COPA Syndrome-From Pathogenesis to Treatment.

Coatomer subunit α (COPA) syndrome is a mendelian autosomal dominant immune dysregulation disease characterized by early onset lung disease in the form of diffuse alveolar hemorrhaging or interstitial lung disease, frequently associated with arthritis, glomerulonephritis, and high titer autoantibodies usually mimicking other autoimmune diseases. While immunosuppressive medication has been effective in controlling arthritis, data on long-term lung disease control remains scarce, which poses a real challenge as the progression of lung disease is the main cause of poor life expectancy in COPA patients. Nevertheless, JAK inhibitor therapy seems to be the most promising therapeutic choice now.

Assessment of Neutrophil-Lymphocyte Ratio and Systemic Immune-Inflammation Index in Systemic Scleroderma Patients with Focus on Cardiopulmonary Manifestations.

Objectives: To quantify levels of two inflammation-related indexes, namely neutrophil-lymphocyte ratio (NLR) and systemic immune-inflammation index (SII) in systemic scleroderma patients and determine the association with clinical manifestations and features of heart ultrasound.

Methods: The study group consisted of 34 patients with diagnosis of systemic scleroderma which were admitted to the hospital during 2015-2019. Patient data included the presence and type of clinical manifestations of systemic scleroderma, chest imaging to screen for lung disease, heart ultrasound reports and the laboratory investigations needed to quantify inflammatory indexes.

Association between Left Atrial Function and Survival in Systemic Sclerosis.

Systemic sclerosis (SSc) is a multisystemic autoimmune disorder in which cardiac involvement is frequent and portends negative prognosis. Left ventricular (LV) diastolic dysfunction is one of the most common cardiac alterations in these patients, and left atrial (LA) reservoir strain (Ɛ) measurement using speckle tracking echocardiography has been proposed as a novel parameter for a better assessment of LV diastolic function. Therefore, the aim of this study was to test the prognostic value of Ɛ in a large multicenter cohort of SSc patients.

Capillaroscopic Insights: Exploring the Connection Between Microvascular Changes and Pulmonary Manifestations in Systemic Sclerosis.

Background: Systemic sclerosis (SSc) is a complex connective tissue disease characterized by microangiopathy, immune dysregulation, and fibrosis. Early detection of microvascular abnormalities using nailfold videocapillaroscopy (NVC) is crucial in assessing disease progression and associated disease's involvement such as interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH).

Objective: This study aims to explore the relationships correlation between NVC patterns, clinical manifestations, and systemic complications in SSc.

Skin Ultrasound Assessment of Patients with Systemic Scleroderma-An Observational Study.

This study aims to analyze the changes in dermal thickness in patients with systemic scleroderma (SSc) in comparison with normal skin and also compare clinical forms with diffuse and limited cutaneous involvement. The study group consisted of female patients diagnosed with SSc with a disease history not exceeding 5 years. The areas of interest for ultrasound examination included the proximal phalanx of the third finger, the second intermetacarpal space, and the extension surface of the lower third of the forearm.

Clinical Manifestations in Vacuoles, E1 Enzyme, X-Linked, Autoinflammatory, Somatic (VEXAS) Syndrome: A Narrative Review.

The newly identified refractory adult-onset autoinflammatory syndrome known as VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is brought on by somatic mutations in the ubiquitin-like modifier-activating enzyme 1 (UBA1) gene in hematopoietic stem and progenitor cells that change the expression of the UBA1 isoform. As a result, patients have a variety of hematologic and systemic inflammatory symptoms. All types of medical professionals should treat VEXAS syndrome seriously due to the high fatality rate.

Microbiota-Accessible Boron-Containing Compounds in Complex Regional Pain Syndrome.

The microbiota-gut-brain axis has garnered increasing attention in recent years for its role in various health conditions, including neuroinflammatory disorders like complex regional pain syndrome (CRPS). CRPS is a debilitating condition characterized by chronic neuropathic pain, and its etiology and pathophysiology remain elusive. Emerging research suggests that alterations in the gut microbiota composition and function could play a significant role in CRPS development and progression.

Polymyalgia rheumatica: An update (Review).

Polymyalgia rheumatica (PMR) is a chronic inflammatory disease which affects the connective vascular tissue, characterized by pain accompanied by morning stiffness, predominantly of the neck muscles, hip and shoulder girdle. Usually, patients with this disease are >50 years of age and biological inflammatory syndrome is present with an increase in both the erythrocyte sedimentation rate and C-reactive protein levels, aspects similar to giant cell arteritis. The aim of the present review was to depict the current pathogenic hypothesis, diagnostic and treatment approach for patients with PMR, and novelties since the development of the currently used 2012 European League Against Rheumatism and American College of Rheumatology provisional classification criteria.

