Cranial and extracranial manifestations of giant cell arteritis: a single-center observational study.

INTRODUCTION: Giant cell arteritis (GCA) presents two major phenotypes - cranial (cGCA) and extracranial (exGCA). exGCA may be overlooked. The study aimed to compare the clinical characteristics between cGCA and exGCA. METHODS: Electronic medical records of patients treated between January 2015 and July 2023 at the Department of Rheumatology were searched for the diagnosis of GCA. The clinical characteristics of patients with cGCA, exGCA, and overlapping GCA manifestations were compared. RESULTS: Out of 32 patients with GCA, 20 had cGCA, 7 had exGCA, and 5 had overlap manifestations. The groups did not differ significantly in demographics, clinical signs/symptoms, or laboratory test results. Importantly, the combined group of patients with exGCA and overlap GCA had a statistically significant delay in initiating treatment (median 12 weeks) compared to patients with cGCA (median 4 weeks; p = 0.008). CONCLUSION: Our study confirmed the insidious nature of exGCA, which lacks distinctive clinical symptoms and consequently leads to delayed treatment.

Color fraction as a useful method of imaging synovium vascularization in patients with high activity of rheumatoid arthritis.

Rheumatoid arthritis (RA) is an autoimmune disease with joint inflammation and destruction as the main features that appears with prevalence of 1 to 2% of the general population. Women are three times more likely to suffer from RA than men. Rheumatoid arthritis occurs at any age but commonly over 40-50 years old. In the course of RA each joint may be involved but most frequently the proximal interphalangeal and metacarpophalangeal joints of the hands, wrists, and also small joints of the feet are affected. Symmetrical joint swelling with overgrowth of synovium and hypervascularization confirmed in power Doppler ultrasound imaging are very characteristic for RA. Quantification of vascularization with the color fraction index may be a useful tool to monitor disease activity and in evaluation of inflammation in scientific research. This article aims to present this imaging diagnostic method based on the literature.

Diagnostic and therapeutic difficulties in a patient with chronic recurrent multifocal osteomyelitis coexisting with ulcerative colitis.

Chronic non-bacterial osteomyelitis (CNO) is a rare autoinflammatory bone disease, affecting mainly children. CNO includes a broad clinical spectrum of symptoms and signs, from mild, limited in time, unifocal osteitis to severe, chronic, active or recurrent, multifocal osteomyelitis. In 2014 diagnostic criteria for CNO were proposed, the Bristol Criteria for the Diagnosis of Chronic Non-bacterial Osteitis, taking into account the clinical picture - location and number of inflammatory foci, characteristic changes on radiological examination (X-ray) and magnetic resonance imaging (MRI), C-reactive protein (CRP) concentration, and changes in bone biopsy. The paper presents the case of a four-year-old boy in whom the diagnosis of multifocal osteomyelitis coexisting with ulcerative colitis was established. Attention was paid to the long diagnostic process of the disease, requiring in the first place differentiation with proliferative diseases. The choice of drugs was also a significant problem in the patient described in view of both intolerance of individual preparations and their ineffectiveness.

Selected issues in diagnostic imaging of spondyloarthritides: psoriatic arthritis and juvenile spondyloarthritis.

Spondyloarthritides (also known as spondyloarthropathies) are a group of rheumatic diseases that consists of diversified entities, i.e. ankylosing spondylitis, reactive arthritis, psoriatic arthritis, arthritis in the course of Crohn's disease and ulcerative colitis, and juvenile spondyloarthropathies. In the diagnostics of spondyloarthritides, plain radiography has played a crucial role for years due to its undisputed ability to show distinctive bony changes. Yet as those diseases often manifest themselves by soft tissue pathology and bone marrow inflammation, ultrasonography and magnetic resonance imaging are currently a subject of numerous studies in the quest for setting up diagnostic criteria, especially at early stages of inflammatory processes. In our review, we present an up-to-date insight into classifications, etiopathogenesis and imaging of psoriatic arthritis and juvenile spondyloarthritis.

Combination Treatment of Locoregionally Aggressive Granulomatosis with Polyangiitis and Cranial Base Infiltration.

