Joint Involvement Research Articles

A single-center rheumatology experience of sarcoidosis: observations from 70 patients.

The aim of this study is to determine the demographic, clinical and laboratory characteristics of the patients who followed up with the diagnosis of sarcoidosis, to investigate the distribution frequency of rheumatological findings and to examine the disease management from the perspective of rheumatology. Patients who were followed up with the diagnosis of sarcoidosis in the rheumatology clinic of Ankara City Hospital between November 2019 and November 2022 were evaluated. Demographic, clinical, radiological, serological, laboratory, and histopathological findings, and rheumatological, systemic, and locomotor system examination findings of the patients were obtained from the medical data registered in the hospital. A total of seventy sarcoidosis patients (48.98 ± 11.78 years, %75 female) were included in the study. Joint involvement was observed in 64.3% of cases, skin involvement in 48.6% of cases, and ocular involvement in 25.7% of cases. The ankle was the most frequently involved joint, followed by the knee and small joints in the foot. Corticosteroids were the most used therapeutic agent, and pulmonary and joint findings were the most common reasons for starting treatment. Sarcoidosis is a disease that mimics many diseases, misdiagnosis and treatment should be avoided with a good and fast differential diagnosis. Clinicians, especially rheumatologists, should remember sarcoidosis more frequently and keep it in mind in the differential diagnosis.

Can hydradenitis suppurativa be associated with inflammatory joint involvement? Report of a case series and review of the literature.

Dear Editor, Hidradenitis suppurativa (HS) or acne inversus, is a chronic inflammatory cutaneous disease with a significant negative impact on the patient's quality of life, from a psychological, social and occupational point of view. The disease mainly affects the female sex and occurs between puberty and 40 years of age. It is estimated that in Europe the prevalence of the disease in the general population is 1%.1 Recent studies have shown a high incidence of inflammatory arthritis, in particular spondyloarthritis, in patients with HS.2 In this report, we present 18 cases of patients with hidradenitis suppurativa, visited at the San Gallicano Dermatological Institute, who presented joint symptoms. [...]

A Practical Overview of the Articular Manifestations of Systemic Lupus Erythematosus.

Although it is widely known that joint involvement is the most frequent and prevalent manifestation of systemic lupus erythematosus (SLE), not having a validated organ-specific index for this domain in order to guide its treatment has been a major limitation. In addition, its clinical importance had been underestimated since it was not a vital risk domain; it was never the center of treatment, under the premise that in most cases its progression was slow and did not lead to significant functional disability.However, this concept has been changing due to the greater description of erosions both in ultrasonography and in osteoarticular magnetic resonance, so their identification can establish a more appropriate treatment time and thus avoid joint deformities, which in some cases can become irreversible.Recently, anifrolumab and belimumab have been able to significantly reduce the Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) and British Isles Lupus Assessment Group (BILAG) index scores, along with improvement in quality of life indices and a significant decrease in the required dose of glucocorticoids. Despite this, the ideal moment to consider biological therapy in this domain is not clear, since the clinical examination can sometimes be biased by the pain associated with fibromyalgia or the fatigue associated with SLE.For this reason, perhaps ultrasonography or magnetic resonance imaging, apart from differentiating the joint phenotype, can identify patients in time to define the onset of disease-modifying antirheumatic drugs and rationalize the use of glucocorticoids.The objective of this review is to characterize in detail the joint manifestations of SLE to offer the clinician a practical view of its diagnosis and treatment.

42590 Successful treatment of psoriatic nails and sub-clinical joint involvement with lindigo naturalis

42590 Successful treatment of psoriatic nails and sub-clinical joint involvement with lindigo naturalis

Role of HLA class I and II alleles in susceptibility to ankylosing spondylitis in Chinese Han.

