Rheumatology (Oxford, England)最新文献

{"title":"Myositis-specific autoantibodies and QTc changes by ECG in idiopathic inflammatory myopathies.","authors":"Sine Søndergaard Korsholm,&nbsp;Daniel C Andersson,&nbsp;John Bonde Knudsen,&nbsp;Maryam Dastmalchi,&nbsp;Axel C P Diederichsen,&nbsp;Oke Gerke,&nbsp;Nanna Witting,&nbsp;Søren Jacobsen,&nbsp;Redi Pecini,&nbsp;Tina Friis,&nbsp;Markus E Krogager,&nbsp;Ingrid E Lundberg,&nbsp;Louise P Diederichsen","doi":"10.1093/rheumatology/keac013","DOIUrl":"https://doi.org/10.1093/rheumatology/keac013","url":null,"abstract":"<p><strong>Objectives: </strong>The aim of this study was to investigate cardiac involvement detected by ECG in patients with idiopathic inflammatory myopathies (IIMs) and to evaluate possible associations between the autoantibody profile and ECG changes in these patients.</p><p><strong>Methods: </strong>In a Scandinavian cross-sectional study, patients were included from two Danish centres and one Swedish centre. Resting 12-lead ECG was investigated in 261 patients with IIM compared with 102 patients with systemic sclerosis (SSc) and 48 healthy controls (HCs). ECG changes were correlated to clinical manifestations and myositis-specific and myositis-associated autoantibodies (MSAs and MAAs, respectively).</p><p><strong>Results: </strong>Patients with IIM had a longer mean corrected QT (QTc) duration and more frequently presented with prolonged QTc (≥450 ms; P = 0.038) compared with HCs. A longer QTc duration was recorded in SSc compared with IIM [433 ms (s.d. 23) vs 426 (24); P = 0.011], yet there was no significant difference in the fraction with prolonged QTc (SSc: 22%, IIM: 16%; P = 0.19). In multivariable regression analyses, anti-Mi2 (P = 0.01, P = 0.035) and anti-Pl-7 (P = 0.045, P = 0.014) were associated with QTc duration and prolonged QTc in IIM. Elevated CRP was associated with prolonged QTc (P = 0.041).</p><p><strong>Conclusion: </strong>The presence of QTc abnormalities was as common in patients with IIM as in patients with SSc, including prolonged QTc seen in almost one-fifth of the patients. Anti-Mi2, anti-Pl-7 and elevated CRP may serve as biomarkers for cardiac disease in IIM, but needs to be confirmed in a larger prospective study.</p>","PeriodicalId":379078,"journal":{"name":"Rheumatology (Oxford, England)","volume":" ","pages":"4076-4086"},"PeriodicalIF":5.5,"publicationDate":"2022-10-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39697244","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"34 Subtype frequencies and demographic characteristics of juvenile idiopathic arthritis in Batna, Algeria","authors":"D. Hadef, S. Slimani, M. C. Khamari, W. Mekaoussi, A. Belot, P. Quartier","doi":"10.1093/rheumatology/keac496.030","DOIUrl":"https://doi.org/10.1093/rheumatology/keac496.030","url":null,"abstract":"Abstract Background Juvenile idiopathic arthritis (JIA) is the most common chronic rheumatic disease in childhood. There is a disparity in the prevalence of Juvenile idiopathic arthritis (JIA) subsets between different geographical areas or ethnic groups. In Arabic and African populations, data describing JIA are scarce. However, the epidemiological studies remain the best tool to understand the disease and to improve its management. Objectives To determine subtype, frequency, demographic and clinical features of JIA in Batna -Algeria- and to compare the findings with other JIA populations worldwide. Methods A multicentre retrospective descriptive study was conducted in Batna health centers (public and private sectors), over a seven-year period from January 2013 to December 2019, based on data collected on JIA patients. As public sector source, we referred to the department of pediatrics of the university hospital center (CHU Benflis Touhami Batna), and as private sector source, we referred to private adult rheumatologists based in Batna. The studied variables were: gender, age at the initial symptoms, age at diagnosis, JIA subtype based on International League of Associations for Rheumatology (ILAR) criteria, symptoms at onset, disease duration at the latest follow up, presence of uveitis, auto antibodies (antinuclear antibodies, Rheumatoid Factor and anti-CCP) pattern, joint imaging results, JIA status at the time of enrolment and the latest follow-up. Results The study included a total of 69 cases of JIA that were being followed in Batna health centers over the study period. The female to male ratio was 1.83. The median age at diagnosis was 9 years (range 1–16). Forty-six patients (72%) were diagnosed within the first year after disease onset. At the