Darier-Roussy Subcutaneous Sarcoidosis Masquerading as Multiple Abscesses: A Report of a Rare Case.

Sarcoidosis is an idiopathic multisystemic granulomatous disease that mainly affects the lungs. Darier-Roussy subcutaneous sarcoidosis is among the specific and least encountered skin manifestations of sarcoidosis. In this case study, we report how subcutaneous sarcoidosis could mimic multiple abscesses presentation and hinder reaching a definitive diagnosis. A 65-year-old female presented with five, multiple, deep-seated skin lesions on the forearm, chest, and scalp. The lesions showed redness and tenderness. The patient also experienced arthralgia in the right ankle. Laboratory workup of the patient showed a high erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), and white blood cell (WBC) count. The patient was suspected to have multiple abscesses, which were managed with antibiotics with no response. Thus, a computed tomography (CT) scan of the chest was done and showed mediastinal lymphadenopathy. A biopsy was taken from one of the right forearm skin lesions, and it revealed characteristic features consistent with sarcoidosis. The patient was managed with hydroxychloroquine and a tapering dose of prednisone. Therefore, subcutaneous sarcoidosis should be included in the differential diagnosis of subcutaneous lumps.

Extraarticular manifestations of juvenile idiopathic arthritis and their impact on health-related quality of life.

OBJECTIVES: The objective of this study is to investigate extraarticular manifestations (EAMs) in patients with juvenile idiopathic arthritis (JIA) and assess their impact on health-related quality of life (HRQoL) among these patients. METHODS: This cross-sectional analytic study was carried out on 117 patients with JIA. EAMs were identified clinically by history and examination. Sicca symptoms, peripheral neuropathy, enthesitis, and skin lesions were picked up during clinical examination. Pulmonary involvement was evaluated by high-resolution CT chest. Patients were assessed by abdominal ultrasonography to assess the size of liver and spleen. Atlantoaxial subluxation was evaluated by cervical spine x-rays. Patients were evaluated by Pediatric Quality of Life Inventory-4 (PedsQL-4) and PedsQL-3 arthritis module. RESULTS: The median age of patients was 14 years with a median disease duration 4 years, 82.9% were females. Of the studied 117 JIA patients, 85 patients (72.6%) had at least one EAM. Persistent fatigue (51.3%) was the most prevalent EAM, followed by recurrent skin rash (16.2%), enthesitis (15.4%), recurrent fever (13.7%), and uveitis (12%). Patients with EAMs scored significantly lower in physical functioning (p = 0.001), emotional functioning (p < 0.001), social functioning (p = 0.005), and school functioning (p = 0.001). Regarding PedsQL arthritis module, patients with EAM had also significantly lower scores than did patients without EAM on the domains of pain and hurt (p < 0.001), daily activities (p = 0.008), and worry (p = 0.001). RESULTS: EAMs are prevalent among JIA patients and have a negative impact on their HRQoL. So, early identification and treatment are highly recommended. Key Points • A large percentage of JIA patients experienced at least one extraarticular manifestation (EAM). • Persistent fatigue and recurrent skin rash are the most prevalent EAMs in JIA patients. • JIA patients with EAMs have worse scores in almost all domains of HRQoL.

Pulmonary embolism versus pulmonary vasculitis in Hughes-Stovin syndrome: Characteristic computed tomography pulmonary angiographic findings and diagnostic and therapeutic implications. HSS International Study Group.