The objective of the study was to clarify the associations of HLA class I and II alleles with ankylosing spondylitis (AS) among Chinese Han. We performed HLA genotyping and Sanger sequencing for 68 HLA-B*27(-), 62 HLA-B*27(+) AS patients, and 70 controls. Case-control analyses and separate analyses of HLA-B*27(-) patients were performed. One-way ANOVA and Kruskal-Wallis multiple comparisons test were used to analyze the effects of HLA-A\B\C\DRB1\DQB1 alleles on clinical characteristics of HLA-B*27(-) and HLA-B*27(+) patients. In the HLA-B*27(+) group, positive associations were seen with A*11:02, B*27:04, B*27:05, C*02:02, C*12:02, and DRB1*04:01 and negative associations were seen with A*33:03, B*07:02, B*57:01, and C*07:02. The age at onset was greater in HLA-B*27(-) patients than in HLA-B*27(+) patients (30.03 ± 15.15 vs. 23.08 ± 7.79 years). In the HLA-B*27(-) group, those with A*01:01, B*13:01, B*13:02, C*01:02, C*04:01, DQB1*02:01, DQB1*06:01, and DRB1*03:01 had an earlier onset than those without these alleles, while patients carrying B*40:02, C*07:02, C*12:02, C*15:02, DQB1*05:02, and DQB1*05:03 had a delayed onset. In the HLA-B*27(-) group, A*32:01(+), C*08:01(+), and DRB1*04:05(-) women were likely to develop AS. In the HLA-B*27(+) group, DQB1*03:02(+) women may be more likely to develop AS. DRB1*12:02 and HLA-B*27 interacted with the distribution of AS-affected sites. In the HLA-B*27(+) group, DRB1*12:02(+) patients were likely to have peripheral joint involvement. HLA class I and II alleles other than HLA-B*27 contribute to AS predisposition and characteristics among Chinese Han patients.

In Systemic Sclerosis Patients, Peripheral Blood CD21low B Cells and Serum IL-4 and IL-21 Influence Joint Involvement.

Systemic sclerosis (SSc) patients have an increased frequency of CD21low B cells and of serum interleukin-4 (IL-4) and IL-21, each possible markers of joint involvement in inflammatory arthritis. The aim of this study was to investigate the possible influence of CD21low B cells, IL-4, and IL-21 on joint involvement in a cohort of 52 SSc patients. The DAS28-ESR was correlated with CD21low B cells (r = 0.452, p < 0.001), IL-4 (r = 0.478, p < 0.001), and IL-21 (r = 0.415, p < 0.001). SSc patients with a DAS28-ESR > 3.2 had more CD21low B cells (12.65% (IQR: 7.11-13.79) vs. 5.08% (IQR: 3.76-7.45), p < 0.01), higher IL-4 levels (132.98 pg/mL (IQR: 99.12-164.12) vs. 100.80 pg/mL (IQR: 62.78-121.13), p < 0.05), and higher IL-21 levels (200.77 pg/mL (IQR: 130.13-302.41) vs. 98.83 pg/mL (IQR: 35.70-231.55), p < 0.01) than patients with a DAS28-ESR ≤ 3.2. The logistic regression analysis models showed that the DAI (OR: 2.158 (95% CI: 1.120; 4.156), p < 0.05) and CD21low B cells (OR: 1.301 (95% CI: 1.099; 1.540), p < 0.01), the DAI (OR: 2.060 (95% CI: 1.082; 3.919), p < 0.05) and IL-4 level (OR: 1.026 (95% CI: 1.006; 1.045), p < 0.01), and the DAI (OR: 1.743 (95% CI: 1.022; 2.975), p < 0.05) and IL-21 level (OR: 1.006 (95% CI: 1.000; 1.011), p < 0.05) were independently associated with a DAS28-ESR > 3.2. An elevated CD21low B cell percentage, IL-4 level, and IL-21 level was associated with higher articular disease activity in patients, suggesting a possible role in the pathogenesis of SSc joint involvement.

Gender Disparities in Ankylosing Spondylitis: An Algerian Retrospective Analytical Cohort Study