latest follow-up, the median disease duration onset was 1 year (range 1–8 years). There were 34 oligoarthritis (49.3%), 9(13%) rheumatoid factor (RF) negative polyarticular JIA, 8(11.6%) RF positive polyarticular JIA, 6(8.7%), systemic arthritis, 6(8.7%) enthesitis-related arthritis, 3(4.3%) psoriatic arthritis and 3(4.3%) undifferentiated arthritis. Nine patients (18.7%) were anti-nuclear antibody (ANA) positive, and 21 patients (30.4%) had indeterminate ANA status. Conclusion Oligoarthritis was the most common JIA subtype in our study. The RF positive polyarthritis frequency was higher than in literature. Prospective multicentre studies are necessary to better identify the JIA peculiarities in our country. Disclosure of Interest None declared","PeriodicalId":379078,"journal":{"name":"Rheumatology (Oxford, England)","volume":"86 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2022-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"127134585","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"84 Methotrexate toxicity in a patient with Takayasu arteritis; a case report","authors":"Athul Kooliyath, A. Migowa","doi":"10.1093/rheumatology/keac496.080","DOIUrl":"https://doi.org/10.1093/rheumatology/keac496.080","url":null,"abstract":"Abstract Background Methotrexate is an antimetabolite commonly used as a chemotherapeutic and as a steroid sparing agent in multiple rheumatologic conditions. The common side effects are well documented and usually seen in patients receiving high dose therapy and hence more commonly seen in oncology. Here, we discuss a case on methotrexate toxicity in a patient being treated for Takayasu arteritis. Methods This was a retrospective case review Results A seventeen-year-old was seen in the rheumatology service with a diagnosis of Takayasu arteritis after being initially worked up due to hypertension. Methotrexate therapy was started at an initial dose of 25 mg to be taken weekly. She was later seen in the pediatric out-patient unit 10 days post initiation of therapy with a history of inability to feed due to oral sores and severe abdominal pain. She also complained of odynophagia and hence had poor oral intake. On further history, it was noted that the patient was taking the medication on a daily basis as opposed to the prescribed weekly regimen. On exam, she was noted to have extensive ulceration of her buccal mucosa, gingiva and tongue. She was also found to have severe epigastric tenderness. Her genital regions were however spared. A diagnosis of methotrexate toxicity was made and she was admitted to the high dependency unit for management. Methotrexate was immediately discontinued and a hyper hydration regimen was initiated with intravenous fluids (125 ml/m2/h). Her pain was managed with an intravenous morphine infusion. Particular care was taken to avoid drugs such as proton pump inhibitors (PPIs), non-steroidal anti-inflammatory drugs (NSAIDs) and Sulphur containing drugs. A rescue was initiated with 15 mg/m2 of folinic acid given 6 hourly. The patient made a full recovery and was discharged 4 days later. Discussion and Conclusions Methotrexate is a drug with varied and severe adverse effects. Toxicity with methotrexate affects multiple systems and can cause end organ damage. It is therefore of paramount importance to recognize toxicity early and manage appropriately before onset of end organ damage. The above patient was risk of hepatotoxicity, renal toxicity, Neurologic toxicity, methotrexate induced lung injury, hematologic toxicity and hypersensitivity. She developed mucositis as a result of the medication. Enhancing renal elimination of the drug is at the core of management. This can be done with hyperhydration, alkalinisation of urine and avoiding drugs that slow elimination of methotrexate such as PPIs, NSAIDs, tyrosine kinase inhibitors, sulfa drugs and penicillin like drugs. Once end organ damage is established, therapy is supportive and highly dependent on the organ systems involved and the extent of injury. It is necessary for clinicians to be well versed with the adverse effects expected with methotrexate as evidenced by this case for prompt diagnosis and sound management. It is also vital to counsel patients and parents about the ","PeriodicalId":379078,"journal":{"name":"Rheumatology (Oxford, England)","volume":"29 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2022-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"127316212","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"004 Systemic autoinflammatory diseases: a multicenter cohort study","authors":"Ayşe