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February 2015
Daphna Paran MD and Yaakov Naparstek MD
In the past decade we have witnessed a dramatic change in the management of autoimmune diseases, such as rheumatoid arthritis and spondyloarthropathies, due to the development of new biologic drugs designed to target key mediators in the autoimmune process. However, the development of similar target-specific drugs for the management of SLE has not been as successful. The B cell has long been considered central to the pathogenesis of SLE and has been regarded as an important target for biologic drugs. Several B cell-targeted drugs have been developed and although the mechanisms seem promising, most of the studies published to date have failed to achieve their primary endpoints, leading to an ongoing debate regarding the role of B cell therapy in SLE. The present report discusses the pros and cons of B cell-targeted therapy in SLE, reviews the clinical studies, and offers possible explanations for the discrepancies between randomized control studies and real-life experience. 

 
Shirish R. Sangle MBBS MD and David P. D’Cruz MD FRCP
Attila Kovacs MD PhD, Adelina G. Siminischi MD, Beáta Baksay MD, Andras Gall MD, Maria Takacs MD and Zoltan Szekanecz MD PhD
Siniša Roginic MD, Alan Jelic MD, Asja Stipic-Markovic MD PhD, Artukovic Marinko MD, Irena N. Artukovic MD and Martinovic-Kaliterna Dusanka MD PhD
Adam Austin MD, Angela Tincani MD, Shaye Kivity MD, María-Teresa Arango MSc and Yehuda Shoenfeld MD FRCP MaACR
Nurit Katz-Agranov MD, Amir Tanay MD, Daniel Bachar MD and Gisele Zandman-Goddard MD
Abdulla Watad MD, Alessandra Soriano MD, Hananya Vaknine MD, Yehuda Shoenfeld MD FRCP MaACR and Howard Amital MD MHA
Abdulla Watad MD, Marina Perelman MD, Ribhi Mansour MD, Yehuda Shoenfeld MD FRCP MaACR and Howard Amital MD MHA
January 2015
Maria A. Martínez-Godínez MSc MD1, Maria P. Cruz-Domínguez DSc, Luis J. Jara MD, Aarón Domínguez-López DSc, Rosa A. Jarillo-Luna DSc, Olga Vera-Lastra MD, Daniel H. Montes-Cortes DSc, Rafael Campos-Rodríguez DSc, Dulce M. López-Sánchez MSc, Cesar M. Mejía-Barradas DSc, Enrique E Castelán-Chávez MSc and Angel Miliar-García DSc

Background: The activated NLRP3 inflammasome is associated with the etiology of fibrotic diseases. The role of inflammasomes in SSc is still poorly understood.

Objectives: To determine the expression of NLRP3 (nucleotide-binding domain, leucine-rich-repeat-containing family, pyrin domain-containing 3) in the skin of patients with systemic sclerosis (SSc) and its relationship with pro-inflammatory cytokines and vascular mediators expression.

Methods: Skin biopsies were taken from 42 patients with either limited or diffuse SSc (21 lcSSc and 21 dcSSc), and from 13 healthy individuals. Using real-time polymerase chain reaction (PCR), the relative expression of caspase-1, IL-1β, IL-18, IL-33, TGF-β, ET-1, iNOS and eNOS genes, were measured. The location of NLRP3 and IL-1β were also determined by immunohistochemistry. Clinical characteristics were evaluated.

Results: The mean age of the patients was 49.3 ± 12.9 (lcSSc), 44.6 ±1 3.8 (dcSSc), and 45 ± 14.1 (healthy individuals). Compared to healthy individuals, the skin of both subtypes of SSc showed a significant increase (P < 0.05) in NLRP3, caspase-1, IL-1β, IL-18 and ET-1. Samples of lcSSc also showed a significant increase of eNOS (P < 0.029), iNOS (P < 0.04) and TGF-β (P < 0.05). Dermal fibrosis evaluated by modified Rodnan skin score (MRSS) had significant correlation with NLRP3, IL-1β, IL-18, and ET-1. Immunohistochemical analysis showed stronger staining of NLRP3 and IL-1β cytoplasmic expression in the keratinizing squamous epithelium of skin from SSc patients compared to controls.