Dickkopf-Related Protein 1 (DKK-1) as a Possible Link between Bone Erosions and Increased Carotid Intima-Media Thickness in Psoriatic Arthritis: An Ultrasound Study.

Psoriatic arthritis (PsA) is a heterogenous systemic inflammatory disorder that affects peripheral joints and skin, but also causes inflammation at entheseal sites, digits (dactylitis) and the axial skeleton. Despite considerable advances, our understanding of the pathogenesis and management of PsA is hampered by its complex clinical expression. We enrolled patients who met the ClASsification for Psoriatic Arthritis (CASPAR) criteria for PsA ( = 17), and healthy controls ( = 13).

Outcomes of Prospectively Followed Pregnancies in Rheumatoid Arthritis: A Multicenter Study from Romania.

Women with rheumatoid arthritis (RA) may carry an increased risk of adverse pregnancy outcomes (APO). The aims of this study were to compare pregnancy outcomes in RA patients as compared to the general obstetric population (GOP) and to identify a risk profile in RA. A case-control study was conducted on 82 prospectively followed pregnancies in RA and 299 pregnancies from the GOP.

Novel Biomarkers Predictive of Diabetic Charcot Foot-An Overview of the Literature.

: Although Charcot diabetic foot (CDF) is a frequent complication of diabetic neuropathy, less is known about the possibility of its early prevention. : A review of the original articles published in English, using the "biomarkers AND Charcot's foot" criterion, resulted in 33 articles from the PubMed database and seven articles from the Web of Science database. The five duplicates were eliminated, and two independent reviewers selected the most relevant articles, leaving a total of 21 articles.

Computer-Aided Diagnosis of Pulmonary Nodules in Rheumatoid Arthritis.

(1) Background: Rheumatoid arthritis (RA) is considered a systemic inflammatory pathology characterized by symmetric polyarthritis associated with extra-articular manifestations, such as lung disease. The purpose of the present study is to use CAD in the detection of rheumatoid pulmonary nodules. In addition, we aim to identify the characteristics and associations between clinical, laboratory and imaging data in patients with rheumatoid arthritis and lung nodules.

Colchicine versus Physical Therapy in Knee Osteoarthritis.

Background: The treatment of osteoarthritis remains a major challenge due to the unavailability of a disease-modifying medication and the limitations of current therapeutic perspectives, which mainly target the symptoms, not the disease itself. The purpose of our study is to compare the efficacy of colchicine treatment versus physical therapy. Methods: The study included 62 patients diagnosed with knee osteoarthritis (KOA) according to the American College of Rheumatology (ACR) criteria, hospitalized within the time frame of October 2020−March 2022 in the Department of Rehabilitation Medicine and Physical Therapy of the Emergency Clinical County Hospital of Craiova.

The Outcome of Surgical Treatment for the Neuropathic Diabetic Foot Lesions-A Single-Center Study.

The prevalence of diabetic foot complications is continuously increasing as diabetes has become one of the most important "epidemics" of our time. The main objective of this study was to describe the appropriate surgical intervention for the complicated neuropathic diabetic foot; the secondary goal was to find the risk factors associated with minor/major amputation and good or adverse surgical outcomes. This is an observational, retrospective study conducted between 1 January 2018 and 31 December 2019, which included 251 patients from the General Surgery Department at the Dr I.

Anti-MDA5 Amyopathic Dermatomyositis-A Diagnostic and Therapeutic Challenge.

Clinically amyopathic Dermatomyositis (CADM) is a rare subtype of idiopathic inflammatory myositis, associated with no muscular manifestations, which is more frequent in Asian women. Anti-melanoma differentiation-associated gene 5 (MDA5) antibodies are a recently discovered type of specific autoantibodies associated with myositis. The anti-MDA5 DM was initially described in Japan and later it was discovered that the target antigen was a protein implicated in the innate immune response against viruses, that is encoded by the melanoma differentiation-associated gene 5.

Novel Biomarkers, Diagnostic and Therapeutic Approach in Rheumatoid Arthritis Interstitial Lung Disease-A Narrative Review.