Tanatar, Özlem Akgün, Ş. Çağlayan, S. Özdel, G. O. Yener, M. Çakan, Kübra Öztürk, H. E. Sönmez, B. Sözeri, N. Ayaz","doi":"10.1093/rheumatology/keac495.004","DOIUrl":"https://doi.org/10.1093/rheumatology/keac495.004","url":null,"abstract":"Abstract Background Systemic autoinflammatory diseases (SAIDs) are a group of hereditary diseases characterized by inflammatory attacks accompanied by clinical findings such as fever, skin rash, abdominal pain, and musculoskeletal symptoms and with high acute phase response. Objectives This study sought to define the characteristics, to report 24-month follow-up of children with SAIDs and to compare the performances of both the previously validated classification criteria Federici and new Eurofever/PRINTO classification criteria in a multicentre cohort under real-life conditions. Method The medical charts of 60 children diagnosed with SAIDs (CAPS, TRAPS and HIDS) who were followed up regularly every 1–3 months between 2010 and 2021 in 6 pediatric rheumatology centers in Turkey were retrospectively analyzed. Demographic data, clinical findings, laboratory results and treatments were recorded. All patients were analyzed by NGS panel containing 16 genes. Results A total of 60 patients with SAID were evaluated. Among them 30 (50%) were male and 30 (50%) were female. The median age at disease onset and diagnosis were 22 (0–193) months and 72 (2–213) months, respectively. The current age was median 10.3 (1.7–20.4) years. All patients were follow-up with a median 31 (6–97) months. Among 60 patients, 22 were diagnosed with CAPS, 8 with TRAPS and 30 with HIDS. The frequency of clinical symptoms was 95%, arthralgia 63.3%, gastrointestinal complaints 58.3%, urticarial rash 56.7%, myalgia 48.3%, lymphadenopathy 33.3%, respectively. Urticarial rash (86.4%), cold induced rash (45.5%) and arthritis (50%) were significantly higher in CAPS patients compared with other two groups (p< 0.05). Lymphadenopathy (46.7%) was more common in HIDS patients (p = 0.03). When we applied the diagnostic/classification criteria to our cohort, 71.7% met the Federici criteria, 53.3% fulfilled the Eurofever/PRINTO classification criteria. When performance of two diagnostic criteria (Federici and Eurofever/PRINTO criteria) was compared, the proportion of patients fulfilling the Eurofever/PRINTO criteria higher in TRAPS patients compared with other groups (p = 0.008). Statistically significant decreases were found in AIDAI scores, CRP, ESR levels from the 1st month and in the leucocyte counts from the 6th month of the treatment in CAPS patients (p = <0.001, p = 0.035, p = 0.03 and p = 0.006, respectively). In addition, AIDAI scores were correlated with CRP values (p = 0.047). In HIDS patients, there was a statistically significant decrease in AIDAI scores and ESR levels from the 1st month, CRP levels from the 3rd month, and leucocyte counts from the 12th month of treatment (p = <0.001, p = 0.003, p = <0.001 and p = 0.034, respectively). In addition, ESR values were correlated with CRP (p = 0.006). In TRAPS patients, on the other hand, there was a statistically significant decrease in AIDAI scores from the 1st month of treatment (p = 0.018). At the last visit, 93.3% of the patients wer","PeriodicalId":379078,"journal":{"name":"Rheumatology (Oxford, England)","volume":"62 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2022-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"115513483","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"50 The spectrum of pediatric lupus: data from the Kenya Pediatric Rheumatology Registry (KAPRI)","authors":"Zayani Seif, Akid Alaa, Hadj Salem Radhia, Chouchane Chokri, Thabet Farah, A. Migowa, J. Orwa","doi":"10.1093/rheumatology/keac496.046","DOIUrl":"https://doi.org/10.1093/rheumatology/keac496.046","url":null,"abstract":"Abstract Background Pediatric rheumatic diseases are chronic illnesses that impart a significant disease burden upon children and their families (1–3). Furthermore, they have the potential to cause physical disability, diminished quality of life and significant direct and indirect costs (1–3). Determination of the burden and clinical characteristics of pediatric lupus is a critical first step to improving access to care and optimizing use of existing health systems for the well-being of these patients (4). Childhood-onset systemic lupus erythematosus (cSLE) is a prototype autoimmune condition characterized by simultaneous or sequential organ and system involvement, with unpredictable flare and high levels of morbidity and mortality (5). A pediatric rheumatology registry is critical