Neutrophil-to-Lymphocyte Ratio as a Biomarker for Clinical Response After Autologous Hematopoietic Stem Cell Transplantation in Systemic Sclerosis.

Introduction: Systemic sclerosis is a complex disease that affects various target organs, making it difficult to assess response and determine remission or relapse. A baseline Neutrophil-to-Lymphocyte Ratio (NLR) >2.95 is associated with severe progressive skin and lung disease and decreased 5-year survival in systemic sclerosis (SSc).

Outcomes in progressive systemic sclerosis treated with autologous hematopoietic stem cell transplantation compared with combination therapy.

Objectives: Autologous hematopoietic stem cell transplantation (AHSCT) has been shown to improve long-term survival for early diffuse progressive SSc compared with CYC. CYC, however, does not provide a long-term benefit in SSc. The combination of MMF and rituximab is a potent alternative regimen.

Giant Cell Arteritis: State of the Art in Diagnosis, Monitoring, and Treatment.

Giant cell arteritis (GCA) is the most prevalent subtype of vasculitis in adults. In recent years, there has been substantial improvement in the diagnosis and treatment of GCA, mainly attributed to the introduction of highly sensitive diagnostic tools, incorporation of modern imaging modalities for diagnosis and monitoring of large-vessel vasculitis, and introduction of highly effective novel biological therapies that have revolutionized the field of GCA. This article reviews state-of-the-art approaches for the diagnosis, monitoring, and treatment options of GCA.

Nailfold video capillaroscopy as a useful diagnostic tool in systemic vasculitis.

Background: Nailfold video capillaroscopy (NVC) enables us a direct view of the microvasculature. Only several capillaroscopy studies in adult patients with vasculitis have been reported.

Aim: To characterize NVC changes in vasculitis.

Low Urine Secretion of Semaphorin3A in Lupus Patients with Proteinuria.

Immune semaphorins are important in controlling both innate and adaptive immune responses. The regulatory role of semaphorin3A (sema3A) in systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), and other autoimmune diseases is widely reported. Decreased levels of serum sema3A were shown to correlate with SLE disease activity.

Autologous Hematological Stem Cell Transplantation for Systemic Sclerosis in Israel.

Background: Autologous hematological stem cell transplantation (HSCT) is a novel therapy for systemic sclerosis (SSc) that has been validated in three randomized controlled trials.

Objectives: To report the first Israeli experience with HSCT for progressive SSc and review the current literature.

Methods: Five SSc patients who were evaluated in our department and were treated by HSCT were included.

Crowned dens syndrome, yet another rheumatic disease imposter.

Objective: Crowned dens syndrome (CDS) is defined as acute cervical or occipital pain due to a local inflammatory reaction related to calcifications in the ligaments surrounding the odontoid process. Virtually, all previous descriptions of CDS have related to calcium pyrophosphate dehydrate (CPPD) arthropathy.

Methods: We prospectively identified a total of twenty-four consecutive inpatients with Crowned dens syndrome from January 2016 to December 2017 in our institution.

Ulcerative Colitis and Familial Mediterranean Fever: Can Anakinra Treat Both?

Anakinra is a biological drug used in rheumatoid arthritis and several autoinflammatory diseases. Its main side effects are injection site reactions and increased infection rate. We present a 28-year-old man with familial Mediterranean fever, whose disease went into remission on anakinra, with concomitant flare of his ulcerative colitis.

Infliximab for the treatment of refractory polyarteritis nodosa.

Polyarteritis nodosa (PAN) is a necrotizing vasculitis predominantly affecting medium and small size arteries. Cyclophosphamide, a drug with narrow therapeutic range and poor safety profile, constitutes the treatment of choice for PAN vasculitis with major organ involvement. To describe our clinical experience in treating refractory PAN with infliximab (a TNF inhibitor), a drug with good tolerability and better safety profile than cyclophosphamide.

Lysyl oxidase-a possible role in systemic sclerosis-associated pulmonary hypertension: a multicentre study.