Rheumatoid arthritis (RA) is considered a systemic inflammatory disease marked by polyarthritis which affects the joints symmetrically, leading to progressive damage of the bone structure and eventually joint deformity. Lung involvement is the most prevalent extra-articular feature of RA, affecting 10-60% of patients with this disease. In this review, we aim to discuss the patterns of RA interstitial lung disease (ILD), the molecular mechanisms involved in the pathogenesis of ILD in RA, and also the therapeutic challenges in this particular extra-articular manifestation.

Interobserver Reliability of Magnetic Resonance Imaging of Sacroiliac Joints in Axial Spondyloarthritis.

Introduction: Axial spondyloarthritis (axSpA) is characterized by damage to the axial skeleton and entheses, and is often associated with extra-articular manifestations, in the presence of the human leukocyte antigen (HLA) B27. The aim of our study is to assess the performance of rheumatologists in interpreting the inflammatory and structural damage to sacroiliac joints, in comparison to radiologists.

Material And Methods: The present study included a total of 34 patients diagnosed with axSpA, according to the Assessment of SpondyloArthritis International Society (ASAS) criteria for axSpA, examined from January 2021 to November 2021 in the Departments of Rheumatology and Radiology and Medical Imaging of the University of Medicine and Pharmacy of Craiova.

COVID-19-A Trigger Factor for Severe Immune-Mediated Thrombocytopenia in Active Rheumatoid Arthritis.

Thrombocytopenia is defined as a platelet count below 150,000/mm for adults. There is still controversy about whether individuals with platelet counts of 100,000/mm to 150,000/mm should be classified as having genuine thrombocytopenia or borderline thrombocytopenia. Thrombocytopenia is considered mild when the platelet count is between 70,000 and 150,000/mm and severe if the count is less than 20,000/mm.

A Study on Dermatomyositis and the Relation to Malignancy.

The idiopathic inflammatory myopathies (IIM) are a group of heterogeneous systemic diseases which include as main subtypes: polymyositis (PM), dermatomyositis (DM) and inclusion body myositis (IBM). The key feature of IIMs is the muscle weakness, accompanied by a characteristic skin rash in DM patients. The overall risk for malignancy in IIM is higher compared to the age-and sex-matched general population.

Non-alcoholic fatty liver disease and hematologic manifestations (Review).

Non-alcoholic fatty liver disease (NAFLD) is a multisystem disease, and it is associated with numerous extra-hepatic manifestations or additional co-occurring diseases. The aim of the present review was the identification and management of the hematologic manifestations of NAFLD. One of the triggers is considered to be iron abnormalities.

Rheumatic Immune-Related Adverse Events-A Consequence of Immune Checkpoint Inhibitor Therapy.

The advent of immunotherapy has changed the management and therapeutic methods for a variety of malignant tumors in the last decade. Unlike traditional cytotoxic chemotherapy, which works by interfering with cancer cell growth via various pathways and stages of the cell cycle, cancer immunotherapy uses the immune system to reduce malignant cells' ability to escape the immune system and combat cell proliferation. The widespread use of immune checkpoint inhibitors (ICIs) over the past 10 years has presented valuable information on the profiles of toxic adverse effects.

The Role of Clinical and Ultrasound Enthesitis Scores in Ankylosing Spondylitis.

Introduction: Ankylosing spondylitis (AS) is a chronic inflammatory disease, part of the spondyloarthritis (SpA) group, characterized by axial (spine and sacroiliac joints), entheseal, and peripheral joint involvement, which is frequently associated with extra-articular manifestations.

Material And Methods: The study included a number of 30 patients diagnosed with AS according to the New York modified criteria, with history of entheseal pain, hospitalized between 2016-2018 in the Department of Rheumatology of the Emergency County Hospital of Craiova.

Results: Regarding the Belgrade Ultrasound Enthesitis Score (BUSES) score and the disease activity calculated using the Ankylosing Spondylitis Disease Activity Score (ASDAS), they did not show a statistically significant association ( = 0.

Complex regional pain syndrome as a paraneoplastic disorder associated with metatypical basal cell carcinoma.

Metatypical basal cell carcinoma (MTBCC) is a rare form of tumor, which associates the clinical and histopathological (HP) characteristics of both basal cell carcinoma (BCC) and squamous cell carcinoma (SCC), with a 5% chance for the development of metastases. The reference diagnosis remains the HP confirmation from the lesional tissue. The current report illustrates the case of a 74-year-old patient, diagnosed with MTBCC consequently to the biopsy from the clinically malignant lesion with HP and immunohistochemical examination, currently in clinical remission following surgical treatment.