in defining the spectrum of pediatric lupus within the region, the clinical characteristics of the disease, the outcomes and responses to various interventions. The Kenya Pediatric Rheumatology Registry (KAPRI) registry offers a unique opportunity to pioneer and spearhead a systematic and organized format in collecting pertinent clinical information that will offer information on current clinical scenarios and offer a platform to conduct other research projects to improve pediatric lupus healthcare in sub-Sahara Africa. Our objective was to determine the clinic-epidemiological profile of paediatric lupus patients by describing their baseline patient characteristics and clinical features at the Aga Khan University Medical College East Africa who were enrolled into the KAPRI registry from inception in March 2019 to December 2021. Methods All patient records were selected from the KAPRI registry database using the ICD 10 code M32 that denotes Systemic Lupus Erythematosus (SLE) Age, gender, laboratory, clinical features at diagnosis, treatment options offered at time of diagnosis were extracted from the database. Results Among the 207 patients enrolled thus far in the registry, 7 had a diagnosis of cSLE (3.4%). The commonest symptom among the patients were joint pain, fever and rash. Other clinical features and outcomes are highlighted in the table 1 below; Conclusion Over a 2-year period, 57% of our cSLE patients (4 of 7) achieved remission while 43% (3of 7) were lost to follow up. Further studies are required to elucidate predictors of good clinical response and reasons for loss to follow up among our cSLE patients. References 1. Brunner, H., Huggins J and Klein-Gitelman MS. Pediatric SLE—towards a comprehensive Management plan Nat. Rev. Rheumatol. 7, 225–233 (2011). 2. Kamphuis, S. and Silverman, E. D. Prevalence and burden of pediatric-onset systemic lupus erythematosus Nat. Rev. Rheumatol. 6, 538–546 (2010). 3. Migowa A, Colmegna I, Hitchon C, Were E, Ng’ang’a E, Ngwiri T, et al. The spectrum of rheumatic in-patient diagnoses at a pediatric hospital in Kenya. Pediatric Rheumatology (2017) 4. Trindade, V.C., Carneiro-Sampaio, M., Bonfa, E. et al. An Update on the Manag","PeriodicalId":379078,"journal":{"name":"Rheumatology (Oxford, England)","volume":"23 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2022-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"124750820","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"85 Systemic lupus erythematosus in Tunisian children: a case series","authors":"H. Rouag, S. Zayani, A. Akid, T. Ghedira, E. Sfar, R. Hadj salem, C. Chouchane, F. Thabet, S. Chouchane","doi":"10.1093/rheumatology/keac496.081","DOIUrl":"https://doi.org/10.1093/rheumatology/keac496.081","url":null,"abstract":"Abstract Background Childhood–onset systemic lupus erythematosus (c-SLE) is the prototype of a multisystem, inflammatory, heterogeneous autoimmune condition, characterized by simultaneous or sequential organ involvement. Compared with the adult-onset form, c-SLE have a worse prognosis. Objectives To review the epidemiological, and bio-clinical, characteristics of a c-SLE case series. Methods The files of patients diagnosed as c-SLE in the pediatrics department of Monastir, Tunisia from January 2004 to March 2022 were reviewed. Mean and standard-deviation were used to express normally-distributed variables, as verified by the Kolmogorov-Smirnov statistical test. Results Fourteen patients were collected. Female to male ratio was 6:1. Mean ages at lupus onset and diagnosis were 9.9 ± 1.4 years, [5–13.8 years] and 10.75 ± 2.3 years [6–14 years], respectively. Only two children had a family history of autoimmune disease. The initial admission was motivated primarily by skin and musculoskeletal manifestations, in 64.3% and 51.7% of cases, respectively. General signs (fever, asthenia) were observed in 35.7% of cases, hematological and gastrointestinal manifestations in 28.6% of cases each. In 3 cases, upper gastric endoscopy was performed prior to admission, in view of abdominal pain and vomiting. The physical examination noted various abnormalities. Malar rash (50%) and discoid lupus (28.6%) were the most frequent cutaneous manifestations, while skin biopsy was performed in three cases, all in keeping with lupus. The musculoskeletal manifestations were arthralgia (71.4%), arthritis and myositis (14.3%). Hematological manifestations included thrombocytopenia and leukopenia in 4 cases, as well as 3 cases of auto-immune hemolytic anaemia and splenomegaly. Renal manifestations were proteinuria in 7, haematuria in 