Primary hypertrophic osteoarthropathy - a rare cause of pain and arthritis in children. Description of 5 cases.

Primary hypertrophic osteoarthropathy (PHOA) is a very rare disease. The typical triad of symptoms, i.e. digital clubbing, periosteal bone formation with bone and joint deformities and skin hypertrophy, may be accompanied by other specific conditions. In the majority of patients, the picture of the disease is incomplete. The dominant clinical symptom may be osteoarticular complaints. Moreover, the final confirmation of the diagnosis of the primary form of hypertrophic osteoarthropathy requires the analysis of much more frequent secondary causes of the disease. Diagnosing primary osteoarthropathy in children is particularly difficult. Some children report joint pain before the onset of the other symptoms of osteoarthropathy, while the physical and imaging examinations show features of arthritis. This can lead to misdiagnoses including the diagnosis of juvenile idiopathic arthritis (JIA) and the unnecessary use of immunosuppressive treatment. The present description of five patients from the Paediatric Rheumatology Department indicates diagnostic difficulties in children with PHOA. All of them were examined due to pain and features of arthritis. We observed an incomplete clinical picture of the disease. One patient required a revision of the previous diagnosis of JIA and discontinuation of ineffective treatment with disease-modifying antirheumatic drugs (DMARDs). PHOA should always be considered in the differential diagnosis of arthritis in children, due to the slow and often atypical development of symptoms, including the presence of pain and arthritis as the predominant symptom of the disease.

Conventional Radiography and Ultrasound Imaging of Rheumatic Diseases Affecting the Pediatric Population.

Juvenile idiopathic arthritis is the most frequent rheumatic disease in the pediatric population, followed by systemic lupus erythematosus, juvenile scleroderma syndromes, juvenile dermatomyositis, chronic recurrent multifocal osteomyelitis, and juvenile vasculopathies. The imaging approach to inflammatory connective tissue diseases in childhood has not changed dramatically over the last decade, with radiographs still the leading method for bony pathology assessment, disease monitoring, and evaluation of growth disturbances. Ultrasonography is commonly used for early detection of alterations within the intra- and periarticular soft tissues, assessing their advancement and also disease monitoring. It offers several advantages in young patients including nonionizing radiation exposure, short examination time, and high resolution, allowing a detailed evaluation of the musculoskeletal system for the features of arthritis, tenosynovitis, enthesitis, bursitis, myositis, as well as pathologies of the skin, subdermis, vessels, and fasciae. In this pictorial essay we discuss radiographic and ultrasound inflammatory features of autoimmune pediatric inflammatory arthropathies: juvenile idiopathic arthritis, lupus erythematosus, juvenile scleroderma, juvenile dermatomyositis and polymyositis.

Juvenile systemic sclerosis - observations of one clinical centre.