Objective: Lysyl oxidase (LOX) is an extracellular enzyme that cross-links collagen fibrils. LOX was found to be increased in serum of SSc patients and was suggested to be related to skin fibrosis, yet a vascular source of LOX has been demonstrated in idiopathic pulmonary arterial hypertension (iPAH). We aimed to validate elevated LOX serum levels in SSc and to study its correlation with clinical characteristics and investigate its main source at the tissue level.

A high and equal prevalence of the Q703K variant in NLRP3 patients with autoinflammatory symptoms and ethnically matched controls.

Objectives: Cryopyrin associated periodic syndromes (CAPS) comprise a spectrum of autoinflammatory disorders of varying severity caused by mutations in the NLRP3 gene. The NLRP3-Q703K allele has been reported both as a functional polymorphism and as a low penetrance mutation.

Methods: To describe the clinical phenotype of subjects with the Q703K allele and to report the frequency of this allele among patients with autoinflammatory symptoms and healthy controls.

[THE ROLE OF SEMAPHORIN 7A IN SYSTEMIC SCLEROSIS].

Introduction: Semaphorins are a large group of membrane bound and secreted proteins. The semaphorins were first recognized for their important role in neurodevelopment and specifically their repulsive axonal growth guidance during embryonic development. Recently, semaphorins have also been found to have an important role in the regulation of the immune system, thus denoted as "immune semaphorins".

[SUSTAINABILITY OF RITUXIMAB IN CONCOMMITANT TREATMENT WITH METHOTREXATE OR LEFLUNOMIDE IN PATIENTS WITH RHEUMATOID ARTHRITIS].

Introduction: Rituximab is a biologic agent approved for the treatment of rheumatoid arthritis (RA) in combination with methotrexate (MTX) or leflunomide (LEF). However, limited data in the literature suggests that rituximab may have the same efficacy profile whether used in combination with MTX or as monotherapy. The aim of our study is to compare the sustainability of rituximab as monotherapy to combined therapy with MTX or LEF in Israeli patients with RA.

Radionuclide Methods in the Diagnosis of Sacroiliitis in Patients with Spondyloarthritis: An Update.

Sacroiliitis, inflammation of the sacroiliac joint (SIJ), is the hallmark of ankylosing spondylitis and spondyloarthritis (SpA) in general. The arsenal of recommended diagnostic modalities for imaging of the SIJ is scanty and, in practice, includes only conventional X-rays and magnetic resonance imaging (MRI). This review suggests that bone scintigraphy, particularly single-photon emission computed tomography (SPECT) with calculation of indices, or SPECT in combination with low-dose computed tomography (CT) can be a sensitive and specific tool for the diagnosis of sacroiliitis and can be used as part of the individualized approach to the diagnosis of axial SpA.

Semaphorin 3A, a potential immune regulator in familial Mediterranean fever.

Objectives: Semaphorin 3A (sema3A) plays a regulatory role in immune responses with effects on both T and B regulatory cells. Familial Mediterranean fever (FMF) is an autoinflammatory disease, yet a possible role for regulatory T and B cells has been described.

Methods: 17 FMF patients during attack and then in remission, 8 FMF patients with smoldering disease and 12 healthy controls were enrolled.

Q Fever Risk in Patients Treated with Chronic Antitumor Necrosis Factor-Alpha Therapy.

Q fever is a zoonotic bacterial disease caused by Coxiella burnetii. Tumor necrosis factor-alpha (TNF-α) plays a pivotal role in the defense against infection with this Gram-negative coccobacillus. Theoretically, patients who are treated with anti-TNF-α medications are at risk for developing chronic Q fever.

Tocilizumab Efficacy in a Patient with Positive Anti-CCP Chronic Lyme Arthritis.

Context: Lyme arthritis, a manifestation of tick-borne Lyme disease, can prove to be refractory to classic treatment.

Case Report: We present a case of a 48-year-old male, diagnosed with chronic Lyme arthritis, refractory to recurrent and prolonged courses of doxycycline, ceftriaxone, as well as hydroxychloroquine and methotrexate. The patient responded partially to tumor necrosis factor (TNF)-alpha blockade by etanercept and, finally, entered long-term remission after his treatment was switched to tocilizumab.