Background: Ankylosing spondylitis (AS) is an inflammatory disease that can impair function and lower quality of life, which causes spinal bone fusion and new bone production. Several studies have been done on the differences in damage between women and men. Objectives: This study aimed to assess the differences between men and women with AS regarding clinical characteristics, structural damage, and treatment used by Algerian patients. Methods: In this study, 292 patients diagnosed with AS at the Hassani Abdelkader Hospital Rehabilitation Department of Sidi Bel Abbes Region between 2018 and 2021 were enrolled. The studied parameters were age, disease duration, age at disease onset, morning stiffness, joint involvements, laboratory data, disease activity, and treatments. All data were processed and analyzed via Excel and SPSS 20.0 (Statistical Package for the Social Sciences, IBM Corporation, Chicago, IL, August 2011). Results: We assessed 166 females and 126 males. The mean age at disease onset was 31.69 ± 10.675 vs. 30.38 ± 10.250 years, and morning stiffness duration was 26.08 ± 26.977 vs. 23.80 ± 26.529 minutes in females and males, respectively. Positive HLA-B27 was noted more in 71.9% of women and 69.6% of men. Also, the female group suffered more from their lumbar (100%) and high inflammation (83.8% elevated erythrocyte sedimentation rate (ESR) and 59.9% positive C-reactive protein (CRP)) rather than females who suffered from a long disease duration (7.28 ± 4.867 vs. 6.74 ± 3.584). Peripheral joints were primarily affected in males than females (knees involvement: 26.4% vs. 3.6% and hips involvement: 69.6% vs. 3%, respectively). In addition, high disease activity was more noted in men (52.8% vs. 41.9%, respectively). The most noted comorbidities, including uveitis, psoriasis, and diabetes, affected more females than males (40% vs. 29.9%, 4.8% vs. 2.4%, and 2.4% vs. 1.2%, respectively). Concerning the treatment, men used more sulfasalazine than women, 86.4% vs. 76%, while women used more Humira than men (88.6% vs. 74.4%, respectively). Conclusions: According to our findings, women had a higher inflammation rate and more damage in their lumbar, requiring more sulfasalazine and Humira treatment than men who suffered from their peripheral joints and higher disease activity.

Malondialdehyde Serum Levels in Patients with Systemic Sclerosis Relate to Dyslipidemia and Low Ventricular Ejection Fraction.

Systemic sclerosis (SSc) is a chronic disease characterized by vasculopathy with the involvement of dysfunctional microcirculatory vessels. Features of the disease include progressive fibrosis of the skin and internal organs and systemic inflammation characterized by the presence of circulating autoantibodies and proinflammatory cytokines. Furthermore, macrovascular disease and atherosclerosis are more common in patients with SSc than in the general population. Oxidative stress plays a crucial role in the development of several processes, including endothelial dysfunction, cancer, inflammation, and atherogenesis. Malondialdehyde (MDA) is a well-established marker of oxidative stress. In this work, we have analyzed the relationship between serum MDA levels and clinical, laboratory, and vascular characteristics in a well-characterized cohort of 53 patients with SSc. A multivariable analysis was performed to study the relationship between circulating MDA and disease characteristics in patients with SSc. Cardiovascular assessment was also performed, including ultrasonography of the carotid and aorta, and echocardiography. MDA showed a significant and positive relationship with the serum levels of lipid profile molecules such as total cholesterol (β coefficient = 0.006 (95% CI: 0.0004 to 0.01), nmol/mL, p = 0.037) and LDL cholesterol (β coefficient = 0.008 (95% CI: 0.001 to 0.01) nmol/mL, p = 0.017). On the contrary, most manifestations of the disease, including skin, lung, and joint involvement, as well as the presence of digital ulcers, were not related to MDA. However, high MDA levels were significantly and independently associated with lower ventricular ejection fraction after adjustment for covariates (β coefficient = -0.04 (95% CI: -0.06 to -0.02), nmol/mL, p = 0.001). In conclusion, serum MDA levels were related to higher levels of total and LDL cholesterol and a lower left ventricular ejection fraction in patients with SSc. MDA could serve as a potential biomarker of dyslipidemia and heart failure in SSc.

How do symptoms of each joint contribute to global pain, disease activity and functional disability in rheumatoid arthritis?-A comprehensive association study using a large cohort.

Established assessment tools for patients with rheumatoid arthritis (RA), including disease activity scores (DASs), disease activity indexes (DAIs), visual analog scales (VASs), and health assessment questionnaires (HAQs), are widely used. However, comparative associations between joint involvement and disease status assessment tools have rarely been investigated. We included a dataset of 4016 patients from a large RA cohort from 2012 to 2019. The tenderness and swelling of each joint were counted as a symptom, with 70 and 68 affected joints throughout the body, respectively. The relative contribution of various joints to the disease status assessment tools, VAS scores, and functional disability indexes was analyzed using multiple regression analysis. The wrist showed the most significant contribution overall, especially in DASs and VASs, while the metacarpophalangeal and proximal interphalangeal joints made significant contributions to DASs and DAIs, but not to VASs and HAQs. The shoulder and the elbow significantly contributed to HAQs, but only the shoulder did to the VASs. The knee universally contributed to all of the tools, but the ankle played a minor but important role in most assessment tools, especially in HAQs. Similar but different contribution ratios were found between the sets of DASs, DAIs, VASs, or HAQs. Each joint makes a unique contribution to these assessment tools. The improvement or aggravation of symptoms in each joint affects the assessment tools in different manners.