OBJECTIVES: The systemic form of scleroderma (SSc) in children is a very rare disease; therefore, it is recognized relatively late, which increases the risk of complications. The aim of the study was to assess the clinical symptoms of juvenile systemic sclerosis (JSSc) in our cohort patients. MATERIAL AND METHODS: A group of (N = 22) scleroderma patients aged between 2 and 16 years were observed. Demographic data and all clinical results obtained during 16 years of observation in the clinic of rheumatic diseases of developmental age were collected and analysed. RESULTS: In all observed children the major JSSc criterion was found, i.e. skin thickening proximal to the metacarpal phalangeal and/or metatarsophalangeal joints. Other symptoms are presented as follows: nailfold capillary abnormalities - 100%, Raynaud's phenomenon - 90.9%, sclerodactyly - 27.3%, digital tip ulcers - 27.3%, dysphagia - 18.2%, gastroesophageal reflux - 27.3% (assessed in only 10 children), arrhythmias - 22.7%, heart failure - 9.1%, new-onset arterial hypertension - 9.1%, pulmonary fibrosis - 72.7%, pulmonary arterial hypertension - 9.1%, neuropathy - 13.6%, carpal tunnel syndrome - 4.5%, tendon friction rubs - 4.5%, arthritis - 22.7%, and myositis - 13.6%. There were no cases of renal crisis. Decreased diffusing capacity of oxygen was confirmed in 12 patients (58.3%). The presence of antinuclear antibodies was noticed in 86.7% of patients, and among SSc selective autoantibodies: anticentromere - 31.8%, anti-topoisomerase I - 18.2%, anti-PM-Scl 100 or 75 - 45.5%, anti-RP11, Th/To, PCNA in total in 27.3% were presented. In 4.5% of cases, apart from the presence of anti-PM-Scl autoantibodies, positive lupus band test, reduced concentration of complement, and antiphospholipid antibodies were also found. In 59% of studied children, the body mass index was below the 25th percentile. CONCLUSIONS: The presented retrospective analysis shows that the occurrence of Raynaud's phenomenon with changes in nailfold capillaroscopy is the best screening toll for the assessment of risk of JSSc. All patients of developmental age with Raynaud's phenomenon, especially in the case of the appearance of antinuclear antibodies, should be monitored with capillaroscopy regardless of other laboratory or imaging tests.

The use of radiosynovectomy in patients with juvenile idiopathic arthritis. Assessment of treatment efficacy and safety.

OBJECTIVES: The aim of the study was to evaluate the usefulness of knee joint radiosynovectomy (RS) in patients suffering from juvenile idiopathic arthritis (JIA). MATERIAL AND METHODS: One hundred RS procedures performed in 58 patients with JIA in average age 10.4 years were evaluated. RESULTS: After 6 weeks, a decrease in the number of cases with joint pain from 90.3% to 29%, with joint oedema from 100% to 74.5%, with joint exudate from 100% to 60.6%, with gait disorders from 19.4% to 3.2%, with joint mobility disorders from 51.1% to 26.6% in the RS cases was observed. A reduction of the score in the Colorado scale from 10.9 to 4.66, in the pain visual analogue scale (VAS) from 50 to 10, in the illness VAS assessed by the patient/parent from 69.9 to 32.4, in the illness VAS assessed by the physician from 68.8 to 36.9 was observed. Six months after the RS procedure, a reduction in the number of cases with joint pain from 89.5% prior to the procedure to 29.5%, with oedema from 100% to 58.3%, with exudate from 100% to 46.9%, with gait disorders from 20% to 2.1%, with joint motility disorders from 51.1% to 26.1% was achieved. The score in the Colorado scale was reduced from 10.9 to 4.04, in the pain VAS from 40 to 0, in the illness VAS assessed by the patient/parent from 69.7 to 27.9, in the illness VAS assessed by the physician from 68.8 to 32.4. In ultrasound examinations, the greatest improvement compared to the initial condition was recorded in the 6th month after the RS. Radiosynovectomy was positively evaluated by parents and patients in 34 anonymous surveys. Early and late observations (average 1473 days) did not show lesions at the isotope injection site, and no neoplastic lesions were observed. CONCLUSIONS: Radiosynovectomy is a valuable therapeutic option for local treatment in patients with JIA.

Imaging of cervical spine involvement in inflammatory arthropathies: a review.

The cervical spine can be affected by many types of inflammatory arthropathies, and the most common autoimmune diseases with cervical spine involvement are rheumatoid arthritis (RA), spondyloarthritis (SpA), and juvenile idiopathic arthritis (JIA). The clinical symptoms of cervical spine pathologies are often nonspecific or absent; therefore, imaging plays a crucial diagnostic role. RA is the most prevalent autoimmune disease; it often leads to cervical spine instability and subsequent myelopathy. In SpA, due to new bone formation, the characteristic lesions include syndesmophytes, parasyndesmophytes, and bone ankylosis, but instabilities are rare. In JIA, early apophyseal bone ankylosis is characteristic, in addition to impaired spinal growth. The aim of this review article is to discuss the imaging pathologies found in patients with RA, SpA, and JIA in the early and advanced stages. This knowledge would be helpful in the proper diagnosis and treatment of these diseases.

