Braswellbladt0760

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is sometimes complicated by anti-glomerular basement membrane (GBM) disease. Proteases, including elastase, released from neutrophils activated by ANCA are implicated in the pathogenesis of AAV. Epitopes of anti-GBM antibody exist in the α3-subunit non-collagenous (NC1) domain of collagen type IV [Col (IV)]. This region, called α3(IV)NC1, is structurally cryptic. This study aimed to determine the production mechanism of anti-GBM antibody in AAV.

We first examined whether α3(IV)NC1 could be revealed by the digestion of formalin-fixed, paraffin-embedded (FFPE) normal kidney sections and Col (IV) by proteases, including neutrophil elastase, using immunohistochemistry (IHC) and enzyme-linked immunosorbent assay (ELISA). Next, the reveal of α3(IV)NC1 and the infiltration of CD11c+ macrophages in the affected kidneys were evaluated by IHC and immunofluorescent staining using FFPE sections. Finally, the production of anti-GBM antibody in AAV rats was determined by ELISA.

α3(IV)NC1 was revealed by the digestion of FFPE normal kidney sections and Col (IV) by proteases. Although the reveal of α3(IV)NC1 was observed in sclerotic glomeruli regardless of causative diseases, CD11c+ macrophages near α3(IV)NC1 were characteristics of AAV. Anti-GBM antibody was produced subsequent to ANCA in some AAV rats. IHC demonstrated the reveal of α3(IV)NC1 in affected renal tissues and the infiltration of CD11c+ macrophages around the sites.

The collective findings suggest that, in AAV, proteases released from neutrophils activated by ANCA digest Col (IV) and result in the reveal of α3(IV)NC1, CD11c+ macrophages present GBM epitopes, and then the host's immune system produce anti-GBM antibody.

The collective findings suggest that, in AAV, proteases released from neutrophils activated by ANCA digest Col (IV) and result in the reveal of α3(IV)NC1, CD11c+ macrophages present GBM epitopes, and then the host's immune system produce anti-GBM antibody.

To define the clinical spectrum time-course and prognosis of non-Asian patients positive for anti-MDA5 antibodies.

We conducted a multicentre, international, retrospective cohort study.

149 anti-MDA5 positive patients (median onset age 53 years, median disease duration 18 months), mainly females (100, 67%), were included. Dermatomyositis (64, 43%) and amyopathic dermatomyositis (47, 31%), were the main diagnosis; 15 patients (10%) were classified as interstitial pneumonia with autoimmune features (IPAF) and 7 (5%) as rheumatoid arthritis. The main clinical findings observed were myositis (84, 56%), interstitial lung disease (ILD) (108, 78%), skin lesions (111, 74%), and arthritis (76, 51%). The onset of these manifestations was not concomitant in 74 cases (50%). Of note, 32 (21.5%) patients were admitted to the intensive care unit for rapidly progressive-ILD, which occurred in median 2 months from lung involvement detection, in the majority of cases (28, 19%) despite previous immunosuppressive treatmenthould be considered even when baseline autoimmune screening is negative, anti-Ro52 kDa antibodies are positive, and radiology findings show a NSIP pattern.

The idiopathic inflammatory myopathies (IIMs) are a heterogeneous group of immune-mediated systemic disorders that commonly target skeletal muscles. The aim of our review is to remind clinicians to be vigilant of common mimickers, and what red flags to look for to avoid misdiagnosis.

We reviewed the clinical documentation and investigation results of illustrative real-life case examples of significant IIM mimickers with valuable learning points. Following an initial diagnosis of IIM, the patients had been referred to our Adult Neuromuscular Service at Salford Royal Hospital Northern Care Alliance NHS Foundation Trust, UK.

Four cases, two males and two females, were analysed. Retrospective review of key case-specific features suggestive of alternative diagnoses were identified and described, prompting a broader discussion of common disease groups that can mimic IIM.

The presentation of IIM is heterogeneous and the differential diagnosis wide. Several non-inflammatory conditions can present as mimickers of IIM, each requiring a different management approach.

The presentation of IIM is heterogeneous and the differential diagnosis wide. Several non-inflammatory conditions can present as mimickers of IIM, each requiring a different management approach.

Vascular Behçet's disease (VBD) is a systemic vasculitis involving both arterial and venous vessels of all sizes and occurring in up to 40% of patients with BD. VBD is the main cause of mortality in BD. Although commonly seen around the Mediterranean region, comparative studies in VBD are lacking. We aimed to compare the course and therapeutic approaches of VBD in two large cohorts from Turkey and France.

We included 291 VBD patients (female/male63/228, mean age 41.2±11.3 years) who were followed up in the Department of Internal Medicine and Clinical Immunology at Pitié-Salpêtrière Hospital, Sorbonne University, Paris, France (n=131) and Rheumatology Division of Marmara University School of Medicine, Istanbul, Turkey (n=160). All clinical and demographical data were acquired from patient charts retrospectively.

Smoking, family history for BD, HLA-B*51 presence and pathergy positivity were significantly higher in Turkish patients (TR), while neurologic involvement was more prominent in the French (FR) group. After a median follow-up of 77 months, 562 vascular events occurred including 440 venous events, 115 arterial events and 7 cardiac thrombi. In 79 (29%) patients, first vascular event developed before BD diagnosis and for 77 (28%) of them, vascular involvement was the presenting sign of the disease. First relapse developed in 130 (44.7%) patients after median 24.5 (1-276) months of follow-up (TR 46.3% (n=74), FR 42.7% (n=56), p=0.56). Survival graph revealed that FR cohort has 1.64 times increased recurrent event risk compared to TR cohort (HR=1.64 (1.1-2.44), p=.014) and although did not reach to statistical significance, IS treatment after the first vascular event decreased further vascular events (HR= 0.66 (0.43-1.01, p=.057).