OBJECTIVES: To present a personalized approach in three cases of treatment-resistant, locoregionally aggressive forms of cANCA-positive granulomatosis with polyangiitis (GPA) and skull base involvement. METHODS: Three patients with GPA and skull base involvement were described alongside a critical review of the current literature. RESULTS: All presented patients suffered from GPA with an inflammatory tumor at the skull base, alongside cerebellopontine angle involvement, cranial nerve palsies, cerebellar disorders, concomitant hearing loss, and severe otalgia. Symptoms were associated with progressive granulomatous destruction of the temporal bone, laryngopharynx, and central nervous system infiltration. Treatment with cyclophosphamide and high doses of glucocorticoid steroids were ineffective but subsequent therapy with rituximab was successful in the presented cases. The literature review showed that the course of the disease with skull base involvement is associated with poorer clinical and radiological responses to standard pharmacotherapies. CONCLUSION: Granulomatous inflammation localized in the skull base is associated with a more aggressive disease progression and is less likely to respond to pharmacotherapy. Standard induction therapy with cyclophosphamide and glucocorticoid steroids may be ineffective. A better response may be achieved by using rituximab and concomitant local treatment with glucocorticoid steroid injections.

Diagnostic agreement between radiofrequency echographic multispectrometry and dual-energy X-ray absorptiometry in the assessment of osteoporosis in a Polish group of patients.

INTRODUCTION: Osteoporosis is still underdiagnosed in Poland, partly due to limited accessibility to the gold-standard diagnostic technique, that is, dual-energy X-ray absorptiometry (DXA) of the proximal femur and lumbar spine. The use of radiofrequency echographic multispectrometry (REMS) as an alternative diagnostic tool might be of particular interest because this technique is nonionizing, the devices are portable, and their utilization relatively cheap. OBJECTIVES: The aim of this study was to assess the agreement between a novel quantitative technique (REMS) and DXA in the evaluation of bone mineral density and diagnosis of osteoporosis. PATIENTS AND METHODS: All recruited patients (n = 116) underwent DXA and REMS of the proximal femur and lumbar spine. The diagnostic agreement of REMS was assessed through a direct comparison with DXA results, with separate analysis for the proximal femur and lumbar spine scans. Additional sub-analysis of the impact of sex, age, and BMI was performed. RESULTS: After the exclusion of patients due to significant skeletal impairments, missing results, and erroneous reports, 66 scans of the femur and 58 scans of the lumbar spine were analyzed. The diagnostic agreement between the results of DXA and REMS was 82.8% in the lumbar spine group and 84.8% in the femur group. Strong correlations between REMS and DXA results were found in both groups, regardless of the sex, age, and BMI. CONCLUSION: Radiofrequency echographic multispectrometry showed a significant diagnostic agreement with the corresponding DXA measurements. The study further confirms the usefulness of REMS in the assessment of osteoporosis.

Role of ultrasonography in the diagnosis of rheumatic diseases in light of ACR/EULAR guidelines.

In the past years, ultrasound imaging has become an integral element of the diagnostic process in rheumatic diseases. It enables the identification of a range of inflammatory changes in joint cavities, sheaths and bursae, and allows their activity to be assessed. In 2012, experts of the European Society of Musculoskeletal Radiology prepared recommendations concerning the role of ultrasonography in the diagnosis of musculoskeletal diseases. Ultrasound was considered the method of choice in imaging peripheral synovitis. Moreover, ultrasound imaging has been popularized thanks to the new classification criteria for rheumatoid arthritis issued by the American College of Rheumatology and European League Against Rheumatism in 2010. They underline the role of ultrasound imaging in the detection of articular inflammatory changes that are difficult to assess unambiguously in the clinical examination. These criteria have become the basis for recommendations prepared by experts from the European League Against Rheumatism concerning medical imaging in rheumatoid arthritis. Nine of ten recommendations concern ultrasonography which is relevant in detecting diseases, predicting their progression and treatment response, monitoring disease activity and identifying remission. In the new criteria concerning polymyalgia rheumatica from 2012, an ultrasound scan of the shoulder and pelvic girdle was considered an alternative to clinical assessment. Moreover, the relevance of ultrasonography in the diagnosis and monitoring of peripheral spondyloarthropathies was widely discussed in 2014 during the meeting of the European League Against Rheumatism in Paris.

Imaging of juvenile idiopathic arthritis. Part I: Clinical classifications and radiographs.

Juvenile idiopathic arthritis is the most common autoimmune systemic disease of the connective tissue affecting individuals at the developmental age. Radiography is the primary modality employed in the diagnostic imaging in order to identify changes typical of this disease entity and rule out other bone-related pathologies, such as neoplasms, posttraumatic changes, developmental defects and other forms of arthritis. The standard procedure involves the performance of comparative joint radiographs in two planes. Radiographic changes in juvenile idiopathic arthritis are detected in later stages of the disease. Bone structures are assessed in the first place. Radiographs can also indirectly indicate the presence of soft tissue inflammation (i.e. in joint cavities, sheaths and bursae) based on swelling and increased density of the soft tissue as well as dislocation of fat folds. Signs of articular cartilage defects are also seen in radiographs indirectly - based on joint space width changes. The first part of the publication presents the classification of juvenile idiopathic arthritis and discusses its radiographic images. The authors list the affected joints as well as explain the spectrum and specificity of radiographic signs resulting from inflammatory changes overlapping with those caused by the maturation of the skeletal system. Moreover, certain dilemmas associated with the monitoring of the disease are reviewed. The second part of the publication will explain issues associated with ultrasonography and magnetic resonance imaging, which are more and more commonly applied in juvenile idiopathic arthritis for early detection of pathological features as well as the disease complications.

