Cardiovascular comorbidities in psoriatic arthritis: state of the art.

Psoriatic arthritis (PsA) is a complex multi-system immune-mediated condition, characterised by a high comorbidity burden, one of the most prevalent of which is cardiovascular disease (CVD), affecting up to 80% of patients. This narrative review explores the current understanding of cardiovascular comorbidities in PsA, focusing on mechanistic pathways, risk assessment, and the impact of treatment choices on cardiovascular health. Here, we outline the role of inflammatory cytokines, immune system dysregulation, and genetic predispositions in PsA, not only as drivers of musculoskeletal manifestations but also atherosclerosis and endothelial dysfunction, giving rise to cardiovascular pathology. Given these insights, accurately assessing and predicting cardiovascular risk in PsA patients is a critical challenge. This review evaluates traditional risk calculators as well as innovative biomarkers and imaging techniques, emphasising their utility and limitations in capturing the true cardiovascular risk profile of PsA patients. There are multiple complexities surrounding the treatment of PsA in the context of concurrent CVD, and therapeutic choices must carefully balance efficacy in managing PsA symptoms with the potential cardiovascular implications. A multidisciplinary approach, integrating dermatological, rheumatological, and cardiological perspectives, amongst others, to optimise patient outcomes, is key. Overall, a heightened clinical awareness and research focus on cardiovascular comorbidities in PsA is warranted, aiming to refine risk assessment strategies and therapeutic interventions that holistically address the multifaceted needs of patients with PsA.

Management and outcomes of interstitial lung disease associated with anti-synthetase syndrome: A systematic literature review.

BACKGROUND: Anti-synthetase syndrome (ASS) is a chronic autoimmune condition, with interstitial lung disease (ILD) being a key feature. This systematic literature review (SLR; CRD42023416414) aimed to summarise treatments and outcomes of ILD associated with ASS (ASS-ILD). METHODS: Databases were searched for articles discussing ASS-ILD management and outcomes, published 1946-September 2023.Screening and data-extraction were performed by two reviewers. Meta-analysis, using a random effects model, and paired t-tests, were undertaken where appropriate to evaluate post-treatment-change in Pulmonary Function Tests (PFT). RESULTS: Ten articles were included, comprising 514 patients: 67.8% female, mean age 52.4years (SD4.6). Baseline high-resolution computed tomography was documented in 447 patients (86.9%); the most common pattern was non-specific interstitial pneumonia (n = 220; 49.2%). The most common myositis-associated autoantibody was anti-Jo1 (48%) with 27.8% having associated anti-Ro52 antibodies.Pooled estimates, after meta-analysis, for baseline Forced Vital Capacity (FVC) was 60.8% predicted (SE 2.1), and Diffusion Capacity of Lungs for Carbon Monoxide (DLco) was 49.8% (SE 3.5). After one-year, pooled improvement in FVC was 14.1% from baseline (SE 3.1) and in DLco was 15.1% (SE 2.8). Paired t-test demonstrated significant overall improvement in FVC (p = 0.007) and DLco (p = 0.002).Patients receiving RTX had 12.2% improvement in FVC and 2.9% increase in DLco at one-year; for patients receiving CYC, there was 17% improvement and 6.3% increase, respectively. 28 deaths were reported. CONCLUSION: Our SLR, the first to summarise management and outcomes of ASS-ILD, found no conclusive difference between effectiveness of treatments. More robust trials are required to reduce morbidity and mortality resulting from ASS-ILD.

Evaluating the competency of ChatGPT in MRCP Part 1 and a systematic literature review of its capabilities in postgraduate medical assessments.

OBJECTIVES: As a large language model (LLM) trained on a large data set, ChatGPT can perform a wide array of tasks without additional training. We evaluated the performance of ChatGPT on postgraduate UK medical examinations through a systematic literature review of ChatGPT's performance in UK postgraduate medical assessments and its performance on Member of Royal College of Physicians (MRCP) Part 1 examination. METHODS: Medline, Embase and Cochrane databases were searched. Articles discussing the performance of ChatGPT in UK postgraduate medical examinations were included in the systematic review. Information was extracted on exam performance including percentage scores and pass/fail rates. MRCP UK Part 1 sample paper questions were inserted into ChatGPT-3.5 and -4 four times each and the scores marked against the correct answers provided. RESULTS: 12 studies were ultimately included in the systematic literature review. ChatGPT-3.5 scored 66.4% and ChatGPT-4 scored 84.8% on MRCP Part 1 sample paper, which is 4.4% and 22.8% above the historical pass mark respectively. Both ChatGPT-3.5 and -4 performance was significantly above the historical pass mark for MRCP Part 1, indicating they would likely pass this examination. ChatGPT-3.5 failed eight out of nine postgraduate exams it performed with an average percentage of 5.0% below the pass mark. ChatGPT-4 passed nine out of eleven postgraduate exams it performed with an average percentage of 13.56% above the pass mark. ChatGPT-4 performance was significantly better than ChatGPT-3.5 in all examinations that both models were tested on. CONCLUSION: ChatGPT-4 performed at above passing level for the majority of UK postgraduate medical examinations it was tested on. ChatGPT is prone to hallucinations, fabrications and reduced explanation accuracy which could limit its potential as a learning tool. The potential for these errors is an inherent part of LLMs and may always be a limitation for medical applications of ChatGPT.

