excessive production and accumulation of histiocytes in multiple tissues and organs. Sites of involvement may include long bones, skin, eyes, lungs, brain, pituitary gland, and additional tissues and organs. The disease course varies depending on the organ and degree of involvement. In this case report, we presented the case of a 54-year-old female patient diagnosed with ECD, manifesting with squamous, enduring plaque lesions on the skin.[LANGUAGE=”Turkish”]Erdheim-Chester hastalığı (ECH) Langerhans hücre dışı histositlerin proliferasyonu ile seyreden nadir bir multisistem hastalığıdır. Birden fazla doku ve organda aşırı histiyosit üretimi ve birikimi ile karakterizedir. Tutulum bölgeleri arasında uzun kemikler, deri, göz, akciğerler, beyin, hipofiz bezi ve/veya ek doku ve organlar yer alabilir. Hastalık seyri organ tutulumu ve tutulum derecesine bağlı olarak değişkenlik gösterir. Bu olgu raporunda, deride skuamöz endüre plak lezyonlarla seyreden ECH tanısı konmuş 54 yaşında bir kadın olguyu ele aldık.

Cutaneo us vasculitis (CV) has a broad spectrum of etiologies, and drugs are one of the main culprits. With the increasing use of targeted therapies in medicine, especially in rheumatology and oncology, the number of CV cases reported due to these drugs has increased. Therefore, the recognition and treatment of CV associated with targeted agents have become more and more important. In the literature, anti-TNFs (n = 73, 59.5%), secukinumab (n = 7, 6%), rituximab (n = 5, 4%), tocilizumab (n = 1, 0.8%), ustekinumab (n = 8, 6.5%), abatacept (n = 3, 2.4%), Janus kinase inhibitors (n = 3, 2.4%), alemtuzumab (n = 3, 2.4%), and immune checkpoint inhibitors (n = 20, 16%) have been reported as responsible agents. However, our knowledge of the pathogenetic mechanisms is fairly limited, and the standardized management is yet to be established. Furthermore, though it is uncommon, this complication may pose a safety issue. In this manuscript, we reviewed the literature on CV with or without systemic involvement related to targeted agents. We also proposed the pathogenetic mechanisms of these adverse events. Thus, we aimed to make it easier for clinicians to manage similar cases by reviewing the diagnosis and treatment processes.Key Points• It should be kept in mind that targeted therapies can lead to CV with or without systemic signs, which sometimes withholds their uses.• Since targeted therapies are often used in the treatment of autoimmune and inflammatory diseases, CV associated with targeted therapies is difficult to recognize, and it is often mistakenly attributed to the underlying disease.• Although the pathogenesis of CV associated with targeted therapies is not fully understood, there are several proposed mechanisms.• Corticosteroids are the mainstay of treatment after discontinuation of the offending targeted agent.

PP patients were exposed to a higher number of biological agents than controls [median (Q1-Q3): 1 (1–2) vs. 1 (0–1), p = 0.005]. Besides palmoplantar involvement, trunk and extremity involvement were present in a few patients with PP and less frequent than in the control group (12% vs. 50%, p = 0.02 and 18% vs. 57%, p = 0.02, respectively). [...]patients with PP had significantly faster response times than patients with classical-type psoriasis. [...]even if the treatment options in PP and classical psoriasis are not different overall, biological switches can be deferred in PP. Concurrence of autoimmune/inflammatory diseases and the number of previously used biologics seem to be important predisposing factors towards PP. [...]wisely changing the biological therapy, especially in the presence of multiple autoimmune/inflammatory diseases, is suggested.