Almost half of patients relapsed of VBD within 2 years after the first vascular event. Immunosuppressants decrease VBD relapses.

Almost half of patients relapsed of VBD within 2 years after the first vascular event. Immunosuppressants decrease VBD relapses.

To establish the value of a modified Disease Activity score with Optical Spectral Transmission score (DAS-OST) without joint counts but with a HandScan score, versus that of DAS28, to classify rheumatoid arthritis (RA) as active versus inactive, with as reference standard the rheumatologist's clinical classification.

RA patients with at least one HandScan and DAS28 measurement performed at the same visit were included. Data was extracted from medical records, as was the clinical interpretation as active or inactive RA by the rheumatologist. Logistic regression analyses were performed to calculate areas under the receiver operating characteristics (AU-ROC) curves. The clinical interpretation was used as reference standard in all analyses, and disease activity measures were used as predictor variables. The performance of predictor variables (AU-ROCs) was compared.

The data of 1505 RA patients were used for analyses. The highest AU-ROC of 0.88 (95%CI 0.85-0.90) was shown for DAS28; AU-ROC of DAS-OST was 0.78 (95%CI 0.75-0.81), difference 0.10, p<0.01.

Compared to DAS28, DAS-OST classified RA statistically significantly less well as active versus inactive, when using the clinical classification as reference standard. However, a DAS-modification without joint scores might have a place in strategies limiting routine outpatients' visits to the rheumatologist.

Compared to DAS28, DAS-OST classified RA statistically significantly less well as active versus inactive, when using the clinical classification as reference standard. However, a DAS-modification without joint scores might have a place in strategies limiting routine outpatients' visits to the rheumatologist.

The aim of this study was to evaluate the prevalence and severity of overactive bladder syndrome (OAB) and sexual dysfunction in fibromyalgia (FM) patients, as well as their relationship with disease severity.

Consecutive adult female patients with FM were enrolled. Patients filled in a comprehensive questionnaire package including demographic variables, disease severity assessment (revised Fibromyalgia Impact Questionnaire [FIQR]), neuropathic pain features (PainDetect Questionnaire [PDQ]), severity of OAB symptoms (Overactive Bladder Symptom Score [OABSS]), and determining sexual functioning (Female Sexual Function Index [FSFI]).

The study included 481 patients, 116 (24.11%) had mild OAB, 82 patients (17.04%) had moderate OAB, and 34 patients had serious OAB (7.06%). In 14.17% of patients the bladder condition was causing them major issues in terms of discomfort. In 7.87% of patients the bladder condition was causing them significant problems. Sexual dysfunctions were found in 91 patients (18.91%). Using the FSFI as dependent variable, multivariate analysis revealed a positive relationship between sexual dysfunction and variables of disease burden (FIQR, p<0.0001; PDQ, p<0.0001, widespread pain index [WPI], p=0.0037). Using OABSS as the dependent variable, multivariate regression revealed a substantial contribution from FIQR (p<0.0001), PDQ (p=0.0037), and WPI (p=0.0030).

FM has the potential to affect both psychological and physiological processes in women with OAB and sexual dysfunction. These results emphasize the importance of a multidisciplinary approach to treat patients with overactive bladder syndrome and sexual dysfunction in FM.

FM has the potential to affect both psychological and physiological processes in women with OAB and sexual dysfunction. These results emphasize the importance of a multidisciplinary approach to treat patients with overactive bladder syndrome and sexual dysfunction in FM.

Rheumatoid arthritis (RA) is a chronic disease, requiring frequent patient-provider interaction and self-monitoring. We developed a novel mobile health smartphone app with a voice-enabled feature to help patients virtually track disease activity and ask general questions about RA.

With a user-centered design (UCD) approach, we developed a voice-enabled app (VEA) which was then tested in two focus groups of patients (n=8) and one with providers (n=4). Voice enablement and a question and answer (Q & A) library function were previously requested by patients. Based on focus group feedback, the VEA was refined and tested with 26 patients for 56 days. The VEA asked patients to fill in daily patient-reported outcomes (PROs) and complete the trial with a satisfaction survey.

Of the 26 patients in the VEA trial, 77% were female and 50% were aged 55 and older. Adherence to daily PROs during the 56-day trial was 66%, with <1% of PROs completed using the voice-enabled feature. PROMIS short forms and RADAI-5 PROs remained stable. buy Entinostat Of the 22 satisfaction survey respondents, 86% were satisfied with their overall experience with the app and 18.5% were satisfied with voice enablement. The voice assistant had an 86% success rate at understanding and answering interactions regarding surveys and a 44% success rate regarding Q & A interactions.

We developed a novel VEA through a UCD framework and conducted pilot testing. Adherence was moderate and RADAI-5 and PROMIS measures were stable. Based on satisfaction results, PROs may not be the best use of voice enablement technology.

We developed a novel VEA through a UCD framework and conducted pilot testing. Adherence was moderate and RADAI-5 and PROMIS measures were stable. Based on satisfaction results, PROs may not be the best use of voice enablement technology.