BACKGROUND AND AIM: Hughes-Stovin syndrome (HSS) is a rare systemic vasculitis with widespread venous/arterial thrombosis and pulmonary vasculitis. Distinguishing between pulmonary embolism (PE) and in-situ thrombosis in the early stages of HSS is challenging. The aim of the study is to compare clinical, laboratory, and computed tomography pulmonary angiography (CTPA) characteristics in patients diagnosed with PE versus those with HSS. METHODS: This retrospective study included 40 HSS patients with complete CTPA studies available, previously published by the HSS study group, and 50 patients diagnosed with PE from a single center. Demographics, clinical and laboratory findings, vascular thrombotic events, were compared between both groups. The CTPA findings were reviewed, with emphasis on the distribution, adherence to the mural wall, pulmonary infarction, ground glass opacification, and intra-alveolar hemorrhage. Pulmonary artery aneurysms (PAAs) in HSS were assessed and classified. RESULTS: The mean age of HSS patients was 35 ± 12.3 years, in PE 58.4 ± 17 (p < 0.0001). Among PE 39(78 %) had co-morbidities, among HSS none. In contrast to PE, in HSS both major venous and arterial thrombotic events are seen.. Various patterns of PAAs were observed in the HSS group, which were entirely absent in PE. Parenchymal hemorrhage was also more frequent in HSS compared to PE (P < 0.001). CONCLUSION: Major vascular thrombosis with arterial aneurysms formation are characteristic of HSS. PE typically appear loosely-adherent and mobile whereas "in-situ thrombosis" seen in HSS is tightly-adherent to the mural wall. Mural wall enhancement and PAAs are distinctive pulmonary findings in HSS. The latter findings have significant therapeutic ramifications.

Clinical implications of seropositive and seronegative autoantibody status in rheumatoid arthritis patients: A comparative multicentre observational study.

Background and Objectives: Rheumatoid factor (RF) and anti-cyclic citrullinated protein (anti-CCP) have been used to improve the diagnosis and prognosis of rheumatoid arthritis (RA). However, their association with RA disease phenotypes, individually and in combination, is not well studied. The aim of the study was to compare patients' and disease characteristics, activity and severity in double seronegative (DNRA), single seropositive RF, single seropositive anti-CCP and double seropositive (DPRA) patients. Methods: Adults subjects with RA from Egyptian College of Rheumatology (ECR) database who had RF and anti-CCP results available were included. Demographic, clinical features, disease activity score 28 (DAS28), Health Assessment Questionnaire (HAQ) and laboratory data were collected and compared among different RA groups. Results: 5268 RA patients with mean age of 44.9±11.6 years, and 4477 (85%) were females. 2900 (55%) had DPRA, 892 (16.9%) had single positive RF, 597 (11.3%) had single positive anti-CCP while 879 (16.7%) had DNRA. Patients with DPRA had significantly high percentage of metabolic syndrome (19.3%, P < 0.001), and functional impairment using HAQ (P = 0.01). Older age (RRR [relative risk ratio]: 1.03, 95%CI: 1.0, 1.0, P = 0.029), greater DAS28 (RRR: 1.51, 95%CI: 1.2, 1.9, P < 0.001), higher steroid use (RRR: 2.4, 95%CI: 1.36, 4.25, P = 0.002) were at higher risk of DPRA while longer disease duration (RRR: 1.08, 95%CI: 1.01, 1.16, P = 0.017) and fibromyalgia syndrome (RRR: 2.54, 95%CI: 1.10, 5.88, P = 0.028) were associated with higher odds of single positive RF status. Conclusion: Dual antibody-positive status has higher disease activity and severity, and higher chance of development of metabolic syndrome; highlighting the implicated role of inflammation, atherogenesis and cardiovascular disease risk in RA.

Musculoskeletal symptoms in systemic lupus erythematosus patients and their impact on health-related quality of life.

OBJECTIVES: To evaluate the musculoskeletal (MSK) symptoms experienced by SLE patients and determine how those symptoms relate to their health-related quality of life (HRQoL). MATERIALS AND METHODS: This is a cross-sectional study that was carried out on 103 adult SLE patients. sociodemographic, clinical, and therapeutic data were recruited. They were asked to complete the following: Nordic Musculoskeletal, Short-Form McGill Pain, and Lupus QoL Questionnaires. RESULTS: The mean age was 30.81 ± 9.44 years. There was a total of 86 females and 17 males (F: M:5:1). Almost all the patients reported MSK symptoms (96.1%). The maximum number of patients reported pain in the right and left wrist and hand (64.1%, 63.1%, respectively). One-fourth (25.2%) described at least five bodily sites of MSK symptoms, while 70.9% had more than five sites of MSK symptoms. Most of the patients described the pain as discomforting (40.8%). Patients with MSK symptoms scored significantly worse in all domains. In addition, the QoL scores of patients with more than 5 body sites of MSK symptoms were significantly lower than those of patients with fewer than 5 sites of MSK symptoms. CONCLUSION: SLE patients have a high MSK burden, and MSK symptoms have a negative impact on HRQoL in these patients.