Costovertebral and costotransverse joint involvement in spondyloarthritis.

Costovertebral (CV), costotransverse (CTr), sternoclavicular (SC), and manubriosternal (MS) joints are impacted in spondyloarthritis (SpA) patients; however, clinical aspects of these involvements require additional evaluation. A total of 281 SpA patients who had undergone chest computed tomography (CT) for any reason between 2010 and 2020 were included in the study. SpA patients were divided into three groups, ankylosing spondylitis (AS), non-radiographic axial SpA, and psoriatic arthritis. Thirty age- and sex-matched rheumatoid arthritis (RA) patients and 30 non-rheumatic disease individuals were selected for comparison. An experienced radiologist reviewed 24 CV, 20 CTr, 2 SC, and 1 MS joints from a thorax CT for each patient. All joints were classified as: normal (0); suspicious (1), mild (2), moderate (3), or severe (4). Total CV and CTr joint scores differed between diseases (p < .001). Male AS patients had higher CV and CTr scores than female AS patients (male CV score: 52 [range 0-96] and CTr score: 22 [range 0-80]; female CV score: 20 [range 0-96] and CTr score: 12 [range 0-79]). Strong negative correlations were detected in AS patients between chest expansion and CV (r = -0.703 p = .007) and CTr (r = -0.763 p = .002) joint involvement; positive correlations between CV and CTr joints, and modified Stoke Ankylosing Spondylitis Spinal Score (p < .05); and no significant association for MS and SC joints. CV and CTr joint involvement on thorax CT was more severe in AS and negatively affected chest expansion. The use of thorax CT scans performed for other indications in the examination of these joints may be advantageous for the early beginning of rehabilitation programs targeted at maintaining chest mobility.

Distinct NOD2 mutations reported in three families with Blau syndrome (BS) from a single center in India - Case series and review of literature

ObjectiveBlau syndrome (BS), considered a rare pediatric autoinflammatory disease, is characterised by a triad of granulomatous arthritis, dermatitis and uveitis. Here we present a tale of three families visited in our outpatient department in the last two years (2020−2022) where more than one member was affected with either skin, ophthalmological and joint involvement with either biopsy-proven granuloma or genetic mutation at NOD2 gene suggesting the diagnosis of BS. Case seriesThe first family had three affected members where the mother and her two children had skin changes, polyarthritis and a pathogenic mutation in NOD2 gene (exon 4, c.1000C > T, p.Arg334Trp) suggesting BS. The second family had two affected members where both mother and her son had uveitis, skin changes with NOD2 mutation at exon 4 with c.1147G > A (p Glu 383 Lys) variant. The son also had polyarthritis and his skin biopsy was suggestive of granulomatous inflammation. In the third family with two affected members, we found a mutation in NOD2 on exon 4 (c 1324C > T, p.Lys 442 Phe) which was described as pathogenic with only one report published till date. ConclusionThese three cases presented to us within the last two years and led to a diagnosis of BS in three other family members with discrete mutations (commonest to rarest) on the NOD2 gene in the three families.

Early diagnosis of coxitis in ankylosing spondylitis

Coxitis is one of the characteristic clinical manifestations of ankylosing spondylitis (AS). Hip joint (HJ) involvement in AS is considered an un-favorable prognostic factor. Early detection of coxitis is of great importance, since with the timely initiation of adequate treatment, the risk of developing irreversible changes in HJ can be reduced.The lecture discusses the issues of clinical and instrumental diagnosis of coxitis in AS and presents an algorithm for its early detection developed by the authors.

