Intermittent abdominal pain in IgA vasculitis

ABSTRACT Objective: To assess intermittent abdominal pain in IgA vasculitis patients and its relation to demographic data, clinical manifestations and treatments. Methods: A retrospective cohort study included 322 patients with IgA vasculitis (EULAR/PRINTO/PRES criteria) seen at the Pediatric Rheumatology Unit in the last 32 years. Sixteen patients were excluded due to incomplete data in medical charts. Intermittent abdominal pain was characterized by new abdominal pain after complete resolution in the first month of disease. Results: Intermittent abdominal pain was observed in 35/306 (11%) IgA vasculitis patients. The median time between first and second abdominal pain was 10 days (3–30 days). The main treatment of intermittent abdominal pain included glucocorticoid [n=26/35 (74%)] and/or ranitidine [n=22/35 (63%)]. Additional analysis showed that the frequency of intermittent purpura/petechiae (37 vs. 21%; p=0.027) and the median of purpura/petechiae duration [20 (3–90) vs. 14 (1–270) days; p=0.014] were significantly higher in IgA vasculitis patients with intermittent abdominal pain compared to those without. Gastrointestinal bleeding (49 vs. 13%; p<0.001), nephritis (71 vs. 45%; p=0.006), glucocorticoid (74 vs. 44%; p=0.001) and intravenous immunoglobulin use (6 vs. 0%; p=0.036) were also significantly higher in the former group. The frequency of ranitidine use was significantly higher in IgA vasculitis patients with intermittent abdominal pain versus without (63 vs. 28%; p<0.001), whereas the median of ranitidine duration was reduced in the former group [35 (2–90) vs. 60 (5–425) days; p=0.004]. Conclusions: Intermittent abdominal pain occurred in nearly a tenth of IgA vasculitis patients, in the first 30 days of disease, and was associated with other severe clinical features. Therefore, this study suggests that these patients should be followed strictly with clinical and laboratorial assessment, particularly during the first month of disease course.

Sonographic Tophi and Inflammation Are Associated With Carotid Atheroma Plaques in Gout

Objective: Gout and cardiovascular disease are closely related, but the mechanism connecting them remains unknown. This study aims to explore whether urate crystal deposits and inflammation (assessed by ultrasound) are associated with carotid atherosclerosis.Methods: We included consecutive patients with crystal-proven gout newly presenting to a tertiary rheumatology unit. Patients under urate-lowering treatment were excluded. Ultrasound assessment was performed during intercritical periods. Musculoskeletal scans evaluated six joints and four tendons for urate crystal deposits (double contour, aggregates, and tophi), and power Doppler (PD) signal (graded 0–3) as a marker of local inflammation. The sum of locations showing deposits or a positive PD signal (≥1) was registered. Carotids were scanned for increased intima-media thickness (IMT) and atheroma plaques, according to the Mannheim consensus. Associations were analyzed using logistic regression.Results: The study included 103 patients showing sonographic crystal deposits at the examined locations (mean sum 9.9, minimum 2); tophi were the most frequent. Two-thirds of participants presented a positive PD signal (30.1% grade 2–3). In the carotid scans, 59.2% of participants showed atheroma plaques, and 33.0% increased IMT. Tophi (odds ratio [OR] 1.24; 95% confidence interval [CI] 1.03–1.50) and a positive PD signal (OR 1.67; 95% CI 1.09–2.56) were significantly associated with atheroma plaques, while an increased IMT showed no sonographic association.Conclusion: Sonographic crystal deposits and subclinical inflammation were consistently observed in patients with intercritical gout. Tophi and a positive PD signal were linked to carotid atherosclerosis. Our findings may contribute to understanding the complex relationship between gout and atherosclerosis.

The Influence of Rheumatoid Arthritis on Work Productivity among a Sample of Iraqi Patients

Background: Rheumatoid Arthritis (RA) is a chronic inflammatory disorder that impact daily life activities. The impact on work productivity is critical because of persistent work disability. Aim: To evaluate work productivity in RA patients compared to healthy controls, and to assess the impact of socio-demographic characteristics on work productivity. Methods: This was a case-control study conducted at the Rheumatology Unit of Baghdad Teaching Hospital, Medical City during the period from August 2020 to the end of March 2021. Seventy-two patients with RA were selected and compared with 72 healthy subjects as a control group. All patients were diagnosed according to ACR/EULAR 2010 RA classification criteria. The socio-demographic and clinical features, lifestyle practices, and disease activity of the patients and controls were all recorded during interviews. A standardized Arabic version of the Workplace Activity Limitation Scale (WALS) and the Job Limitations Questionnaire were used to assess the impact of RA on work productivity (WLQ-25). Results: The vast majority of patients were females (58.1%). Positive rheumatoid factor was reported in 94.4% of the patients. The patients showed a significantly lower WALS total score and higher WLQ-25 total score median (IQR) compared with controls. Conclusion: Active RA impairs the work productivity which was influenced by CDAI score and negatively associated with the use of DMARDs.