Uveitis associated juvenile idiopathic arthritis - presentation of recently published international recommendations for diagnostic and therapeutic procedures.

Uveitis is one of the most common extra-articular manifestations in juvenile idiopathic arthritis (JIA) and occurs in 20-30% of children with this disease. Uveitis occurs at various frequencies and varies in character in individual clinical subtypes of the disease. Normally it is asymptomatic or mildly symptomatic; thus, in 85% of the patients the first symptoms of uveitis and its subsequent episodes pass unobserved. In 75% of cases inflammation affects both eyes. Most often it occurs in the oligoarticular subtypes of JIA, especially in patients aged 2-4 years, who are diagnosed with anti-nuclear antibodies in serum. Uncontrolled uveitis may lead to severe complications that may result in vision loss. To date, there are no generally accepted diagnostic and therapeutic standards for this disease. This article presents the latest recommendations by ophthalmologists and rheumatologists for the detection, treatment, and monitoring of children with JIA-associated uveitis.

Magnetic resonance imaging of the knee joint in juvenile idiopathic arthritis.

Juvenile idiopathic arthritis (JIA) is an umbrella term for a group of diseases in children younger than 16 years old lasting six weeks or longer. Synovitis may lead to destructive and irreversible joint changes with subsequent functional impairment. Prompt diagnosis is essential to prevent permanent joint damage and preserve joint functionality. In the course of JIA both the axial and peripheral skeleton may be involved in the inflammatory process, but the knee joint is most frequently affected. New drugs and treatment protocols have forced the need for diagnosis at the earliest possible stage. Magnetic resonance imaging (MRI) allows early detection of lesions and constitutes a superior diagnostic imaging method. Synovitis, tenosynovitis, enthesitis, bursitis, osteitis, cartilage loss, bone cysts, and erosions are lesions diagnosed in JIA, and they can be precisely imaged in MRI. This article aims to present MRI inflammatory features of the knee in children with JIA based on the literature.

Assessment of anti-HBs antibody concentration in children with juvenile idiopathic arthritis treated with biological drugs, vaccinated against viral type B hepatitis in infancy.

OBJECTIVES: The introduction of vaccinations against viral hepatitis B in the years 1994-1996 in Poland significantly improved the epidemiological situation of hepatitis B virus (HBV) infections in our country. According to the report of the National Institute of Public Health - National Institute of Hygiene in 2018, 40 cases of acute hepatitis B were noted while still in the 1980s between 10 and 20 thousand new cases were reported annually. The aim of the study was to determine whether in children treated with biological drugs (adalimumab, etanercept, infliximab) due to juvenile idiopathic arthritis (JIA), vaccinated against hepatitis B in infancy, a protective concentration of anti-HBs antibodies persists. In patients, the value ≥ 10 mIU/ml is regarded as a protective concentration of antibodies, determined at least four weeks after administration of the last vaccine dose. Among healthy individuals, presence of anti-HBs antibodies in any concentration means seroprotection. No booster vaccinations are recommended in basically vaccinated healthy individuals. MATERIAL AND METHODS: The concentrations of anti-HBs antibodies were determined in 56 children with JIA (38 girls - 67.9% and 18 boys - 32.1%) aged from 2 years and 4 months to 17.5 years, treated for at least three months with biological drugs. The diagnosis of JIA was made based on the International League of Associations for Rheumatology (ILAR) criteria. All studied patients were at the stable stage of the disease and received a full course of hepatitis B vaccination during infancy (in accordance with 0,1,6 months injection scheme). RESULTS: In the studied children a protective anti-HBs antibody concentration was found in 34 cases (60.7%), and 22 children (39.3%) had anti-HBs antibody concentration < 10 mIU/ml (in these children no seroprotection was found). CONCLUSIONS: The post-vaccination antibody concentration should be determined in children with JIA, treated with biological drugs and, in case of absence of a protective concentration, revaccination should be started.