Ocular Manifestations in Juvenile Behçet's Disease: A Registry-Based Analysis from the AIDA Network.

INTRODUCTION: This study aims to characterize ocular manifestations of juvenile Behçet's disease (jBD). METHODS: This was a registry-based observational prospective study. All subjects with jBD from the Autoinflammatory Diseases Alliance (AIDA) Network BD Registry showing ocular manifestations before 18 years were enrolled. RESULTS: We included 27 of 1000 subjects enrolled in the registry (66.7% male patients, 45 affected eyes). The median (interquartile range [IQR]) age at ocular involvement was 14.2 (4.7) years. Uveitis affected 91.1% of eyes (anterior 11.1%, posterior 40.0%, panuveitis 40.0%), retinal vasculitis 37.8% and other manifestations 19.8%. Later onset (p = 0.01) and male predominance (p = 0.04) characterized posterior involvement. Ocular complications occurred in 51.1% of eyes. Patients with complications had earlier onset (p < 0.01), more relapses (p = 0.02) and more prolonged steroidal treatment (p = 0.02). The mean (standard deviation [SD]) central macular thickness (CMT) at the enrolment and last visit was 302.2 (58.4) and 293.3 (78.2) µm, respectively. Fluorescein angiography was pathological in 63.2% of procedures, with a mean (SD) Angiography Scoring for Uveitis Working Group (ASUWOG) of 17.9 (15.5). At the last visit, ocular damage according to the BD Overall Damage Index (BODI) was documented in 73.3% of eyes. The final mean (SD) best corrected visual acuity (BCVA) logMAR was 0.17 (0.47) and blindness (BCVA logMAR < 1.00 or central visual field ≤ 10°) occurred in 15.6% of eyes. At multivariate regression analysis, human leukocyte antigen (HLA)-B51 + independently predicted a + 0.35 change in the final BCVA logMAR (p = 0.01), while a higher BCVA logMAR at the first assessment (odds ratio [OR] 5.80; p = 0.02) independently predicted blindness. CONCLUSIONS: The results of this study may be leveraged to guide clinical practice and future research on this rare sight-threatening condition.

Musculoskeletal symptoms and their impact on health-related quality of life in chronic nonbacterial osteomyelitis patients.

INTRODUCTION: Chronic non-bacterial osteomyelitis (CNO) is a rare, non-infection- related inflammatory disorder that affects children and teens. Clinical manifestations of CNO range widely from moderate, time-limited, monofocal inflammation of the bone to extreme multifocal or chronically active inflammation of the bone. OBJECTIVES: The main aim of this study was to explore the correlation between musculoskeletal (MSK) symptoms and health-related quality of life (HRQoL) in patients with CNO. METHODS: Children and adults with CNO and their parents were asked to answer a web-based survey. The survey consisted of multiple questions centered around demographic, clinical and therapeutic data, MSK discomfort form based on the Nordic MSK Questionnaire and HRQoL based on Pediatric Quality of Life Inventory-4 (PedsQL-4) and PedsQL rheumatology module. The inclusion criteria included diagnosis of CNO before the age of 18. Patients who had malignancies or any chronic rheumatic, MSK, neurological disease prior to CNO onset were excluded. RESULTS: There was a total of 68 participants, mostly females (66.2%), with median age 14 years and median disease duration 4.75 years. The median number of bones affected by CNO was 5 and ranged from 1 to 24 bones. Among the studied patients, 45 patients (66.2%) had MSK manifestations at the last month. The most commonly affected part was ankle and feet (26.5%). Regarding HRQoL, patients with MSK manifestations had lower scores than did patients without in PedsQL-4 (p < 0.001) including domains of physical functioning (p < 0.001), emotional functioning (p = 0.033), social functioning (p < 0.001) and school functioning (p = 0.007) in addition to lower scores in PedsQL rheumatology module (p < 0.001) including domains of pain and hurt (p < 0.001), daily activities (p < 0.001), treatment (p = 0.035), worry (p = 0.001) and communication (p < 0.001). CONCLUSION: MSK manifestations have a negative impact on HRQoL in CNO patients. So, early identification and treatment are highly recommended.