Dystrophic calciphylaxis in panniculitis: features of the clinical picture and diagnosis

The clinical presentation of lobular panniculitis (PN) associated with calciphylaxis (CP, calcification) can vary widely and may be associated with joint and internal organs involvement, making the diagnosis of the disease difficult.Objective: to evaluate the frequency and significance of CP in patients with PN using long-term prospective follow-up.Material and methods. From 2018 to 2023, at the V.A. Nasonova Research Institute of Rheumatology 217 patients with referral diagnosis "erythema nodosum" or "panniculitis" were examined. In 19.3% of cases (9 men and 33 women aged 37 to 72 years) CP was confirmed with an average disease duration of 56.3±11.2 months. Clinical examination of patients was performed according to the standards recommended by the Russian Association of Rheumatologists. International criteria were used to confirm the diagnosis of systemic lupus erythematosus (SLE), idiopathic inflammatory myopathies (IIM), systemic sclerosis (SS), and lipodermatosclerosis (LDS). In 12 patients with indurations, pathological examination of biopsy specimens of skin and subcutaneous fatty tissue from the area of induration was performed, which allowed confirming the diagnosis of idiopathic lobular PN (ILPN) in 3 cases. Four grades of calcification were distinguished according to the size and depth of the calcifications. In addition, considering the type of radiological changes and clinical manifestations, four subtypes of CP were identified: mousse-like, stone-like, mesh-like and lamellar-like.Results and discussion. In the study group, the ratio of women to men was 3.6:1, and the mean age was 43.8±7.6 years. On clinical examination we determined, in 60% of cases CP predominantly stone-like subtype (71.4%) of first grade (47.6%), which was significantly more frequently located on the upper and/or lower extremities and/or trunk (57.1%; p=0.05). Using clinical, laboratory and instrumental data, we confirmed the development of CP in ILPN (n=3), SLE (n=3), LDS (n=21), IIM (n=5), SS (n=1), and idiopathic CP (n=9) with a mean disease duration of 8.7±2.4 years.An increase in ESR and CRP levels occurred in different diseases, while urinary syndrome was associated with SLE (66.6%) and an increase in creatinine phosphokinase with IIM. Decreased calcium and 25-hydroxyvitamin D levels and increased phosphorus and parathyroid hormone levels were found in many patients studied.Conclusion. In the absence of clear diagnostic criteria for CP in patients with PN, early diagnosis is critical for the development of an effective multidisciplinary treatment plan.

Fostering concentration and engagement of peer mediators in primary classrooms as joint concentration agents

The concept of joint concentration agents suggests that kids actively promote and sustain Fostering Concentration (FC) and engagement between their classmates. Fostering concentration and engagement in the current setting means establishing an atmosphere that motivates kids to pay FC and actively engage in educational activities. This might include a variety of tactics, approaches, and interventions that educators use to improve their students' capacity for sustained FC and engagement throughout class activities. Primary Classrooms (PC) strongly emphasize social and emotional development in addition to intellectual study. Students pick up social skills such as cooperating, sharing with peers, and developing empathy. Kids with developmental difficulties, such as Autism Spectrum Disorder (ASD), risk being excluded from other kids of the same age. Additionally, studies show that focused therapies and support benefit kids with ASD, even in inclusive environments. Joint Concentration (JC) has been recognized as a critical talent for improving period-suitable social skills and announcements. JC is a joint involvement between two persons on a third item or event. An increasing corpus of research describes successful interventions on JC skills for kids with ASD; despite this, few studies document the results of an intervention that uses a natural change agent. In the current research, Response to Joint Attention (RJA) behaviours shown by seven pre-schoolers with ASD was assessed using independent concurrent multiple baseline designs. The Peer Mediators (PM) can carry out a direct interference with accuracy, and the seven peers' overall RJA behaviours improved as a result of the intervention, as did the number of peer bids and replies to those bids.&#x0D;

A case series on recurrent and persisting IgA vasculitis (Henoch Schonlein purpura) in children