Clinical features and outcome of mixed connective tissue disease in developmental age - observational study from one center.

OBJECTIVES: Mixed connective tissue disease is a rare systemic connective tissue disease of developmental age and it includes the features of arthritis, polymyositis/dermatomyositis, systemic lupus erythematosus and systemic sclerosis, with presence of anti-ribonucleoprotein antibodies (anti-RNP) in serum. Early diagnosis of the disease is difficult but essential in preventing development of systemic complications, which are often irreversible. International literature does not report many studies on large cohorts of children with this disease. The aim of this retrospective study was to define clinical characteristics and long-term results of treatment of the disease in 60 children with mixed connective tissue disease hospitalized in the period between 1978 and 2018. The diagnosis was established on the basis of Kasukawa's criteria. MATERIAL AND METHODS: It was a group of 60 children (46 girls and 16 boys) aged 10.5 on average (4-16.5). When assessing general symptoms at the onset of the disease according to Kasukawa's criteria, the highest number, over 80% of children, demonstrated symptoms suggesting SLE, about 40% suggesting DM and about 25% suggesting SSC. In the period of observation the number of children with clinical symptoms suggesting SSC increased. The most common clinical symptoms included Raynaud syndrome, arthritis and myositis and the most common irregularities in the test results included presence of anti-RNP antibodies and rheumatoid factor and hematological symptoms such as leukopenia/thrombocytopenia. Restrictive lung function impairment was demonstrated by 20% of children. Treatment most often included combined therapy (glucocorticosteroids + methotrexate/azathioprine). RESULTS: In 70% of the patients stable improvement was observed. Remission concerned 7% of the patients, frequent exacerbations were found in almost 20% of patients, and 2 children (3.5%) died. CONCLUSIONS: The long term observations of patients in developementeal age with mixed connective tissue disease revealed that the majority of them had domination of SLE symptoms, only in 7% achieved remission and 70% remained in stable improvement. Serious infections with septic state were the cause of death in two cases.

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.

Rheumatic fever - new diagnostic criteria.

Rheumatic fever (RF) is an autoimmune disease associated with group A ß-hemolytic streptococcal infection, in the course of which the patient develops carditis, arthritis, chorea, subcutaneous nodules and erythema marginatum. Rheumatic fever diagnosis is based on the Jones criteria, developed in 1944, then revised twice by the American Heart Association (AHA), in 1992 and recently in 2015. The last revision of the Jones criteria consists mainly in the supplementation of the major criteria with echocardiographic examination, the introduction of a concept of subclinical carditis and the isolation of low, medium and high risk populations among the patients. AHA recommends that all the patients with suspected RF undergo Doppler echocardiographic examination after the Jones criteria have been verified, even if no clinical signs of carditis are present.

Eosinophilic cystitis and cholangitis - systemic disease triggered by mycobacterium tuberculosis?

Eosinophilic cystitis (EC) is a rare inflammatory disorder of the urinary tract characterized by infiltration of bladder with eosinophils. The cause remains unclear, immunological mechanisms have been implicated in pathogenesis. Potential etiological factors include: tumors, allergy, parasitic infections, trauma. The disease may have a variable course, from a mild self-limiting, through common symptoms like: dysuria, hematuria, abdominal pain, tumor, to severe renal failure, with eosinophilic infiltration of the other organs and systemic complications. Treatment depending on disease severity and etiology is pharmacological and/or surgical. Here we report a case of a previously healthy 16-year old girl with inflammatory tumor in the liver hilum infiltrating extrahepatic biliary tract who developed three months later haematuria with acute dysuric signs and renal failure. Based on histopathological findings diagnosis of eosinophilic cystitis was established. Tests for Mycobacterium tuberculosis were positive. To our knowledge, EC association with cholangitis and tuberculosis have never been reported before.