6, and hypertension in 2 (with renal failure in one of the patients). The renal biopsy that was performed in one subject showed a class 2 lupus nephritis. Pleural effusion was observed in 3, pneumonia in 3, pericarditis in 2, myo-pericarditis in 1 and central nervous system (CNS) lupus in 1. Relevant results of the laboratory workup are illustrated in the following table:85 Table 1 Laboratory test Performed (%) Abnormal results (%) Erythrocyte sedimentation rate 14 (100%) 13 (92%) Antinuclear anti-bodies 14 (100%) 13 (92%) AntiDNA antibodies 7 (50%) 5 (71%) AntiSm antibodies 8 (57%) 5 (62%) Antiphospholipid antibodies 8 (57%) 5 (62%) Complement 11 (78%) 9 (81%) The formal diagnosis of SLE was established according to the ACR-1997 criteria in 7 cases (50%), the SLICC-2012 in 4 cases (28.6%) and EULAR/ACR-2019 in 3 cases (21.4%). The c-SLE diagnosis was associated with coeliac disease and Hashimoto thyroiditis in two of the subjects respectively. The therapeutic management was based on corticosteroids in 11 cases, hydroxychloroquine in 3, while cyclophosphamides and immunoglobulin were used for two subjects respectively. The outcomes were heterogene","PeriodicalId":379078,"journal":{"name":"Rheumatology (Oxford, England)","volume":"4 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2022-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"129434064","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"25 Impact of early feeding on the occurrence of juvenile idiopathic arthritis","authors":"E. Rabhi, W. Triki, H. Ferjani, D. B. Nessib, K. Maatallah, D. Kaffel, W. Hamdi","doi":"10.1093/rheumatology/keac496.021","DOIUrl":"https://doi.org/10.1093/rheumatology/keac496.021","url":null,"abstract":"Abstract Background Juvenile idiopathic arthritis (JIA) is considered to be an autoimmune disease, which is a result of an immune reaction caused or triggered by environmental factors in a genetically susceptible individual. However, interactions between early feeding and risk Juvenile idiopathic arthritis (JIA) remains largely unexplored. Objectives To determine the impact of early alimentation on the occurrence of JIA in Tunisia. Methods We conducted a case-control study including 26 children with JIA fulfilling the international league against rheumatism (ILAR) 2010 criteria compared with a homogeneous control group of 21 children hospitalized in a paediatric orthopedic unit for traumatic reasons and healthy for any chronic inflammatory rheumatism. Parents of patients were asked after having their consent to participate. We collected the following data: Type of JIA, juvenile disease activity score (JADAS), type of feeding, total duration of breastfeeding, duration of exclusive breastfeeding and age at introduction of gluten. Patients were divided into two groups: group 1 (G1): JIA patients, group 2 (G2): control group. Data were analyzed using the SPSS statistical package. A p-value < 0.05 was considered significant. Results Forty-seven children (26 diagnosed with JIA and 21 controls) with a mean age of 10.07 years ± 4.96 were enrolled. JIA subtypes were in decreasing order of frequency: oligoarticular (45.8%), enthesitis-related arthritis (20.8%), undifferentiated JIA (12.5%), seronegative polyarticular JIA (8.3%), juvenile psoriatic arthritis (8.3%), and seropositive polyarticular JIA (4.2%). The mean JADAS was 6.966 ± 5.38. There was no significant difference between the G1 and G2 with regard to the duration of breastfeeding (12.23 ± 3.8 months vs 8.14 ± 4.1 months, p = 0.09), exclusive breastfeeding (3.8 ± 3.6 vs 4.1 ± 2.62, p = 0.40) and age at introduction of gluten (G1: 8.48 ± 5.30 months vs G2: 7.48 ± 1,2 months, p = 0.35) Conclusion Our study didn’t show any impact of early alimentation on the occurrence of JIA in Tunisia. However, more studies are necessary to conclude these issues.","PeriodicalId":379078,"journal":{"name":"Rheumatology (Oxford, England)","volume":"23 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2022-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"129890494","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"61 The nailfold videocapillaroscopy in pediatric Behçet’s disease: a multi-center study","authors":"F. Çakmak, Şeyda Doğantan, Rana İşgüder, Müşerref Kasap Cüceoğlu, Ş. Çağlayan, H. E. Sönmez, Özlem Akgün, B. Sözeri, A. Kısaarslan, E. Ünsal, S. Özen, N. Ayaz","doi":"10.1093/rheumatology/keac496.057","DOIUrl":"https://doi.org/10.1093/rheumatology/keac496.057","url":null,"abstract":"Abstract Background Behçet's disease (BD) is a chronic inflammatory disease characterized by recurrent oral aphthous