BackgroundIgA vasculitis (IgAV) is a small vessel vasculitis that is more common in childhood. Very limited evidence exists on patients who experience an atypical disease course. The aim of this study was to describe a cohort of children diagnosed with recurrent or persisting IgAV to identify any themes associated with their disease course and areas of unmet needs.MethodsA single centre retrospective study of children diagnosed with recurrent or persisting IgAV at Alder Hey Children’s Hospital (Liverpool, UK). Clinical data, including features at presentation and during follow up, potential triggers, abnormal laboratory and histology results, treatment and outcome at last clinical review were retrospectively collected. Key themes were identified.ResultsA total of 13 children met the inclusion criteria (recurrent disease, n = 4; persisting disease, n = 9). Median age at first presentation was 10.2 years [2.6–15.5], female:male ratio 1.2:1. Children in the atypical cohort were significantly older than a larger cohort of children who followed a non-complicated disease course (median age 5.5 years (range [0.6–16.7], p = 0.003)). All children re-presented with a purpuric rash (either recurring or persisting), accompanied by joint involvement in 92% of patients (12/13). Disease-modifying anti-rheumatic drugs (DMARDs) were used in 8/13 (62%) children. The median time from first presentation to diagnosis of atypical disease was 18.4 months [5.3-150.8] and the time from first presentation to treatment was 24.1 months [1.8–95.4]. Use of corticosteroids was significantly higher in children with renal involvement (p = 0.026). During follow up, 8/13 (62%) children were admitted at least once, whilst 10/13 (77%) had re-presented at least once to the emergency department. Five (38%) children were referred to psychology services and 7 (54%) children reported feelings of frustration.ConclusionsThis series describes some characteristics of a small cohort of children with atypical IgAV. It also identifies unmet needs in children with atypical IgAV, which includes delays in diagnosis and lengthy waits for treatment, lack of high-quality evidence regarding treatment choices and a high unrecognised disease burden. Further research is needed to study this subgroup of children as evidence is lacking.

Melioidosis as a Mystique Infection: A Study From Central India.

Melioidosis is caused by the Gram-negative bacilli Burkholderiapseudomallei, which is found in contaminated water and soil and spreads via inhalation, inoculation, and ingestion. Melioidosis manifests diversely in immunocompetent and immunocompromised patients, ranging fromasymptomatic to life-threateningrespiratory distress, septic shock, localized tissueinfection, necrotizing pneumonia, and soft organ abscesses. Methods:An 18-month observational study was conductedat a tertiarycenterin central India among various confirmed melioidosis cases, with data gathered and analyzed. Aerobic culture and sensitivity were performed in allstudiedcases,either in blood/body fluid/localized collection- using blood agar media for the culture and disc diffusion method on Mueller Hinton agarfor sensitivity. Other tests, such as radiological imaging, were conducted according to symptoms and signs of localized infection. The melioidosis cases under study were compared on various clinical/presenting parameters.Melioidosis has a variety of risk factors,but we found that, in India, diabetic patients are at a higher risk of this infection, particularly fatal forms, as all of the patients in our study were diabetic.Melioidosis is known to have joint involvement, either as a source of infection or later in the course of the disease. All cases were successfully treated with antibiotics and surgical procedures, demonstrating the significance of determining disease etiology, early diagnosis, and rapid early management. Melioidosis is a potentially fatal disease, particularly in diabetics, with a wide range of symptoms and complications. Physicians face a variety of challenges, including clinical symptoms resembling other chronic illnesses, such as tuberculosis, delays in laboratory confirmation, underdiagnosis, reduced reporting, and a lack of suspicion. Because there are very little data and it is a seldom reported infection from central India, we are publishing a study on seven melioidosis patients.

E41 PAPA Syndrome: response to methotrexate in four members of the same family

Abstract Introduction PAPA syndrome (Pyogenic Arthritis, Pyoderma gangrenosum and Acne) is a rare autosomal dominant disease that mainly affects the joints and skin. It is linked to a mutation of a gene of a protein named PSTPIP1 located on chromosome 15q. Arthritis is destructive, oligoarticular and predominantly affects large joints. It usually begins during childhood and not responsive to most treatments. The diagnosis is late and arises with infectious arthritis, given the destructive and pyogenic nature of arthritis and biological inflammatory syndrome. Aim We describe the cases of four members of the same family affected by PAPA syndrome who were treated empirically with long-term corticosteroids and then methotrexate to improve their joint involvement. Case reports We report the cases of 4 male patients belonging to the same large family (3 brothers and a first cousin), aged 28, 23, 21 and 15 years (patients 1–4, classified by age), affected by PAPA syndrome who presented with recurrent osteoarticular infections treated with antibiotics. They have no other conditions except vitiligo in patient 1 and Down syndrome in patient 3. Diagnosis was made in patient 1 based on joint and skin involvement. Other family members were subsequently called for diagnostic evaluation. A treatment combining methotrexate (10–15 mg/week) and corticosteroids (5 mg/day of prednisolone weaned and discontinued after 6–12 months of initiation) was associated with clinical and biological response in all patients with a decrease in pain, clinical and ultrasound improvement of synovitis and normalization of biological inflammatory markers in all four cases. Methotrexate was well tolerated and maintained as monotherapy, associated with on demand NSAIDs. The current average follow-up is 2.3 years. Conclusion Through our observations, we report a possible beneficial effect and good tolerance of methotrexate on joint involvement in PAPA syndrome. A more rigorous study (larger sample size, control arm) is necessary to validate this treatment.