P034 Macrophage activation syndrome in Juvenile Idiopathic Arthritis

Objectives To describe the epidemiological, clinical-biological, and therapeutic characteristics of children with systemic JIA complicated with a macrophage activation syndrome (MAS). Methods It was a retrospective study, including children with JIA followed at the pediatric rheumatology unit of the children's hospital in Tunis for 22 years (January 1999 to December 2020), and presented MAS during their follow-up. Results We included 40 patients with JIA. Nineteen children (47.5%) presented MAS during the disease. They are 11 boys and 8 girls, with a sex ratio of 1.3. The mean age was 5.31 years (range: 0.66–10.83 years). The circumstances of the occurrence were variable. MAS was inaugural in 10 patients. Three of our patients presented MAS twice. MAS was definitive with clinical and biological markers in 12 cases and only biological in 7 cases. Seventeen of our patients were treated with intravenous Corticosteroids. Seven Childs among them received, in combination, Immunoglobulins (IG). We prescribed a biological treatment in 3 patients. We have mourned only one death. Conclusion MAS is a severe complication of JIA and is the leading cause of death.

Validation of algorithms for selecting rheumatoid arthritis patients in the Tuscan healthcare administrative databases

Validation of algorithms for selecting patients from healthcare administrative databases (HAD) is recommended. This PATHFINDER study section is aimed at testing algorithms to select rheumatoid arthritis (RA) patients from Tuscan HAD (THAD) and assessing RA diagnosis time interval between the medical chart date and that of THAD. A population was extracted from THAD. The information of the medical charts at the Rheumatology Unit of Pisa University Hospital represented the reference. We included first ever users of biologic disease modifying anti-rheumatic drugs (bDMARDs) between 2014 and 2016 (index date) with at least a specialist visit at the Rheumatology Unit of the Pisa University Hospital recorded from 2013 to the index date. Out of these, we tested four index tests (algorithms): (1) RA according to hospital discharge records or emergency department admissions (ICD-9 code, 714*); (2) RA according to exemption code from co-payment (006); (3) RA according to hospital discharge records or emergency department admissions AND RA according to exemption code from co-payment; (4) RA according to hospital discharge records or emergency department admissions OR RA according to exemption code from co-payment. We estimated sensitivity, specificity, positive and negative predicted values (PPV and NPV) with 95% confidence interval (95% CI) and the RA diagnosis median time interval (interquartile range, IQR). Two sensitivity analyses were performed. Among 277 reference patients, 103 had RA. The fourth algorithm identified 96 true RA patients, PPV 0.78 (95% CI 0.70–0.85), sensitivity 0.93 (95% CI 0.86–0.97), specificity 0.84 (95% CI 0.78–0.90), and NPV 0.95 (95% CI 0.91–0.98). The sensitivity analyses confirmed performance. The time measured between the actual RA diagnosis date recorded in medical charts and that assumed in THAD was 2.2 years (IQR 0.5–8.4). In conclusion, this validation showed the fourth algorithm as the best. The time interval elapsed between the actual RA diagnosis date in medical charts and that extrapolated from THAD has to be considered in the design of future studies.

Skin biopsy in Paediatric Rheumatology: 10 years review of practice.