To demonstrate the burden of sexual dysfunction (SD) among females with rheumatic diseases, we conducted a cross-sectional comparative study in patients with systemic sclerosis (SSc), systemic lupus erythematosus (SLE), and Behçet’s syndrome (BS) along with suitable healthy controls (HCs). Age-matched female patients with SSc (n = 50), SLE (n = 49), and BS (n = 54), along with 52 female HCs were included in this study between April and October, 2021. Sociodemographic features were recorded, and psychometric tests, i.e., female sexual function index (FSFI), Beck depression inventory (BDI), body cathexis scale, and marital adjustment test (MAT) were performed. Scale scores were compared, and binary logistic regression was used to identify predictors for SD in the whole group. The total FSFI and body cathexis scores among the patient groups were significantly lower than those of the HCs (p < 0.001). Depression was significantly more frequent in the patient groups. MAT scores did not differ significantly between the study groups. Patients with SSc had the worst scores in each psychometric index, including MAT. Decreased body cathexis score [OR 0.974, 95% CI (0.957–0.991), p = 0.003] and low MAT score [OR 0.937, 95% CI (0.896–0.980), p = 0.005], and being diagnosed with SSc [OR 6.6, 95% CI (1.975–22.498), p = 0.002], SLE [OR 2.7, 95% CI (0.998–7.753), p = 0.050], and BS [OR 2.8, 95% CI (1.100–7.359), p = 0.031], were identified as independent predictors for SD. Body cathexis seems to be the most important independent predictor for SD, and the burden of SD appears heavier in patients with SSc, probably due to poor body image satisfaction.

Amiodarone can be used in a variety of arrhythmias. Given its widespread use, the probability of clinicians encountering its cutaneous adverse effects is high. A few cases of amiodarone-induced cutaneous vasculitis were reported in the literature, probably because it is underdiagnosed in clinical practice. Indeed, amiodarone-related cutaneous reactions may present a wide range of manifestations and are sometimes difficult to diagnose. Herein, we report a case with a sizeable necrotic ulcer on the left lower leg shortly after amiodarone exposure. A rigorous diagnostic study was performed before concluding the diagnosis of amiodarone-induced cutaneous vasculitis, which showed the histopathological features of leukocytoclastic vasculitis. The lesion was almost completely healed by the third month of discontinuation of amiodarone. We did a literature search and found seven cases which were reported as leukocytoclastic or lymphocytic vasculitis. We reviewed previous cases and presented our case in comparison to prior cases.

Erdheim-Chester disease (ECD) is a rare multisystem disease characterized by the proliferation of non-Langerhans histiocytes. It is characterized by excessive production and accumulation of histiocytes in multiple tissues and organs. Sites of involvement may include long bones, skin, eyes, lungs, brain, pituitary gland, and additional tissues and organs. The disease course varies depending on the organ and degree of involvement. In this case report, we presented the case of a 54-year-old female patient diagnosed with ECD, manifesting with squamous, enduring plaque lesions on the skin. Keywords: Erdheim Chester disease, skin, immunosuppressant, BRAF Erdheim-Chester hastaligi (ECH) Langerhans hucre disi histositlerin proliferasyonu ile seyreden nadir bir multisistem hastaligidir. Birden fazla doku ve organda asiri histiyosit uretimi ve birikimi ile karakterizedir. Tutulum bolgeleri arasinda uzun kemikler, deri, goz, akcigerler, beyin, hipofiz bezi ve/veya ek doku ve organlar yer alabilir. Hastalik seyri organ tutulumu ve tutulum derecesine bagli olarak degiskenlik gosterir. Bu olgu raporunda, deride skuamoz endure plak lezyonlarla seyreden ECH tanisi konmus 54 yasinda bir kadin olguyu ele aldik. Anahtar Kelimeler: Erdheim Chester hastaligi, deri, immunosupresan, BRAF