E25 The pattern of juvenile psoriatic arthritis in a tertiary centre in Libya

Abstract Background Juvenile psoriatic arthritis (JPsA) is a relatively rare condition in childhood as it represents ∼5% of the whole Juvenile Idiopathic Arthritis (JIA). There are fewer reports describing the characteristics and outcome of patients with JPsA. Aim To describe the characteristic features and the treatment modalities of JPsA among Libyan children in pediatric rheumatology clinic at the Tripoli Children Hospital which is the only referral clinic covering the western, northern and southern part of Libya and to compare our results with other populations worldwide. Methods All the medical records of the children who were diagnosed to have JPsA according to ILAR classification criteria or Vancouver criteria (definite or probable) from January 2001 to January 2020 were included and retrospectively reviewed. Data were collected about demographic, clinical, and laboratory features and treatment Results The study included a total of 30 cases of JPsA that represents a 12% of total JIA cases over the study period, the mean age at presentation was 5.8 ± 5.3 years with male to female ratio of 1:1, with the mean duration of symptoms prior to diagnosis of JIA of 9 ± 10 months. The foremost common type of arthritis was peripheral polyarthritis (83% &amp; 58.3%), with the Small joints commonly affected, 30% of patients had hip joint involvement. 13% of the patient had psoriasis, half of them before presentation of arthritis. Nail changes secondary to psoriasis was seen in 1 of the cohort, and the percentage of patients with dactylitis among the group was 30%. Family history of psoriasis found in 83% of the cohort, 26% found to be ANA positive. Chronic uveitis occurred in 10%. Patients of the total cohort, ANA positive, Unilateral anterior uveitis being the most common presentation. The following table summarizes the Medications used during the course of the disease At the time of the first visit 100%of total patients fulfilled the Vancouver criteria for juvenile PsA, While 5 of the12 patients didn’t fulfilled the ILAR criteria for JPsA. Conclusions Juvenile psoriatic arthritis constituted 12% of our patients with juvenile idiopathic arthritis. Juvenile psoriatic arthritis constitutes 8% to20% of JIA patients worldwide with equal gender distribution and peak age at presentation of 6–13 years. More than four peripheral joints involvement were the most frequent presentation. Half of the patients had skin involvement before the development of arthritis. Prevalence of chronic anterior uveitis was higher in our cohort than worldwide (10% vs 5% respectively) as an extra articular manifestation. All patients were treated with NSAIDs at presentation and/or during follow-up. Methotrexate was the most commonly used second-line agent Ethics Our study was approved by local ethical committee of pediatric department at Tripoli children hospital.

E10 Temporo-mandibular joint involvement risk factors in juvenile idiopathic arthritis

Abstract Introduction Recent recommendations suppose temporomandibular joint (TMJ) involvement in juvenile idiopathic arthritis (JIA) is a risk factor of poor outcomes and severe disease course. Objectives To compare the clinical characteristics of patients with juvenile idiopathic arthritis with and without TMJ involvement. Methods We analyzed the data of 753 patients with juvenile idiopathic arthritis aged 2–17 years, depending on TMJ arthritis (n = 43; 5.7%) or no TMJ arthritis (n = 710; 94.3%). Clinical, laboratory characteristics and treatment regimens were compared. Odds ratio (OR) analysis of sensitivity (Se) and specificity (Sp) was performed to obtained the predictors of TMJ involvement. Results Despite the similar age of onset, TMJ arthritis was associated with longer course of the disease, polyarticular JIA category, use of systemic corticosteroids and longer achievement of remission with similar rate of biologic administration. TMJ arthritis was associated with involvement of cervical spine, hip and shoulder arthritis. The main risk factors, associated with TMJ involvement in JIA were active joints &amp;gt; 8 (OR = 14.9 (5.8; 38.3), P = 0.0000001), delayed remission &amp;gt; 7 years (OR = 3.1 (1.6; 5.8), P = 0.0004), delayed hip involvement (OR = 4.6 (0.9; 22.6), P = 0.041), hip osteoarthritis (OR = 4.0 (1.2; 13.0), P = 0.014), and avascular necrosis of the hip. Cervical spine arthritis (OR = 10.3 (5.4; 19.8), P = 0.000001), and corticosteroid treatment (OR = 2.3 (1.2; 4.4), P = 0.0007) were associated with TMJ arthritis. Patients with TMJ arthritis required more biologics (OR-3.2 (1.6; 6.2), P = 0.0006). Oligoarticular JIA category and uveitis were protective against TMJ involvement. Data are in the table. Conclusion TMJ involvement is marker of poor disease prognosis. Early initiation of biologics and avoidance of corticosteroids might improve the risk of TMJ involvement. Ethics Written consent was obtained according to the declaration of Helsinki. This retrospective study's protocol was approved by the local Ethical Committee of Saint Petersburg State Pediatric Medical University (protocol number 11/10 from 23.11.2020).