What role do socioeconomic and clinical factors play in disease activity states in rheumatoid arthritis? Data from a large UK early inflammatory arthritis audit.

BACKGROUND: Persistently active rheumatoid arthritis (pactiveRA) may be due to the interplay between biological and non-biological factors. The role of socioeconomic factors remains unclear. OBJECTIVES: To explore which biological and non-biological factors associate with pactiveRA. METHODS: Adults with early RA in the National Early Inflammatory Arthritis Audit, recruited from May 2018 to October 2022, were included if having pactiveRA or persistently low RA (plowRA). The pactiveRA was defined as three consecutive Disease Activity Score-28 joints (DAS28) of >3.2 at baseline, 3 and 12 months. The plowRA was defined as DAS28 ≤3.2 at 3 and 12 months. Stepwise forward logistic regression was used to explore associations with pactiveRA (outcome). Age and gender were included a priori, with socioeconomic factors and comorbidities as exposure variables. RESULTS: 682 patients with pactiveRA and 1026 plowRA were included. Compared with plowRA, patients with pactiveRA were younger (58, IQR: 49-67) versus (62, IQR: 52-72), and included more women (69% vs 59%). The pactiveRA was associated with worse scores in patient-reported outcomes at baseline, and anxiety and depression screens. Overall, there was clear social patterning in pactiveRA, with age-by-gender interaction. Logistic regression indicated age, gender, social deprivation and previous or current smoking, were independently associated with pactiveRA, after controlling for disease severity markers (seropositivity). Depression, lung disease, gastric ulcers and baseline corticosteroid use, were also associated with pactiveRA (p<0.05 for all). CONCLUSION: Socioeconomic factors and deprivation were associated with pactiveRA, independent of clinical and disease characteristics. Identifying 'adverse' socioeconomic drivers of pactiveRA can help tailor interventions according to individual need.

Pulmonary manifestations, treatments and outcomes of IgG4-related disease-a systematic literature review.

Immunoglobulin G4-related disease (IgG4-RD) is a multisystem fibroinflammatory condition. A consistent feature of many cases is pulmonary infiltrates, or respiratory failure. This systematic literature review aims to summarise the pulmonary manifestations of IgG4-RD, including clinical outcomes and treatment. This review was registered on PROSPERO (CRD42023416410). Medline, Embase and Cochrane databases were searched for articles discussing IgG4-RD syndrome. Information was extracted on demographics, type and prevalence of pulmonary manifestations, treatment and clinical outcomes. Initially, after deduplication, 3123 articles were retrieved with 18 ultimately included. A pooled total of 724 patients with IgG4-RD were included, 68.6% male, mean age 59.4 years (SD 5.8) at disease onset. The most frequently described pulmonary manifestation was mediastinal lymphadenopathy (n = 186, 48.8%), followed by pulmonary nodules (n = 151, 39.6%) and broncho-vascular thickening (n = 85, 22.3%). Where treatment was reported, the majority of patients received glucocorticoids (n = 211, 93.4%). Other immunosuppressive therapy included cyclophosphamide (n = 31), azathioprine (n = 18), with mycophenolate mofetil (n = 6), rituximab (n = 6), methotrexate (n = 5) and other unspecified immunomodulators (50). Clinical outcomes were reported in 263 patients, where 196 patients had remission of their disease, 20 had relapse, 35 had stable disease, four had progression and eight patients died from complications of IgG4-RD. This systematic review summarises pulmonary manifestations, treatments and outcomes in patients with IgG4-RD. Pulmonary involvement in IgG4-RD is relatively common, leading to high levels of morbidity and mortality. Glucocorticoids remain the mainstay of treatment, but further work is required to explore the management of patients with pulmonary manifestations in association with IgG4-RD.

Understanding the psychosocial determinants of effective disease management in rheumatoid arthritis to prevent persistently active disease: a qualitative study.

