The Role of Myositis-Specific and Myositis-Associated Autoantibodies and the Activation of Type I Interferon Pathway in the Generation of Clinical Phenotypes of Inflammatory Myopathies.

Idiopathic inflammatory myopathies (IIMs) are a group of heterogeneous autoimmune diseases with a prevalence of 20 cases per 100000 of population. Despite their diversity, IMMs are characterised by several common clinical features such as muscle inflammation, proximal muscle weakness, abnormal electromyography and/or muscle biopsy. Over the last years, it has been increasingly recognised that an array of autoantibodies known as myositis-specific antibodies (MSAs) and myositis-associated antibodies (MAAs) are associated with distinct clinical phenotypes and diverse prognosis. Although the exact underlying mechanism of IIMs is not fully understood, accumulating data suggest that the activation of type I interferon pathway plays a central role in disease development. Previous studies have reported the upregulation of type I interferon (IFN) induced genes in peripheral blood and muscle biopsies derived from myositis patients. Given the heterogeneity of inflammatory myopathies along with the central role of type I IFN pathway in disease pathogenesis, the aim of the current study is to elucidate the link between distinct clinical phenotypes of inflammatory myopathies with the presence of serum MSAs or MAAs, as well as with type I IFN activation.

Epidemiological trends in cutaneous lymphomas in Greece.

Primary cutaneous lymphomas (PCLs) are a heterogenous group of non-Hodgkin lymphomas arising in the skin from T- or B-lymphocytes, for which there is limited epidemiological data available. To describe the disease characteristics and estimate annual incidence rates (IRs) and temporal trends of PCLs and their subtypes in Attica, Greece. A retrospective analysis of all PCL patients, diagnosed in Attica's main haemopathology referral centre from 2009 to 2021, was conducted. In total, 1,189 patients were included; 725 males and 464 females (males__females=1.56). The median age at diagnosis was 62 years. The annual IR was 2.2 new cases per 100,000 individuals. Most patients (n=979, 82.3%) were diagnosed with cutaneous T-cell lymphoma (CTCL) with a crude IR of 1.8 new cases per 100,000 person-years. Mycosis fungoides (MF) was the most common subtype (n=817, 68.7%), followed by lymphomatoid papulosis (LyP) (n=59, 5.0%). The crude IR for MF was 1.5 new cases per 100,000 person-years. Cutaneous B-cell lymphomas (CBCLs) accounted for 17.6% (n=210) of all PCLs (IR: 0.4 new cases per 100,000 person-years). PCL, CTCL and MF incidence rates increased from 2009 to 2019, followed by a decrease in 2020-2021. The incidence rate of CBCL increased steadily during the study period. The annual IRs of PCL in Greece were higher than those reported in other studies from Europe, America and Asia. The increase in IRs from 2009 to 2019 may reflect physicians' improved diagnostic efficiency. The COVID-19 pandemic may be the reason for the decline in PCL, CTCL and MF diagnoses from 2020 to 2021.

Harlequin syndrome in a young girl: An opportunity to rethink the terminology.

Harlequin syndrome is a rare disorder of the autonomic nervous system, presenting as unilateral reduced flushing and sweating of the face induced by exercise, stress, or heat. It is caused by a cervical sympathetic deficit located at the preganglionic or postganglionic level on the non-flushing side. We present a case of an 8 year old with harlequin syndrome and review the other dermatological conditions for which the term "harlequin" is part of the nomenclature.

Lymphoprolipherative skin reactions induced by anti-TNFα: an open question.

Although anti-TNFα agents have revolutionized the treatment of many inflammatory diseases, various concerns have been reported regarding the risks of cancer development, as well as acceleration of the progression of subclinical, preexisting malignancies. In this case series, we investigated the provocative effect of anti-TNFα drugs in the development of cutaneous mycosis fungoides (MF)-like lymphoproliferative reactions. We describe five patients aged between 25-63 diagnosed with autoimmune disorders (psoriatic arthritis - one patient, Crohn's disease - one patient and ankylosing spondylitis - three patients) who received anti-TNFα agents before the development of a cutaneous lymphoproliferative reaction. Histological and immunophenotypical analysis was typical for mycosis fungoides in all of them. Anti-TNFα agents were stopped with regression of the skin rash. A direct effect of anti-TNFα agents in the development of lymphoproliferative reactions (including MF) is suggested and further analyzed. Treatment cessation can be therapeutic.

Comparison of Unilateral versus Bilateral Kyphoplasty in Multiple Myeloma Patients and the Importance of Preoperative Planning.

STUDY DESIGN: Retrospective comparative study and technical note. PURPOSE: To determine if there is a difference in clinical and radiographic parameters between unilateral and bilateral kyphoplasty in a uniform cancer population and to stress the importance of preoperative planning. OVERVIEW OF LITERATURE: While unipedicular kyphoplasty is gaining popularity, a few comparative studies have reported on superior kyphotic reduction with the bipedicular approach. METHODS: We reviewed 69 myeloma patients with 105 operated levels (51 levels were done bilaterally vs. 54 unilaterally). Pain reduction, height restoration, cement volume and complications were recorded up to three months postoperatively. A technical note to identify the skin entry point on the basis of the magnetic resonance imaging and fluoroscopy (lateral view) is being described. RESULTS: Both procedures resulted in significant pain reduction (5.4-5.6/10 points, p=0.8). There was significant height restoration after the operation (p<0.001), while there was no sustained difference between the procedures (p=0.5) up to three months postoperatively. More cement was injected in the bilateral group (4.1 mL vs. 4.9 mL, p=0.002); no difference in cement extravasation in the spinal canal was observed (p=0.5). CONCLUSIONS: There was no difference in the clinical or radiological outcomes between the unilateral and bilateral approaches. Therefore, unilateral kyphoplasty may be performed whenever it is technically feasible and this may be determined preoperatively.