Concordance between muscle mass assessed by bioelectrical impedance analysis and by muscle ultrasound: a cross-sectional study in a cohort of patients on chronic hemodialysis.

BACKGROUND: Sarcopenia is a common problem in hemodialysis (HD) patients, and it is diagnosed by low muscle mass, strength and/or low physical performance. Muscle ultrasound (US) is a non-invasive portable tool that might be used for assessment of muscle mass. The aim of the current study was to investigate the concordance between muscle US and bioelectrical impedance analysis (BIA) in diagnosis of sarcopenia in HD patients. METHODS: This cross-sectional study included 41 HD patients. Sarcopenia was diagnosed according to the European Working Group on Sarcopenia in Older People (EWGSOP). The skeletal mass index was measured by BIA and the muscle strength was measured by handgrip strength. Muscle US was used to measure cross-sectional area (CSA) and thickness of quadriceps and biceps muscles. RESULTS: The current study included 41 patients on HD (25 males), with a mean (SD) age of 44.18 (13.11) years and a median HD duration of 48 months. Sarcopenia was diagnosed in 58.5% of the patients. Patients with sarcopenia had significantly lower quadriceps muscle CSA than those without sarcopenia. The optimal cut-offs of quadriceps muscle CSA for both males and females for the diagnosis of sarcopenia were 2.96 and 2.92 cm2, respectively. CONCLUSION: Sarcopenia is prevalent among Egyptian HD patients. US on quadriceps muscle CSA could be used for diagnosis of sarcopenia in these patients.

Effectiveness and Safety of Biosimilars in Pediatric Non-infectious Uveitis: Real-Life Data from the International AIDA Network Uveitis Registry.

INTRODUCTION: Since many biological drug patents have expired, biosimilar agents (BIOs) have been developed; however, there are still some reservations in their use, especially in childhood. The aim of the current study is to evaluate the efficacy and safety of tumor necrosis factor (TNF) inhibitors BIOs as treatment for pediatric non-infectious uveitis (NIU). METHODS: Data from pediatric patients with NIU treated with TNF inhibitors BIOs were drawn from the international AutoInflammatory Disease Alliance (AIDA) registries dedicated to uveitis and Behçet's disease. The effectiveness and safety of BIOs were assessed in terms of frequency of relapses, risk for developing ocular flares, best-corrected visual acuity (BCVA), glucocorticoids (GCs)-sparing effect, drug survival, frequency of ocular complications, and adverse drug event (AE). RESULTS: Forty-seven patients (77 affected eyes) were enrolled. The BIOs employed were adalimumab (ADA) (89.4%), etanercept (ETA) (5.3%), and infliximab (IFX) (5.3%). The number of relapses 12 months prior to BIOs and at last follow-up was 282.14 and 52.43 per 100 patients/year. The relative risk of developing ocular flares before BIOs introduction compared to the period following the start of BIOs was 4.49 (95% confidence interval [CI] 3.38-5.98, p = 0.004). The number needed to treat (NNT) for ocular flares was 3.53. Median BCVA was maintained during the whole BIOs treatment (p = 0.92). A significant GCs-sparing effect was observed throughout the treatment period (p = 0.002). The estimated drug retention rate (DRR) at 12-, 24-, and 36-month follow-up were 92.7, 83.3, and 70.8%, respectively. The risk rate for developing structural ocular complications was 89.9/100 patients/year before starting BIOs and 12.7/100 patients/year during BIOs treatment, with a risk ratio of new ocular complications without BIOs of 7.1 (CI 3.4-14.9, p = 0.0003). Three minor AEs were reported. CONCLUSIONS: TNF inhibitors BIOs are effective in reducing the number of ocular uveitis relapses, preserving visual acuity, allowing a significant GCs-sparing effect, and preventing structural ocular complications. TRIAL REGISTRATION: ClinicalTrials.gov ID NCT05200715.