BACKGROUND: According to epidemiological studies, psychosocial factors are known to be associated with disease activity, physical activity, pain, functioning, treatment help-seeking, treatment waiting times and mortality in people with rheumatoid arthritis (RA). Limited qualitative inquiry into the psychosocial factors that add to RA disease burden and potential synergistic interactions with biological parameters makes it difficult to understand patients' perspectives from the existing literature. AIM: This study aimed to gather in-depth patient perspectives on psychosocial determinants that drive persistently active disease in RA, to help guide optimal patient care. METHODS: Patient research partners collaborated on the research design and materials. Semistructured interviews and focus groups were conducted online (in 2021) with patients purposively sampled from diverse ethnicities, primary languages, employment status and occupations. Data were analysed using inductive thematic analysis. RESULTS: 45 patients participated across 28 semistructured interviews and three focus groups. Six main themes on psychosocial determinants that may impact RA management were identified: (1) healthcare systems experiences, (2) patient education and health literacy, (3) employment and working conditions, (4) social and familial support, (5) socioeconomic (dis)advantages, and (6) life experiences and well-being practices. CONCLUSION: This study emphasises the importance of clinicians working closely with patients and taking a holistic approach to care that incorporates psychosocial factors into assessments, treatment plans and resources. There is an unmet need to understand the relationships between interconnected biopsychosocial factors, and how these may impact on RA management.

Incidence, management and prognosis of new-onset sarcoidosis post COVID-19 infection.

BACKGROUND AND AIM: SARS-CoV-2 infection has been linked to hyperinflammation in multiple organs with a potential mechanistic link with resulting autoimmunity. There have been reports of many inflammatory complications following COVID-19, including sarcoidosis. A literature review on new-onset sarcoidosis following COVID-19 is lacking. We evaluated potential associations between COVID-19 and development of new-onset sarcoidosis. METHODS: Articles discussing biopsy-proven sarcoidosis after confirmed COVID-19 infection, published 1956 until April 2023, were included. All article types were deemed eligible except opinion and review articles. RESULTS: A pooled total of 15 patients with new-onset diagnosis of sarcoidosis after COVID-19 infection were included, 45.5% female, mean age 46.1 years (standard deviation 14.7) at onset of sarcoidosis. Patients were from: Europe (n=11); North America (n=2); South America (n=1); Asia (n=1). The mean time between COVID-19 infection and diagnosis of sarcoidosis was 56.3 days, although this ranged from 10 to 140 days. Organ systems predominantly affected by sarcoidosis were: pulmonary (n=11); cutaneous (n=3); cardiac (n=2); ocular (n=1); systemic (n=1) (with overlapping features in certain patients). Sarcoidosis was treated as follows: glucocorticoids (n=8); azathioprine (n=1); cardiac re-synchronisation therapy (n=1); heart transplant (n=1). All patients were reported to have survived, with one requiring intensive care admission. CONCLUSIONS: Our result suggests there is a potential link between COVID-19 and new-onset sarcoidosis. The potential mechanism for this is through cytokine mediated immune modulation in COVID-19 infection. Obtaining a tissue sample remains key in confirming the diagnosis of sarcoidosis and this may be delayed during active COVID-19 infection.

Personalised care packages for people with rheumatoid arthritis: a mixed-methods study.

OBJECTIVES: Disease management in rheumatoid arthritis (RA) requires holistic assessment. We aimed to design personalised care packages suitable for people with RA. METHODS: This study was conducted using a mixed-methods approach and exploratory sequential design. Consensus workshops were held, involving people with RA and healthcare professionals (HCPs) treating them. Subsequently, an online survey sought views on future care packages for people with RA at relevant disease progression/stages, based on (1) results from previous quantitative data analyses (eg, socioeconomic/clinical factors), and (2) themes identified during workshops. RESULTS: Two conceptual care pathways were identified: (1) around the time of RA diagnosis, an early opportunity to influence the disease course; (2) for individuals with established RA, emphasising the importance of 'the right MDT member at the right time'.Three care packages were suggested: (1) early care package (around RA diagnosis): introduction to MDT; (2) continuity of care package (established RA): primary/secondary providers; and (3) personalised holistic care package: integral to packages 1 and 2, implemented alongside allied health professionals.The survey received 41 responses; 82.9% agreed that people with RA need a consistent 'early care package' at diagnosis. 85.4% approved of additional care packages tailored to individuals' clinical, psychological and social needs when moving to different stages of their long-term disease. Fleiss' Kappa calculations demonstrated fair level of agreement among respondents. CONCLUSION: Two care pathways, with three tailored care packages, were identified, with potential to improve management of people with RA. Future research will help to determine if such care packages can impact clinical (including patient-reported) outcomes.

Clinical characteristics, disease trajectories and management of vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome: a systematic review.