BACKGROUNDSkin biopsy has limited but sometimes distinctly useful place in diagnosis of childhood rheumatologic disorders. For example, skin biopsy is of little value in differentiating lupus from dermatomyositis. The purpose of skin biopsy is to support diagnosis, disease monitoring (e.g.scleroderma) or treatment (e.g., skin cancer). OBJECTIVETo evaluate skin biopsy practices in a tertiary paediatric rheumatology unit in order to determine the usefulness. METHODA retrospective review of medical records of paediatric rheumatology patients who have had skin biopsy over a 10-year period between 2008 and 2018 at Alder Hey Children Hospital.RESULTS &amp; DISCUSSIONA total of 49 skin biopsies with an average of 5 per year was performed. Patients age range from 0.75 -18 years with mean of 10 years. Sites of biopsy and main diagnoses are as shown in figure 1 &amp; 2. Fifty nine percent had excision biopsy while 41% had punch biopsy. Majority of skin biopsies (94%) were performed to aid diagnosis. Severe HSP was the most common indi?cation for skin biopsies requested by rheumatologist. A high proportion of the biopsies (84%) were helpful in the patient care; 55% confirmed clinical suspicions, 25% excluded. CONCLUSION &amp; PERSPECTIVESRheumatologist are selective in the use of skin biopsy for diagnosis. When indicated, skin biopsy is often helpful in the diagnosis and monitoring of rheumatologic disorders. It must be ensured that sufficient information with clear clinico-pathologic question is provided when requesting skin biopsy to enhance positive result.

POS1137 IDENTIFYING ADULT HYPOPHOSPHATASIA IN THE RHEUMATOLOGY UNIT

Background:The most frequent manifestation in adult Hypophosphatasia (HPP) is musculoskeletal pain.1,2 The unspecific nature of its clinical presentation may prevent correct diagnosis.3Objectives:Identifying adult hypophosphatasia in the rheumatology unit.Methods:Over a period of 10 years 9,522 patients were screened in a rheumatological outpatient unit. Serum ALP levels ≤ 40 U/l were found in 524 patients. After screening for secondary causes, 73 patients were invited for clinical evaluation. Genetic testing was performed in 23 patients with suspected HPP. Logistic regression models were used to estimate the association of each clinical factor with HPP.Results:Mutations in the ALPL gene were observed in 57% of genetically screened patients. Arthralgia, fractures and pain were the leading symptoms in HPP patients. Chondrocalcinosis (OR 29.12; 95% CI 2.02-1593.52) and dental disease (OR 8.33; 95% CI 0.93-143.40) were associated with HPP independent of BMI. Onset of symptoms in HPP was at 35.1 (14.3) years, with a mean duration from symptoms to diagnosis of 14.4 (8.1) years. Bone mineral density (BMD) and trabecular bone score (TBS) as well as bone turnover markers were not indicative for HPP.Conclusion:HPP can mimic joint diseases.4 Thus, in patients with uncertain rheumatologic complaints and low ALP, HPP should be considered as potential diagnosis.References:[1]Durrough C, Colazo JM, Simmons J, et al. Characterization of physical, functional, and cognitive performance in 15 adults with hypophosphatasia. Bone 2021;142:115695.[2]Seefried L, Kishnani PS, Moseley S, et al. Pharmacodynamics of asfotase alfa in adults with pediatric-onset hypophosphatasia. Bone 2021;142:115664.[3]Högler W, Langman C, Gomes da Silva H, et al. Diagnostic delay is common among patients with hypophosphatasia: initial findings from a longitudinal, prospective, global registry. BMC musculoskeletal disorders 2019;20(1):80.[4]Seefried L, Dahir K, Petryk A, et al. Burden of Illness in Adults With Hypophosphatasia: Data From the Global Hypophosphatasia Patient Registry. Journal of bone and mineral research: the official journal of the American Society for Bone and Mineral Research 2020;35(11):2171-78.Disclosure of Interests:None declared.