Conclusions: This study identified NLRP3 over-expression in skin of patients with SSc. Skin thickness correlates positively with the NLRP3 inflammasome gene expression and with the vascular mediator and pro-fibrotic ET-1, suggesting that NLRP3 inflammasome plays a role in the pathophysiology of skin fibrosis in human SSc.

Zohar Mor MD MPH MPH, Orly Weinstein MD MHA, Dini Tischler-Aurkin MD MPA, Alex Leventhal MD MPH MPA, Alon Yaniv and Itamar Grotto MD PhD MPH

Background: Since 2006 more than 60,000 migrants arrived in Israel from the Horn of Africa (HoA: Sudan, Eritrea, Ethiopia). They were detained in prison and screened for tuberculosis (TB) by means of an interview and chest X-ray (CXR).

Objectives: To evaluate the yield of this screening process.

Methods: This cross-sectional study evaluated the validity of CXR in a random sample of 1087 of the 5335 HoA migrants (20.4%) who arrived in 2009, and assessed its related costs.

Results: Sixty-two migrants (5.7%) had CXRs with TB-suspicious findings, and 11 of them were finally diagnosed with TB (17.7% of all TB-suspicious CXRs). TB point-prevalence was 1000 cases per 100,000 migrants (1.0%). As no additional TB cases were diagnosed on arrival, CXR sensitivity, specificity and positive predictive value were 100%, 96.1% and 17.7%, respectively. The interview did not contribute to the detection of migrants with TB. Direct costs related to the detection of single TB cases in prison was 17,970 shekels (US$ 4585), lower than the treating cost of 28,745 shekels ($ 7335). During 2008–2010, 88 HoA migrants who had been screened at the prison after crossing the border were later diagnosed with TB in the community. The average annual TB incidence was 132 cases/100,000 migrants. We traced 56 (63.6%) of the CXRs that were performed during detention. Of those, 41 (73.2%) were unremarkable, 8 (14.2%) were TB suspicious and 7 (12.5%) had non-TB-related abnormalities.

Conclusions: CXR-based screening is a valid and cost-saving tool for screening  HoA migrants for TB; the interview has significant limitations. 

Przemyslaw Kotyla MD PhD, Katarzyna Jankiewicz-Ziobro MD PhD, Aleksander Owczarek MD PhD and Eugene J. Kucharz MD PhD

Background: Targeted anti-tumor necrosis factor-alpha (TNFα) therapy in patients with rheumatoid arthritis (RA) has resulted in dramatic improvement in the course of the disease and prognosis. One of the features of RA is hyperplasia of synovial cells, particularly RA synovial fibroblasts (RA-SF), caused partially by impaired apoptosis of RA-SF cells. It has been shown that TNFα may inhibit apoptosis in RA-SF cells and this process may be reversed by the use of TNFα antagonists.

Objectives: To determine the influence of etanercept, an anti-TNFα agent, on sFas (CD 95) receptor.

Methods: We analyzed serum levels of sFaS and TNFα in a group of 26 patients with high RA disease activity who were selected to start treatment with etanercept. Assessment of sFas receptor and TNFα levels was performed before and 6 months after treatment with etanercept.

Results: Treatment with etanercept resulted in increased TNFα levels (log TNFα 0.602 vs. 1.17, P < 0.05) but no change in sFas levels (log sFas 3.17 vs. 3.11, P = 0.37). As expected, treatment resulted in significant reduction in both disease activity and levels of inflammatory markers.

Conclusions: Etanercept may increase TNFα levels in patients with RA. We also speculate that the Fas pathway is not the main apoptotic pathway in patients with RA treated with etenercept, since sFas, a marker of apoptotic activity, remained unchanged and was not influenced by disease activity and concomitant treatment. 

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