and genital ulcers accompanied by eye, joint, skin, gastrointestinal and central nervous system involvement. The vascular involvement may affect both the arterial and venous systems. Nailfold videocapillaroscopy (NVC) is an easy and non-invasive method used in the evaluation of microcirculation. With this study, we aimed to find the characteristics and prevalence of nailfold capillary alterations in patients with juvenile BD and to analyze their possible relationship between clinical characteristics and activity of the disease. Methods Patients aged 5–21 years with a diagnosis of juvenile BD and followed up for at least six months were included in the study. Demographic and clinical characteristics of the patients were recorded. NVC was performed on 8 fingers of both hands, excluding the thumbs, and four consecutive non overlapping fields for each of fingers were evaluated (32 fields per patient). Capillary density, capillary width (arterial width, venous width, apical loop), capillary morphology and the presence of meandering capillary, micro hemorrhage, avascular area, neoangiogenesis, capillary ramification were evaluated from the images. Capillary morphology were evaluated by classifying them into four groups as normal, minor abnormalities, major abnormalities and scleroderma pattern. The presence of abnormilities in at least two fingers were recorded as capillary abnormality. The semi quantitative rating score 1–3 was applied for each capillaroscopic alteration. Results 37 patients from 6 pediatric rheumatology centers were included in the study. The mean age of patients was 17 years (IOR 13–19) and 20 (54.1%) of them were girls. The patients were evaluated in four clusters according to their clinical presentations. Nineteen patients had mucocutaneous involvement, 9 patients had uveitis, 8 patients had vascular and neurological involvement, and 4 patients had gastrointestinal system involvement. During the follow-up period, genital ulcers developed in 22 patients, erythema nodosum in 9 patients, pseudofolliculitis in 18 patients, uveitis in 10 patients, vascular involvement in 8 patients, and neurological involvement in 5 patients. Anterior uveitis was present in five, posterior uveitis in three, panuveitis in one, and retinal vasculitis in three of the patients with ocular involvement. Four patients had lower extremity venous thrombosis, three patients had central nervous system (CNS) thrombosis, and one patient had both lower extremity and CNS thrombosis. When capillary morphology was evaluated; normal morphology was present in 16 patients, minor abnormality in 13 patients, and major abnormality in 8 patients. Median capillary density was 8, capillary length was 325 µm, arterial width was 12 µm, venous width was 16 µm, apical loop width was 18 µm, capillary width was 39 µm, and intercapillary distance was 107 µm. Neoagiogene","PeriodicalId":379078,"journal":{"name":"Rheumatology (Oxford, England)","volume":"78 22","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2022-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"113944409","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"49 Autoimmune hemolytic anemia revealing a familial systemic lupus erythematosus","authors":"Rouag Hatem, Zayani Seif, Akid Alaa, Sfar Emna, Ghedira Tesnime, Hadj Salem Radhia, Chouchane Chokri, Thabet Farah, Chouchane Slah","doi":"10.1093/rheumatology/keac496.045","DOIUrl":"https://doi.org/10.1093/rheumatology/keac496.045","url":null,"abstract":"Abstract Background Systemic lupus erythematosus (SLE) is a heterogeneous autoimmune disease; whose pathogenesis remains poorly understood. The genetic susceptibility to lupus is supported by the high concordance rate of the disease in monozygotic twins (29–57%) compared with dizygotic twins (2%), and by the existence of familial lupus (10–12%). Furthermore, SLE shares genetic and environmental risk factors, as well as pathophysiologic mechanisms with autoimmune hemolytic anaemia (AIHA), which is clearly over-represented in patients with SLE and often occurs before the diagnosis of the underlying disease. Objective We report a case of familial SLE in two siblings, in whom, the main symptom was AIHA. Case report A twelve-year-old girl was referred to our Pediatric department for polyarthralgia, asthenia and anorexia developing over the previous 4–6 months. Regarding family history, she is born of a non-consanguineous marriage and has a 7-year-old brother who has been followed for 2 years for AIHA of undetermined aetiology. The examination upon admission objectified a splenomegaly and jaundice. The