Objective: Few randomized controlled studies Investigating the role of plasma exchange (PLEX) therapy shown no significant benefit in the management of lupus nephritis. However small case series have suggested potential efficacy in certain types of organ involvement in systemic lupus erythematosus (SLE). Methods: We conducted a retrospective review of patient records who received PLEX therapy between October 2013 and March 2022 at our apheresis unit. Patients under the age of 18 and those who underwent PLEX therapy for non-rheumatic and rheumatic diseases other than SLE were excluded from the study. We collected comprehensive data including the primary indication for PLEX therapy, procedural details, concurrent immunosuppressive medications, overall survival, outcomes of organ involvement, and any complications associated with PLEX therapy. Results: Among 58 patients with rheumatic diseases who underwent PLEX therapy we included 17 SLE patients. The main indication for PLEX was catastrophic antiphospholipid syndrome (n=5), diffuse alveolar hemorrhage (DAH) (n=5), neuropsychiatric involvement (n=4), thrombotic microangiopathy (n=2) and renal involvement (n=1). Nine patients experienced severe/opportunistic infections resulting in death only in 1 patient during PLEX. Additionally, 3 patients died due to active disease during PLEX. Among the survived patients PLEX therapy provided remission in 13 patients. Conclusion: PLEX can be regarded as a supplementary treatment along with immunosuppressives, particularly for a subset of SLE patients experiencing conditions such as DAH and neuropsychiatric involvement. Despite high frequency of severe/opportunistic infections only one patient died. Keywords: Plasma exchange, plasmapheresis, systemic lupus erythematosus, lupus Amac: Plazma degisimi (PLEX) tedavisinin lupus nefriti yonetiminde anlamli bir fayda saglamadigini gosteren az sayida randomlze kontrollu calisma bulunmaktadir. Bununla birlikte, kucuk olgu serllerl, slstemik lupus eritematozusun (SLE) bazi organ tutulum tiplerinde plazma degisiminin etklll olabilecegini bildirmistir. Yontem: Ekim 2013 lle Mart 2022 tarihleri arasinda PLEX tedavisi alan hastalarin kayitlarini geriye donuk olarak inceledik. On sekiz yasin altindaki hastalar, SLE disinda romatizmal hastaligi olanlar ve romatizmal hastalik disi nedenlerle PLEX tedavisi yapilan hastalar dislandi. PLEX tedavisinin baslica endikasyonu, islem detaylari, eszamanli olarak kullanilan immunosupresif ilaclar, genel sagkalim, organ tutulumunun sonuclari ve PLEX tedavisi iliskili komplikasyonlar gibi bilgiler not edildi. Bulgular: Romatizmal hastaligi olup PLEX tedavisi uygulanan 58 hastadan 17 SLE hastasi calismaya dahll edildi. PLEX tedavisinin birincil endikasyonlari katastrofik antifosfolipid sendromu (n=5), diffuz alveolar hemorajl (DAH) (n=5), noropsikiyatrik tutulum (n=4), trombotik mlkroanjiyopati (n=2) ve renal tutulum (n=1) idi. PLEX sirasinda 9 hastada ciddi/firsatci enfeksiyonlar gelistigi goruldu. Bir hasta enfeksiyona bagli, 3 hasta aktif hastalik nedeniyle PLEX devam ederken kaybedildi. Sag kalan hastalarin 13'unde PLEX tedavisi ile remisyon saglandi. Sonuc: PLEX, DAH ve noropsikiyatrik tutulum gibi SLE hastalarinin belli bir alt grubu icin immunosupresiflerle birlikte ek bir tedavi olarak degerlendirilebilir. Hastalarimizin yaklasik yarisi ciddi veya firsatci enfeksiyonlarla karsilassa da, yalnizca bir hastada enfekslyona bagli mortalite gozlenmistir. Anahtar Kelimeler: Plazma degisimi, plazmaferez, sistemik lupus eritematozus, lupus