Eosinophilic cystitis and cholangitis - systemic disease triggered by mycobacterium tuberculosis?

Eosinophilic cystitis (EC) is a rare inflammatory disorder of the urinary tract characterized by infiltration of bladder with eosinophils. The cause remains unclear, immunological mechanisms have been implicated in pathogenesis. Potential etiological factors include: tumors, allergy, parasitic infections, trauma. The disease may have a variable course, from a mild self-limiting, through common symptoms like: dysuria, hematuria, abdominal pain, tumor, to severe renal failure, with eosinophilic infiltration of the other organs and systemic complications. Treatment depending on disease severity and etiology is pharmacological and/or surgical. Here we report a case of a previously healthy 16-year old girl with inflammatory tumor in the liver hilum infiltrating extrahepatic biliary tract who developed three months later haematuria with acute dysuric signs and renal failure. Based on histopathological findings diagnosis of eosinophilic cystitis was established. Tests for Mycobacterium tuberculosis were positive. To our knowledge, EC association with cholangitis and tuberculosis have never been reported before.

Whole-Body MRI at Initial Presentation of Chronic Recurrent Multifocal Osteomyelitis, Juvenile Idiopathic Arthritis, Their Overlapping Syndrome, and Non-Specific Arthropathy.

(1) Background: Whole-body magnetic resonance imaging (WB-MRI) is central to defining total inflammatory burden in juveniles with arthritis. Our aim was to determine and compare the initial distribution of lesions in the WB-MRI in patients with chronic recurrent multifocal osteomyelitis (CRMO), juvenile idiopathic arthritis (JIA), their overlapping syndrome (OS), and with Non-specific Arthropathy (NA). (2) Methods: This retrospective single center study was performed on an Avanto 1.5-T MRI scanner with a dedicated multichannel surface coil system. A total of 173 pediatric patients were included with the following final diagnoses: CRMO (15.0%), JIA (29.5%), OS (4.6%), and NA (50.9%). (3) Results: Bone marrow edema (BME) was the most common abnormality, being seen in 100% patients with CRMO, 88% with OS, 55% with JIA, and 11% with NA. The bones of the lower extremities were the most affected in all compared entities. Effusion was seen in 62.5% children with OS, and in 52.9% with JIA, and in CRMO and NA, the exudate was sporadic. Enthesitis was found in 7.8% of patients with JIA and 3.8% with CRMO, and myositis was seen in 12.5% of patients with OS and in 3.9% with JIA. (4) Conclusions: The most frequent indication for WB-MRI in our center was JIA. The most common pathology in all rheumatic entities was BME, followed by effusion mainly seen in in OS and JIA. Enthesitis and myositis were less common; no case was observed in NA.

[Secondary hemophagocytic syndromes]. / Wtórne zespoly hemofagocytarne--aktualny problem interdyscyplinarny.

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening extreme whole body inflammatory state. It results from the pathological hyperactivation of the immune system, because of congenital or acquired abnormalities of cytotoxicity and NK or T cells. Uncontrolled stimulation of lymphocytes and macrophages lead to hypercytokinemia, organ infiltration by these cells and multiple organ failure. There are genetic HLH and secondary HLH, associated with infections, autoimmune disorders, malignancies. The frequency of the secondary form is difficult to estimate because of the wrong and difficult diagosis. The clinical course is often insidious and nonspecific. Symptoms are varied. The most important are: unremitting fever, hepatosplenomegaly. Generalized edema, rash, lymphadenopathy may occur. Liver failure, respiratory, circulatory and multiple organ failure could develop in a very short time. Most common abnormalities in additional tests are: cytopenias, hypofibrinogenaemia, hypertriglyceridaemia, hyperferritinaemia, hypertransaminasaemia, elevated parameters of inflammation (excepting lowering erythrocyte sedimentaion rate). Criteria for diagnosis and therapeutic protocols referto the genetic forms of HLH. Currently, there are no guidelines for secondary HLH. Diagnostic and therapeutic difficulties also arise from clinical picture, similar as in the systemic inflammatory response syndrome, sepsis, multiple organ dysfunction syndrome. We present the clinical presentation, diagnostic pitfalls and treatment of secondary HLH, based on a review of the current literature and our own observations.