Clinical and Ultrasonographic Characteristics of the Achilles Tendon in Hemodialysis Patients.

Background and Objectives: The early recognition of tendon alterations in chronic hemodialysis (HD) patients, an awareness of the factors that influence the condition, and active intervention have considerable clinical relevance. The aim of this study was to investigate the musculoskeletal ultrasound (MSUS) features of the Achilles tendon in chronic HD patients and determine the factors associated with tendon abnormalities. Materials and Methods: This study was conducted on 46 HD patients and 24 sex- and age-matched controls. All participants were evaluated clinically for any signs of Achilles tendon abnormalities. Then, the Achilles tendon was scanned bilaterally using MSUS. Results: Among the 92 Achilles tendons in the HD patients, there was tenderness and swelling of only two (2.2%). Regarding MSUS features, there were statistically significant higher thicknesses in the proximal end (p < 0.001), midpoint (p < 0.001), and distal end (p < 0.001) of the Achilles tendons in the HD patients when compared with the healthy controls. Tendinosis was found in 12 (13%) of the HD patients' Achilles tendons, which was statistically significant in comparison to the healthy controls (p = 0.008). There were statistically significant higher scores of structural abnormalities (p = 0.005), bone erosions (p = 0.017), and calcifications (p = 0.015) in the HD patients when compared to the healthy controls. According to the results of a univariate regression analysis, age and male gender were predictive for US abnormalities in HD patients (p = 0.002 and 0.025, respectively). Conclusions: The Achilles tendon in subjects on chronic HD showed frequent US abnormalities. These abnormalities in HD patients appear to be more related to age and gender and may be asymptomatic.

Tixagevimab and Cilgavimab (Evusheld) Boosts Antibody Levels to SARS-CoV-2 in End-Stage Renal Disease Patients on Chronic Hemodialysis: A Single-Center Study.

Background and Objectives: In addition to a suboptimal and rapidly diminishing response to the coronavirus disease 2019 (COVID-19) vaccine, hemodialysis (HD) patients are at risk for developing a severe COVID-19 infection. In 2022, the combination of cilgavimab and tixagevimab (Evusheld, AstraZeneca) was approved for COVID-19 preexposure prophylaxis in high-risk groups. The purpose of this study was to evaluate the humoral response and short-term safety of this antibody combination in a group of HD patients. Materials and Methods: Seventy-three adult maintenance hemodialysis patients were recruited from a tertiary-care hospital for this double-blinded, non-randomized, placebo-controlled study. Patients were placed into two groups: the intervention group (n = 43) received a single 300 mg dosage of cilgavimab and tixagevimab, while the control group (n = 30) received a saline placebo. The titer of COVID-19-neutralizing antibodies was measured at baseline and after 1 and 6 months. The patients were evaluated for any drug-related adverse effects and monitored for six months for the emergence of any COVID-19-related events. Results: Patients in the intervention group were substantially older and had been on HD for longer (p = 0.002 and 0.006, respectively). The baseline antibody levels were higher in the Evusheld group. The antibody level in the intervention group increased significantly after 1 month and remained consistent for 6 months, whereas the antibody level in the control group fell significantly after 6 months during the study period (Wald χ2 = 30.620, p < 0.001). The drug-related adverse effects were modest and well-tolerated, and only seven patients experienced them. Six months after study enrollment, 10 patients in the intervention group and 6 patients in the control group had been infected with COVID-19, respectively. In the control group, ICU admission and mortality were observed, but in the intervention group, the infection was milder with no aggressive consequences. Conclusions: This study demonstrated the short-term safety and efficacy of tixagevimab-cilgavimab for COVID-19 preexposure prophylaxis in HD patients. These findings require more studies with more HD patients and longer follow-up periods.