AB0143 THE EFFECT OF RHEUMATOID ARTHRITIS AND SPONDILOARTHRITIS ON PREGNANCY: A LONGITUDINAL RETROSPECTIVE COHORT STUDY

Background:Many patients with rheumatoid arthritis (RA) and spondyloarthritis (SpA) are in their childbearing years. Concerns exist regarding the interplay between the rheumatic diseases and the pregnancy (1).Objectives:Actually, there are contradictory data regarding the pregnancy outcome in patients with RA and SpA (2). Thus, we performed this longitudinal retrospective study to evaluate the effect of RA and SpA on pregnancy outcome.Methods:The data of 78 pregnancies of 60 women followed from April 2017 to December 2020 at pregnancy clinic of Internal Medicine Unit, San Bortolo Hospital, Vicenza and Rheumatology Unit, University of Verona were reviewed. Fifty (64.1%) women were affected by RA and 28 (35.9%) by SpA. Information regarding demographic data, disease activity, drug exposure and maternal/foetal outcomes were collected in an electronic database. Details concerning pregnancy complications and congenital malformation were also collected. We compared pregnancy and foetal/neonatal outcome, medication use and disease activity between women affected by RA and SpA. Moreover, we evaluated the effect of disease activity on pregnancy outcome.Results:Overall, there were 70 (86.4%) live births, 10 (12.3%) miscarriages and 1 (1.2%) foetal death. There were three twin pregnancies. Even there was a higher rate of glucocorticoids and bDMARDs use in RA than in SpA group, respectively 40% vs 21% and 70% vs 57,1%, there were no statistical differences regarding drug exposure at conception. Moreover, there were no differences concerning disease activity at conception. Still, a higher rate of glucocorticoids and bDMARDs, respectively 26% vs 10.7% and 46% vs 39.3% were used in RA than in SpA patients during pregnancy. Furthermore, we did not find any statistical differences regarding maternal and foetal/neonatal outcome between pregnancies in the RA and those in the SpA groups. There were four (4.9%), congenital malformation, two (3.8%) in RA group and two (6.9%) in SpA group. About one-third of patients 24 (30.7%) presented a moderate disease activity at conception as evaluated by DAS28PCR and BASDAI. However, there were no significant differences, on maternal and foetal/neonatal outcome in patients with moderate activity disease with respect of those in clinical remission.Conclusion:Even a higher rate of glucocorticoids and bDMARDs were used in RA than in SpA patients, there was no differences on pregnancy outcome between them.References:[1]Ostensen M. Nat Rev Rheumatol. 2017;13:485-493. doi: 10.1038/nrrheum.2017.102.[2]Polachek et al. J Rheumatol 2020;47:161-163. doi: 10.3899/jrheum.190631.Disclosure of Interests:None declared

POS1460-HPR MYRA TOUCH FOR DISEASE ACTIVITY EVALUATION IN RHEUMATOID ARTHRITIS: A COMPARISON OF PATIENT-REPORTED OUTCOMES WITH PHYSICIAN’S ASSESSMENT

Background:Patient-reported outcomes (PROs) have become an essential component of patients’ assessment in the management of Rheumatoid Arthritis(RA).They have been reported to be at least as informative if not more than physician assessed outcomes.MyRA Touch was pioneered by the Rheumatology Unit of Hospital Tuanku Jaa’far in Seremban Malaysia in March 2018,to engage and empower all RA patients on their own disease activity monitoring. It is an electronic platform, designed to enhanced the application of electronic patient reported outcomes (ePROs) among RA patients where they examine and record their own painful and/or swollen joints for DAS28 calculation and report their health assessment through Routine Assessment of Patient Index Data with 3 Measures (RAPID 3).MyRA Touch is an applications (App) that is user friendly and available in four major spoken languages (English, Chinese, Malay and Tamil) with an animated version for patients who are illiterate.Objectives:The objectives of this study are to determine the correlation between:I)Patient-reported and physician reported DAS28 ESR/CRPII)RAPID3 and Clinical Disease activity Index (CDAI)III)RAPID3 and DAS28 ESR/CRP assessed by physician and patientIV)RAPID3 and inflammatory markers ESR/CRP.Methods:This was a cross-sectional study carried out in the Rheumatology Unit of Hospital Tuanku Jaa’far. All data entered through MyRA Touch App from April 2018 till April 2020 was analysed.Results:There were a total of 562 patients who entered the data in the App, 87.9% were women. The ethnic compositions of the study subjects comprised of Indians (36.7%) followed by the Malays (34.7%),Chinese (26.3%) and other ethnics (2.3%). About half of patients (59.8%) were in the 51-70 age group whereas 22.9%,1.8% and 15.5% were in the 31-50,18-30 and above 70 age groups respectively. The majority of our patients (96%) were literate. A total of 54.3% of them received secondary education, 27% primary, 12.2% tertiary and 6.6% did not receive any formal education.There was a high level of correlation between DAS28 ESR/CRP performed by patient and DAS28 ESR/CRP assessed by physician, (r=0.808 for DAS28 ESR and r=0.804 for DAS28 CRP). RAPID3 also showed high level of correlation with CDAI and DAS28 CRP assessed by patient (r=0.700 and r=0.718 respectively). There was a moderate correlation between DAS28 ESR/CRP done by physician with RAPID3 (r=0.656 and r=0.696 respectively).RAPID3 demonstrated little correlation with inflammatory markers ESR and CRP (r=0.141 and r=0.171 respectively).Conclusion:PROs via DAS 28 (ESR/CRP) and RAPID3 showed moderate to high correlation with disease activity assessed by physician. We can empower patients to perform their own disease assessment by using the MyRA Touch App before seeing their physician and the information provided in the App, can help to reduced consultation time. During the COVID-19 pandemic, telemedicine is very much encouraged. By using the MyRA Touch, patients can assess their own tender and swollen joint count on a homunculus, evaluate their own physical function, health and pain using the RAPID3 parameters. The information obtained from the PROs in the MyRA touch App enables the physician to make a more comprehensive virtual assessment of the patient’s condition which helps in treatment decision making. In conclusion, MyRA Touch is an useful tool for disease activity measurement by patient.References:[1]Jenny AA, Diana BC, Omar JC, et al. Usefulness of Patients-Reported Outcomes in Rheumatoid Arthritis Focus Group. Hindawi Publishing Corporation Arthritis, vol 2012,Article ID935187.[2]Ana MO, Clifton OB. Patient Reported Outcomes in Rheumatoid Arthritis Clinical Trials. Curr Rheumato Rep.2015 April;17(4):501.Disclosure of Interests:None declared