Ultrasound and MRI of the foot in children and adolescents newly diagnosed with juvenile idiopathic arthritis.

Aim: To evaluate the spectrum of inflammatory features in foot joints which may be detected on routinely performed ultrasound (US) and magnetic resonance imaging (MRI) in children newly diagnosed with juvenile idiopathic arthritis (JIA). Material and methods: Two groups of children hospitalized in a reference center for rheumatology, newly diagnosed with JIA and suspected of foot involvement in the course of JIA were included in this retrospective study. In the first group of 47 patients aged 1-18 years, the imaging was restricted to US. The second group of 22 patients aged 5-18 years underwent only non-contrast MRI of the foot. Results: The most frequent pathologies seen on US included effusion and synovial thickening in the first metatarsophalangeal joint (MTP1), followed by the tibiotalar joint. Synovial hyperemia on color Doppler US images was present most frequently in the Chopart and midtarsal joints (64%; 7/11 cases), followed by the tibiotalar joint (45%; 5/11), and MTP2-5 joint synovitis (40%; 4/10). Grade 3 hyperemia was present only in four cases; grades 1 and 2 were detected in the majority of cases. On MRI, bone marrow edema was the most frequent pathology, found mostly in the calcaneus (45%; 10/22 cases), while alterations of the forefoot were rare. No cases of bursitis, enthesitis, cysts, erosions or ankylosis were diagnosed in either of the analyzed groups. Conclusions: Routine US of the foot is recommended for early detection of its involvement in JIA in daily clinical practice. Although MRI can identify features of various JIA stages, it is particularly useful for the detection of bone marrow alterations.

Ankle Magnetic Resonance Imaging in Juvenile Idiopathic Arthritis Versus Non-Juvenile Idiopathic Arthritis Patients with Arthralgia.

This retrospective case-control study aimed to evaluate whether Magnetic Resonance Imaging (MRI) enables differentiation of ankle arthritis in Juvenile Idiopathic Afrthritis JIA from ankle arthralgia of unknown aetiology in patients clinically suspected of JIA. Forty-four children, at ages 5-16, who underwent MRI of the ankle from January 2016 to March 2021 for clinically suspected active ankle arthritis in the course of JIA were included. MRI findings in both groups-patients with the final diagnosis of JIA and without final diagnosis of JIA-were compared and scored. The sum of the scores of 22 ankle lesions in an individual patient (active, destructive and developmental), so-called the MRI summarized score, was calculated and tested in terms of the most optimal diagnosis of JIA. Interobserver agreement was calculated. Inflammatory features were seen on MRI in 38 out of all the included patients (86%). The most common lesions in both groups were effusion in the tibio-talar joint (68% in JIA and 64% in the arthralgia group) and effusion in subtalar joint (64% in JIA vs. 59% in the arthralgia group). In general, more lesions were identified in the JIA group than in non-JIA. However, only tenosynovitis was significantly more common in the JIA vs. non-JIA group (p = 0.031). The MRI summarized score did not allow for discrimination between ankle arthritis in JIA from non-JIA patients; the best levels of sensitivity (32%), specificity (91%), positive predictive value PPV (78%) and negative predictive value NPV (57%) were achieved only at the cut-off point of 10.

Foot and Ankle Conventional Radiography in Juvenile Idiopathic Arthritis: Does It Still Matter?