E62 Paediatric rheumatology cases at department of physical medicine/rheumatology Victoria hospital over nine years

Abstract Introduction Victoria Hospital is a centrally located public hospital in Mauritius with a catchment area attaining around 500 000 people. Paediatric rheumatic cases refer to rheumatic diseases occurring in children below the age of 16. Till date there is no official data on the number of paediatric rheumatic cases seen at Victoria hospital. Objective To study paediatric rheumatic cases seen and followed up over a period of nine years at Victoria Hospital Mauritius. Methods This is a retrospective study conducted in the department of Physical Medicine/Rheumatology. Data was extracted from files of patients below age of 16 following treatment over the last 9 years (December 2013 till December 2022). Basic information on age, gender, family situation, were noted. Clinical information on diagnosis, treatment and outcomes were also noted. Data was processed using IBM SPSS. Results A total of 18 children are being followed by our department since the last 9 years and they had been seen by 3 different rheumatologists. 13 patients (72.2%) were referred internally either through Paediatrics or Unsorted Outpatient units, and 5 patients (27.7%) were referred from private hospitals. The ratio of male to female was 1:5. The mean age of onset of symptoms was 9.7 ± 4.75 years, mean age at which a diagnosis was made was 10.7 ± 4.50 years, and the mean age at which patients were referred to our department was 11.1 ± 4.45 years. The mean diagnostic delay is one year. Four cases are adults now. 9 cases (50%) are being followed for Juvenile Idiopathic Arthritis (JIA) of which 7 (38.9%) had polyarticular JIA (pJIA) and 2 (11.1%) had systemic JIA (sJIA). Paediatric Systemic Lupus Erythematosus accounted for 5 cases (27.8%), 1(5.6%) had Juvenile Dermatomyositis , 1 (5.6%) had Juvenile Fibromyalgia, 1(5.6%) had Psoriatic Arthritis and 1 (5.6%) had Rheumatic Fever. 36.8% of children have single parents and 44.4% of parents did manual jobs. Social status of parents statistically correlate with disease conditions (P &amp;lt; 0.001). Nine (50%) patients were on methotrexate, 8 (44.4%) of JIA were treated with biological DMARDs (bDMARDs), 4 of them achieved remission and has stopped bDMARDs and 4 of them are on maintenance dose for remission. Tocilizumab was the mostly used bDMARDs 5 (62.5%), followed by rituximab 2 (25%); other bDMARDs used were infliximab 1 (12.5%) and adalimumab 1 (12.5%). Uveitis was seen in one of the patients with JIA and is being managed with infliximab. Two patients (one systemic JIA and one SLE) have hip joint involvement. The SLE case has developed osteonecrosis of hip joints. patients (55.5%) are in remission at present, 4 (22.2%) in partial remission, 3 (16.7%) still having repeated flares and one has physical impairment. Discussion This is the first published set of data on Paediatric Rheumatology cases at Victoria Hospital. A diagnostic delay of around one year is noted. 34.5% of children live with single parents and hence might not have received the care and support as required. Furthermore, the cases seen could only be part of the tip of the iceberg as patients are often lost in between paediatric and rheumatology units. Paediatric Lupus patients remain challenging to manage. Conclusion This is the first report of published data on paediatrics rheumatic disease from Victoria Hospital in Mauritius. We need more collaborative data from other centres to work out the prevalence and incidence of paediatric rheumatic diseases in our country. Efforts must be made to shorten diagnostic delay and referral time.