Association between toxoplasmosis and autoimmune rheumatic diseases in Egyptian patients / Asociación entre toxoplasmosis y enfermedades reumáticas autoinmunes en pacientes egipcios

Purpose: To explore the association between T. gondii and autoimmune rheumatic diseases (ARDs). Methods: This study involved 82 patients with ARDs: 44 rheumatoid arthritis (RA), 28 systemic lupus erythematosus (SLE), and 10 systemic sclerosis (SSc) and 61 age- and sex-matched controls. Sociodemographic, clinical, and laboratory data were collected, and disease activity was assessed. Exposure to toxoplasmosis risk factors was investigated. Serological tests for anti-Toxoplasma IgM and IgG antibodies were assessed using ELISA. Results: In SLE patients, a significant difference of T. gondii IgM versus controls was detected (P=.03). In RA and SLE patients, T. gondii IgG showed a significant difference versus controls (34 (77.3%) P=.001 and 18 (64.3%) P=.03, respectively). There was no significant difference in SSc versus controls. Fetal congenital anomalies displayed a significant difference in IgM seropositive compared to seronegative patients (P=.04). Cat exposure showed a significant difference between IgM and IgG seropositive versus seronegative patients (12 (80.0%) P=.02 and 34 (59.6) P=.04, respectively). There was no significant difference in seropositive patients regarding history of abortion, neuro-psychiatric manifestations, disease activity parameters (ESR, CRP), or different regimens of medications. Conclusion: Toxoplasma IgM seropositivity is associated with SLE patients. T. gondii IgG seropositivity is associated with both RA and SLE patients. However, Toxoplasma seropositivity had no association with SSc patients. An association between fetal congenital anomalies and IgM seropositivity was demonstrated. A linkage between cat exposure as a risk factor and toxoplasmosis was suggested among ARD patiants. Exploration of impact of toxoplasmosis on ARDs is a necessity through randomized controlled trials.(AU)

Propósito: Explorar la asociación entre Toxoplasma gondii y enfermedades reumáticas autoinmunes (ERA).Métodos: Este estudio involucró a 82 pacientes con ERA: 44 con artritis reumatoide (AR), 28 con lupus eritematoso sistémico (LES) y 10 con esclerosis sistémica (SSc); y 61 controles emparejados por edad y sexo. Se recopilaron datos sociodemográficos, clínicos y de laboratorio, y se evaluó la actividad de la enfermedad. Se indagó exposición a factores de riesgo de toxoplasmosis. Las pruebas serológicas de anticuerpos IgM e IgG antitoxoplasma se evaluaron mediante ELISA.Resultados: En pacientes con LES se detectó una diferencia significativa de T. gondii IgM vs. controles (p = 0,03). En pacientes con AR y LES, T. gondii IgG mostró una diferencia significativa frente a los controles (34 [77,3%] p = 0,001 y 18 [64,3%] p = 0,03, respectivamente). No hay diferencia significativa en SSc vs. controles. Las anomalías congénitas fetales mostraron una diferencia significativa en los pacientes seropositivos para IgM en comparación con los pacientes seronegativos (p = 0,04). La exposición a los gatos mostró una diferencia significativa entre los pacientes seropositivos para IgM e IgG frente a los seronegativos (12 [80%] p = 0,02 y 34 [59,6] p = 0,04, respectivamente). No hubo diferencias significativas en pacientes seropositivos con respecto a antecedentes de aborto, manifestaciones neuropsiquiátricas, parámetros de actividad de la enfermedad (ESR, CRP) o diferentes regímenes de medicamentos.(AU)