Objective: Meta-analysis of randomized controlled trials showed that plasma exchange (PLEX) has no significant effect on mortality and reduces the 12-month risk of end-stage kidney disease at the cost of increasing the risk of serious infections in patients with ANCA-associated vasculitis (AAV). Methods: We retrospectively reviewed patient charts who underwent PLEX therapy between October 2013 and March 2022 in our apheresis unit. Patients who were under 18 and underwent PLEX therapy for non-rheumatic and rheumatic diseases other than AAV were excluded. We collected all information regarding the primary indication of PLEX therapy, procedure details, concomitant immunosuppressives, overall survival, outcomes of organ involvement, and complications related to PLEX therapy. Results: Twenty-eight patients (Male/Female: 18/10) with AAV were evaluated. Diffuse alveolar hemorrhage (DAH) was the primary indication for PLEX therapy in 6 (21%) patients [myeloperoxidase (MPO)/proteinase-3 (PR-3): 1/5], kidney involvement and/or DAH in 22 (79%) (MPO/PR-3: 10/11). Overall, there were 16 (57%) severe/opportunistic infections and 5 (18%) deaths within the first three months. One (4%) severe infection (COVID-19) and 3 (11%) deaths were observed between 3 and 12 months. Overall, 10 (45%) patients were hemodialysis-dependent at month three, and there were no additional dialysis-dependent patients in the 12th month. Conclusion: most infections and deaths occurred within the first three months of PLEX therapy. The renal outcome was poor in patients with high-risk baseline creatinine levels ([greater than or equal to]5.8 mg/dL). Despite this, having no new dialysis-dependent patients between 3 and 12 months suggests that PLEX still can be an option as an adjunct therapy, especially in some subgroups of AAV patients in daily practice. Keywords: Plasma exchange, plasmapheresis, anti-neutrophil cytoplasmic antibody-associated vasculitis, organ damage, opportunistic infections, immunosuppressive agents Amaci Randomize kontrollu calismalarin meta anallzl, plazma degislminin (PLEX) mortallte uzerinde anlamli blr etkisinin olmadigini ve ANCA-iliskili vaskulit (AAV) hastalarinda ciddi enfeksiyon riskini artirma pahasina 12 aylik son donem bobrek hastaligi riskini azalttigini gostermistir. Yontem: Aferez unitemizde Ekim 2013-Mart 2022 tarihleri arasinda PLEX tedavisi uygulanan hastalarin dosya kayitlari retrospektif olarak incelendi. On sekiz yas alti ve romatizmal olmayan hasta li klari ve AAV disindaki romatizmal hastaliklari nedeniyle PLEX tedavisi alan hastalar calisma disi birakildi. PLEX tedavisinin birincil endikasyonu, islem detaylari, eslik eden immunosupresifler, genel sagkalim, organ tutulumunun sonuclari ve PLEX tedavisi ile ilgili komplikasyonlarla ilgili tum bilgiler toplandi. Bulgular: AAV'li 28 hasta (Erkek/Kadin: 18/10) degerlendirildi. Diffuz alveoler hemoraji (DAH) 6 (%21) hastada [miyeloperoksidaz (MPO)/proteinaz-3 (PR-3): 1/5], bobrek tutulumu ve/veya DAH 22 (%79) hastada PLEX tedavisinin birincil endikasyonuydu (mPO/PR-3: 10/11). Genel olarak, ilk uc ayda 16 (%57) ciddi/firsatci enfeksiyon ve 5 (%18) olum meydana geldi. Uc ila 12 ay arasinda bir (%4) ciddi enfeksiyon (COVID-19) ve 3 (%11) olum gozlemlendi. Genel olarak, 10 (%45) hasta ucuncu ayda hemodiyaliz bagimliydi ve 12. ayda baska diyalize bagimli hasta olmadi. Sonuc: Enfeksiyonlarin ve olumlerin cogu, PLEX tedavisinin ilk uc ayinda meydana geldi. Yuksek riskli baslangic kreatinin duzeyleri ([greater than or equal to]5,8 mg/dL) olan hastalarda renal sonuc kotuydu. Buna ragmen 3 ile 12. aylik sure arasinda yeni diyaliz hastasinin gelismemesi bize gunluk pratikte bazi alt grup hastalarda PLEX tedavisinin hala yardimci tedavi olarak tercih edilebilecegini dusundurmustur. Anahtar Kelimeler: Plazma degisimi, plazmaferez, anti-notrofil sitoplazmik antikorla iliskili vaskulit, organ hasari, firsatci enfeksiyonlar, immunosupresif ajanlar