POS1495-HPR THE EXPERIENCE OF A RHEUMATOLOGY UNIT DURING THE COVID19 LOCKDOWN: TELEMEDICINE ALLOWS A SAFE FOLLOW UP OF PATIENTS WITH RHEUMATIC DISEASES

Background:In March this year, most of the routine activities were cancelled during the streaming of the pandemic in Italy. This prompted a pragmatic reorganization of the traditional care model of nursing and medicine, to quickly give an efficient clinical response. During the first phase of the pandemic, outpatient visits dropped by more than 60%, forcefully shifting to telemedicine to assure continuity of care despite the lockdown.Objectives:The aim of the present work was to describe the strategy adopted during and immediately after the lockdown to assure the follow up of patients and the maintenance of their treatment in an outpatient “virtual” telemedicine clinic dedicated to RDs.Methods:the patient flow to a rheumatology division during the lockdown was evaluated retrospectively from March to September 2020 in accordance with local restrictions, and three periods are described.Results:653/913 (71.5%), 542/542 (100%) and 1.048/1.048 (100%) infusion activities scheduled were performed at the centre for daily infusion and pre-infusion assessment, respectively during the 1st, 2nd and 3rd period. In the outpatient clinic during the 1st period, 96.96% of the cases was shifted to Telemedicine, which decreased to 52.45% in the 2nd period; while in the 3rd period, 97.6% of the performances were carried out at the clinic. Diagnostic procedures, such as ultrasound, capillaroscopy, and joint injection were generally postponed during the 1st period, reduced drastically during the 2nd and performed regularly during 3rd period. Ulcer treatment and the Clinical Trial Unit never stopped their activity. The flow of the activity of the outpatient clinic and the day hospital is represented as monthly trends in graph 1 (See Graph 1).Conclusion:Our data show the feasibility of Telemedicine in a lockdown condition. Shifting stable patients to Telemedicine has the potentiality to minimize the risk of contagion and allow continuity of care. In the future, the use of Telemedicine for specific clinical uses might assure patient assistance also in non-pandemic conditions.References:[1]Rawaf S, Allen LN, Stigler FL et al. Lessons on the COVID-19 pandemic, for and by primary care professionals worldwide. Eur J Gen Pract. 2020 Dec;26(1):129-133. doi: 10.1080/13814788.2020.1820479. PMID: 32985278.[2]McDougall JA, Ferucci ED, Glover J, et al. Telerheumatology: A Systematic Review. Arthritis Care Res (Hoboken). 2017 Oct;69(10):1546-1557. doi: 10.1002/acr.23153. Epub 2017 Aug 22. PMID: 27863164; PMCID: PMC5436947.[3]Romão VC, Cordeiro I, Macieira C, Oliveira-Ramos F, Romeu JC, Rosa CM, Saavedra MJ, Saraiva F, Vieira-Sousa E, Fonseca JE. Rheumatology practice amidst the COVID-19 pandemic: a pragmatic view. RMD Open. 2020 Jun;6(2):e001314. doi: 10.1136/rmdopen-2020-001314. PMID: 32584782; PMCID: PMC7425193.Characters from table content including title and footnotes:Graph 1.Monthly trend for telemedicine and visits during the SARS Cov2 emergencyAcknowledgements:The project (Telereuma) has been supported by an unrestricted grant of Biogen, BMS, and Novartis.Disclosure of Interests:None declared