Objectives: The aim of this study was to evaluate the residual value of Conventional Radiography in children with arthralgia clinically suspected of Juvenile Idiopathic Arthritis (JIA). Materials and Methods: Three hundred seventy-two patients aged 1-18 years suspected of JIA were retrospectively reviewed. All patients had foot and ankle plain films performed in standard two projections: ankle in antero-posterior and lateral, and foot in antero-posterior and oblique. The cohort was divided into two groups: patients with confirmed JIA and non-JIA control group of children with foot and ankle arthralgia without diagnosis of inflammatory connective tissue disease. Radiographic findings in both groups were compared. Results: In 40% of JIA and 70% of non-JIA patients radiographs were normal. All radiographic findings were significantly more common in JIA than in non-JIA group (p = 0.000). Soft tissue swelling was the most frequent abnormality found in JIA patients (31, 51%) and only in 2.41% of non-JIA patients (p = 0.000). Osteoporosis and joint space narrowing were also significantly more common in JIA group (p = 0.000). The majority of imaging findings in non-JIA group were non-inflammatory abnormalities. Conclusion: Conventional radiography is an important tool in differential diagnosis of arthralgia of unknown etiology, as soft tissue swelling, osteoporosis and joint space narrowing are significantly more common in JIA patients as compared with patients without the diagnosis of inflammatory connective tissue disease. However, in case of high clinical suspicion of JIA and normal radiography, we recommend subsequent ultrasound (US) and/or MRI to allow early treatment.

Update on Current Imaging of Systemic Lupus Erythematous in Adults and Juveniles.

Systemic lupus erythematosus (SLE) is an autoimmune disease involving multiple organs and organ systems. Musculoskeletal (MSK) involvement is one of the most frequent and the earliest locations of disease. This disease affects joints and periarticular soft tissues, tendon sheaths and tendons, bones, and muscles. Multimodality imaging, including radiography, ultrasound (US), and magnetic resonance imaging (MRI), plays a significant role in the initial evaluation and treatment follow up of MSK manifestations of the SLE. In this paper, we illustrate MSK imaging features in three clinical forms of SLE, including nondeforming nonerosive arthritis, deforming nonerosive arthropathy, and erosive arthropathy, as well as the other complications and features of SLE within the MSK system in adults and juveniles. Advances in imaging are included. Conventional radiography primarily shows late skeletal lesions, whereas the US and MRI are valuable in the diagnosis of the early inflammatory changes of the soft tissues and bone marrow, as well as late skeletal manifestations. In nondeforming nonerosive arthritis, US and MRI show effusions, synovial and/or tenosynovial hypertrophy, and vascularity, whereas radiographs are normal. Deforming arthritis clinically resembles that observed in rheumatoid arthritis, but it is reversible, and US and MRI show features of inflammation of periarticular soft tissues (capsule, ligaments, and tendons) without the pannus and destruction classically observed in RA. Erosions are rarely seen, and this form of disease is called rhupus syndrome.

Advances in Musculoskeletal Imaging in Juvenile Idiopathic Arthritis.

Over the past decade, imaging of inflammatory arthritis in juvenile arthropathies has significantly advanced due to technological improvements in the imaging modalities and elaboration of imaging recommendations and protocols through systematic international collaboration. This review presents the latest developments in ultrasound (US) and magnetic resonance imaging (MRI) of the peripheral and axial joints in juvenile idiopathic arthritis. In the field of US, the ultra-wideband and ultra-high-frequency transducers provide outstanding spatial resolution. The more sensitive Doppler options further improve the assessment and quantification of the vascularization of inflamed tissues, and shear wave elastography enables the diagnosis of tissue stiffness. Concerning MRI, substantial progress has been achieved due to technological improvements in combination with the development of semiquantitative scoring systems for the assessment of inflammation and the introduction of new definitions addressing the pediatric population. New solutions, such as superb microflow imaging, shear wave elastography, volume-interpolated breath-hold examination, and MRI-based synthetic computed tomography open new diagnostic possibilities and, at the same time, pose new challenges in terms of clinical applications and the interpretation of findings.