Charcot arthropathy in ultrasound examination - a case report.

This article presents a patient with a long history of type 1 diabetes mellitus complicated with neuropathy and Charcot disease. The most common cause of neuropathic osteoarthropathy, called Charcot osteoarthropathy, is poorly controlled diabetes. The clinical picture is characterized by considerable edema, redness and increased skin temperature with relatively slight pain due to injury to nerve fibers responsible for pain sensation. The differential diagnosis should include bacterial or autoimmune arthritis, arthritis associated with gout as well as venous thrombosis and injury. The contribution of a local inflammatory reaction and abnormal bone turnover with excessive osteoclast activity might play a role in the etiopathogenesis of this disease. As a result, osseous and articular destruction progresses rapidly leading to irreversible deformity of the foot. Avoiding weight-bearing and resting the foot in a specially selected plaster cast is the most important part of treatment. Patients with the aforementioned complaints are referred to radiologists for imaging examinations. An ultrasonographer should pay attention to changes typical of Charcot arthropathy, such as: inflammatory and destructive changes in joints of the foot, uneven contour of bones with thickening and periosteal hyperemia as well as soft tissue swelling.

Imaging of juvenile idiopathic arthritis. Part II: Ultrasonography and MRI.

Juvenile idiopathic arthritis is the most common autoimmune systemic disease of the connective tissue affecting individuals in the developmental age. Radiography, which was described in the first part of this publication, is the standard modality in the assessment of this condition. Ultrasound and magnetic resonance imaging enable early detection of the disease which affects soft tissues, as well as bones. Ultrasound assessment involves: joint cavities, tendon sheaths and bursae for the presence of synovitis, intraand extraarticular fat tissue to visualize signs of inflammation, hyaline cartilage, cartilaginous epiphysis and subchondral bone to detect cysts and erosions, and ligaments, tendons and their entheses for signs of enthesopathies and tendinopathies. Magnetic resonance imaging is indicated in children with juvenile idiopathic arthritis for assessment of inflammation in peripheral joints, tendon sheaths and bursae, bone marrow involvement and identification of inflammatory lesions in whole-body MRI, particularly when the clinical picture is unclear. Also, MRI of the spine and spinal cord is used in order to diagnose synovial joint inflammation, bone marrow edema and spondylodiscitis as well as to assess their activity, location, and complications (spinal canal stenosis, subluxation, e.g. in the atlantoaxial region). This article discusses typical pathological changes seen on ultrasound and magnetic resonance imaging. The role of these two methods for disease monitoring, its identification in the pre-clinical stage and establishing its remission are also highlighted.

The value of ultrasound in the diagnosis of limited scleroderma - a case report.

Systemic sclerosis, popularly referred to as scleroderma, is a chronic connective tissue disease with present autoantibodies against platelet-derived growth factor receptor. These antibodies activate directly fibroblasts causing the dermis and internal organs' fibrosis and vascular damage. Additionally, calcific collections, including hydroxyapatite crystals, may develop in subcutaneous tissue and juxta-articular soft tissue. Herein, we report a case of a 72-year-old woman, referred by a rheumatologist for plain radiography and ultrasound examination of hands due to pain and swelling of the fourth finger of the left hand. Dermal induration affecting hands, especially fourth finger on the left side and the Raynaud phenomenon were observed on physical examination. Furthermore, the patient had noticed periodic discharge with a toothpaste consistency from a tiny fistula localised in the vicinity of the fourth finger alterations. The paper emphasises a possible application of the twinkling artefact and MicroPure option on ultrasound examination in differential diagnosis of soft tissue calcifications. Making the correct diagnosis can attribute to precise planning of surgical treatment.

Radial sesamoid fracture of the second MCP joint - a case report.

A fracture of the sesamoid bone of the hand is rarely seen. In most cases, it is strictly associated with the trauma. The patient complains of a long-lasting, due to unclear diagnosis, painful swelling of the joint with an inability to flex the affected finger. Limited blood supply makes the untreated fracture prone to avascular necrosis. Thus, it is extremely important to make a proper diagnosis early. Bilateral comparing the affected areas with ultrasonography seems to be the method of choice. The treatment is conservative or surgical when needed. We present a case of a radial sesamoid fracture of the second metacarpophalangeal joint without a history of trauma.