laboratory workup revealed an autoimmune hemolytic anaemia: haemoglobin: 7.2 g/dl, positive Coombs' test with specific anti globulin IgG and C3d; unconjugated bilirubin (45 µmol/l) and reduced haptoglobin (0.01 g/l). These findings being very evocative of SLE, an antinuclear antibody (AAN) assay was positive at 1/400. The formal SLE diagnosis was established according to the EULAR/ACR 2019 criteria; with a score of 12 (AIHA: 4; positive antiphospholipid antibodies: 2; positive anti-Sm antibodies: 6). The therapeutic management based on corticosteroids at a dose of 1 mg/kg/day, with a favorable clinical and biological outcome. In light of this diagnosis, the case of the aforementioned brother was reviewed, and a familial lupus was suspected. A physical examination and a several biological explorations were done, the results of which allowing to retain the diagnosis of SLE with EULAR/ACR 2019 score of 10 (ANA > 1/80, AIHA : 4, positive anti-phospholipid antibodies: 2, proteinuria: 4) Conclusion Since the new criteria EULAR/ACR 2019 have made it possible to diagnose incomplete forms of lupus, the challenge remains to detect autoimmune thyroiditis, antiphospholipid syndrome, complement deficiencies and other entities that are associated with familial lupus according to scientific literature.","PeriodicalId":379078,"journal":{"name":"Rheumatology (Oxford, England)","volume":"10 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2022-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"127832978","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"71 Estimation of incidence of MIS-C in Cape Town, South Africa","authors":"Claire Butters, Deepthi Abrahams, R. Stander, Timothy Spraklen, Heidi E. Facey-Thomas, L. Zühlke, H. Rabie, C. Scott, K. Webb","doi":"10.1093/rheumatology/keac496.067","DOIUrl":"https://doi.org/10.1093/rheumatology/keac496.067","url":null,"abstract":"Abstract Background Multisystem inflammatory syndrome is a severe manifestation of SARS-CoV-2 in children. The incidence of MIS-C after SARS-CoV-2 infection is poorly understood. There are very few cohorts describing MIS-C in Africa despite MIS-C being more common in Black children worldwide. Methods A cohort of children with MIS-C and healthy children was recruited from May 2020 to May 2021 from the two main paediatric hospitals in Cape Town, South Africa. Clinical and demographic data were collected, and serum was tested for SARS-CoV-2 antibodies. The incidence of MIS-C was calculated using an estimation of population exposure from seroprevalence in the healthy group. Summary data, non-parametric comparisons and logistic regression analyses were performed. Results Sixty-eight children with MIS-C were recruited with a median age of 7 years and 97 healthy children were recruited with a 30% seroprevalence. The estimated incidence of MIS-C was 22/100 000 SARS-COV-2 infections in children under 14 years old in the city at that time. Black children were over-represented in the MIS-C group (62% vs 37%, p = 0.002). The most common clinical features in MIS-C were fever (100%), tachycardia (98.5%), rash (85.3%), conjunctivitis (77.9%), abdominal pain (60.3%) and hypotension (60.3%). Median levels of haemoglobin, sodium, CRP, ferritin, cardiac (pro-BNP, trop-T) and coagulation markers (D-dimer and fibrinogen), neutrophil and white cell count were markedly deranged in MIS-C. Cardiac, pulmonary, central nervous and renal organ systems were involved in 71%, 29.4%, 27.9% and 27.9% respectively. Ninety-four point one per cent patient received intravenous immune globulin, 64.7% received methylprednisolone and 61.7% received both. ICU admission was required in 39.7% patient while 38.2% required inotropic support, 38.2% required oxygen therapy, 11.8% required invasive ventilation and 6% required peritoneal dialysis. The median hospital stay duration was 7 days with no deaths. Conclusion The lack of reports from Southern Africa does not reflect a lack of cases of MIS-C. The clinical manifestations and outcomes of MIS-C in this region highlight the need for improved surveillance, reporting and data to inform diagnosis and treatment. Implications To our knowledge, these are the first data on MIS-C in Africa. This shows that children in Africa are indeed presenting with MIS-C which will increase surveillance around the continent.","PeriodicalId":379078,"journal":{"name":"Rheumatology (Oxford, England)","volume":"5 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2022-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"125571355","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}