Controversial issues in kyphoplasty and vertebroplasty in osteoporotic vertebral fractures.

Kyphoplasty (KP) and vertebroplasty (VP) have been successfully employed for many years for the treatment of osteoporotic vertebral fractures. The purpose of this review is to resolve the controversial issues raised by the two randomized trials that claimed no difference between VP and SHAM procedure. In particular we compare nonsurgical management (NSM) and KP and VP, in terms of clinical parameters (pain, disability, quality of life, and new fractures), cost-effectiveness, radiological variables (kyphosis correction and vertebral height restoration), and VP versus KP for cement extravasation and complications profile. Cement types and optimal filling are analyzed and technological innovations are presented. Finally unipedicular/bipedicular techniques are compared. Conclusion. VP and KP are superior to NSM in clinical and radiological parameters and probably more cost-effective. KP is superior to VP in sagittal balance improvement and cement leaking. Complications are rare but serious adverse events have been described, so caution should be exerted. Unilateral procedures should be pursued whenever feasible. Upcoming randomized trials (CEEP, OSTEO-6, STIC-2, and VERTOS IV) will provide the missing link.

Controversial issues in kyphoplasty and vertebroplasty in malignant vertebral fractures.

BACKGROUND: Kyphoplasty (KP) and vertebroplasty (VP) have been successfully employed in the treatment of pathological vertebral fractures. METHODS: A critical review of the medical literature was performed and controversial issues were analyzed. RESULTS: Evidence supports KP as the treatment of choice to control fracture pain and the possible restoration of sagittal balance, provided that no overt instability or myelopathy is present, the fracture is painful and other pain generators have been excluded, and positive radiological findings are present. Unilateral procedures yield similar results to bilateral ones and should be pursued whenever feasible. Biopsy should be routinely performed and 3 to 4 levels may be augmented in a single operation. Higher cement filling appears to yield better results. Radiotherapy is complementary with KP and VP but must be individualized. CONCLUSIONS: In cases of painful cancer fractures, if overt instability or myelopathy is not present, unilateral KP should be pursued, whenever feasible, followed by radiotherapy. The technological advances in hardware and biomaterials, as well as combining KP with other modalities, will help ensure a safe and more effective procedure. Address.

Adverse prognostic factors and optimal intervention time for kyphoplasty/vertebroplasty in osteoporotic fractures.

INTRODUCTION: While evidence supports the efficacy of vertebral augmentation (kyphoplasty and vertebroplasty) for the treatment of osteoporotic fractures, randomized trials disputed the value of vertebroplasty. The aim of this analysis is to determine the subset of patients that may not benefit from surgical intervention and find the optimal intervention time. METHODS: 27 prospective multiple-arm studies with cohorts of more than 20 patients were included in this meta-analysis. We hereby report the results from the metaregression and subset analysis of those trials reporting on treatment of osteoporotic fractures with kyphoplasty and/or vertebroplasty. RESULTS: Early intervention (first 7 weeks after fracture) yielded more pain relief. However, spontaneous recovery was encountered in hyperacute fractures (less than 2 weeks old). Patients suffering from thoracic fractures or severely deformed vertebrae tended to report inferior results. We also attempted to formulate a treatment algorithm. CONCLUSION: Intervention in the hyperacute period should not be pursued, while augmentation after 7 weeks yields less consistent results. In cases of thoracic fractures and significant vertebral collapse, surgeons or interventional radiologists may resort earlier to operation and be less conservative, although those parameters need to be addressed in future randomized trials.

Defining the indications, types and biomaterials of corpectomy cages in the thoracolumbar spine.

Osteomyelitis, tumors and fractures of the thoracolumbar spine comprise a wide spectrum of pathology of the anterior column that can be safely addressed with cages. Mesh cages have been traditionally used; however, expandable devices are gaining popularity due to better correction of sagittal deformity, less subsidence and technical advantages (easier to insert especially through a posterior approach and tighter fit). In addition, nonmetallic cages (poly-ether-ether-ketone/carbon fibers, hydroxyapatite and ceramics) offer some distinct advantages over titanium, being more inert/biocompatible, osteoconductive and radiolucent. Treatment is also shifted towards minimally invasive surgery, rendering corpectomy a far less-morbid operation than it used to be.

Double approach operation for large extracompartmental proximal thigh tumors.

Resection of large femoral triangle tumors that invade the bone (or vice versa) still remains a challenge. A lateral-only approach would hinder dissection of the mass, away from the femoral vessels, while an iliofemoral-only type of approach would make bone resection and megaprosthetic reconstruction very arduous. The authors describe a two-stage, one-position operation via a double surgical approach: the first stage is comprised by an iliofemoral approach and dissection of the femoral vessels, followed by proximal femoral resection and reconstruction stage. One illustrative case is presented along with the authors overall experience. We believe that this operation facilitates wide tumor resection in a safe and step-wise manner, as not to add to the morbidity of the procedure.