BACKGROUND: VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is a newly discovered autoinflammatory condition characterised by somatic mutation of the UBA1 gene. The syndrome leads to multi-system inflammation affecting predominantly the skin, lungs and bone marrow. METHODS: We undertook a systematic review of the multisystem features and genotypes observed in VEXAS syndrome. Articles discussing VEXAS syndrome were included. Medline, Embase and Cochrane databases were searched. Information was extracted on: demographics, type and prevalence of clinical manifestations, genetic mutations and treatment. Meta-analysis using a random effects model was used to determine pooled estimates of serum markers. RESULTS: From 303 articles, 90 were included, comprising 394 patients with VEXAS. 99.2% were male, with a mean age of 67.1 years (SD 8.5) at disease onset. The most frequent diagnoses made prior to VEXAS were: relapsing polychondritis (n = 59); Sweet's syndrome (n = 24); polyarteritis nodosa (n = 11); and myelodysplastic syndrome (n = 10). Fever was reported in 270 cases (68.5%) and weight loss in 79 (20.1%). Most patients had haematological (n = 342; 86.8%), dermatological (n = 321; 81.5%), pulmonary (n = 297; 75.4%%) and musculoskeletal (n = 172; 43.7%) involvement, although other organ manifestations of varying prevalence were also recorded. The most commonly reported mutations were "c.122T > C pMET41Thr" (n = 124), "c.121A > G pMET41Val" (n = 62) and "c.121A > C pMet41Leu" (n = 52). Most patients received glucocorticoids (n = 240; 60.9%) followed by methotrexate (n = 82; 20.8%) and IL-6 inhibitors (n = 61, 15.4%). One patient underwent splenectomy; 24 received bone marrow transplants. CONCLUSION: VEXAS syndrome is a rare disorder affecting predominantly middle-aged men. This is the first systematic review to capture clinical manifestations, genetics and treatment of reported cases. Further studies are needed to optimise treatment and subsequently reduce morbidity and mortality.

Incidence, clinical features, management and outcomes of ANCA-associated vasculitis in pregnancy- a systematic literature review.

BACKGROUND AND AIM: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are rare multi-system conditions, usually presenting in older age groups. However, younger individuals are also affected. The average increase of childbearing age and lack of studies in pregnancy necessitates this comprehensive review of data to guide the management of AAV in pregnancy. This systematic review (SR) aimed to summarise the incidence, clinical features, management and maternal and foetal outcomes in female patients with AAV. METHODS: The protocol was registered on PROSPERO (CRD42023437482). Articles published in Medline, Embase and Cochrane Databases from 1946 until June 2023 were included. Single case reports, reviews and conference abstracts were excluded. Articles meeting inclusion criteria were examined by two authors. Data on demographics, treatment, clinical features, flares during pregnancy and maternal and foetal outcomes were extracted. RESULTS: Eight studies were included, detailing 82 pregnancies in 64 women. The most common drugs used for remission induction pre-conception were cyclophosphamide, rituximab, prednisolone and azathioprine. Serious maternal complications in pregnancy included progressive tracheal/subglottic stenosis (n=5), renal disease (n=2), preeclampsia (n=10) and miscarriages (n=5). Foetal anomalies were rare (n=5). The mean birth weight was 3.37kgs and mean gestation age was 38.26 weeks. No maternal deaths or vasculitis in newborns were reported.  Conclusions: Patients can have positive maternal and foetal outcomes following strong induction therapy, vigorous monitoring and prompt treatment of flares during pregnancy. Serious complications and flares are not associated with worse outcomes for newborns.

COVID-19 Vaccination-Related Delayed Adverse Events among Patients with Systemic Lupus Erythematosus.

BACKGROUND: The safety profile of COVID-19 vaccination is well documented, but hesitancy among people with immune-mediated inflammatory diseases, often immunocompromised, remains high, partially due to a scarcity of data on safety over a longer term. We herein aimed to assess delayed adverse events (DAEs) occurring >7 days after COVID-19 vaccination in systemic lupus erythematosus (SLE) versus other rheumatic autoimmune diseases (rAIDs), non-rheumatic AIDs (nrAIDs), and healthy controls (HCs). METHODS: Self-reported data were captured within the COVID-19 Vaccination in Autoimmune Diseases (COVAD)-2 online survey, which comprised >150 centres and responses from 106 countries, between February and June 2022. Logistic regression analysis adjusting for important confounders (age, sex, ethnicity) was used to compare groups. RESULTS: Of 7203 eligible individuals, 882 (12.2%) patients had SLE, 3161 (43.9%) patients had rAIDs, 426 (5.9%) patients had nrAIDs, and 2734 (38.0%) were HCs. SLE patients had a median age of 39 years (IQR: 31-50); 93.7% were women. SLE patients reported, more frequently, major DAEs (OR: 1.6; 95% CI: 1.2-2.0; p = 0.001) and hospitalisation (OR: 2.2; 95% CI: 1.4-3.4; p < 0.001) compared to HCs, severe rashes (OR: 2.4; 95% CI: 1.3-4.2; p = 0.004) compared to people with rAIDS, and hospitalisation (OR: 2.3; 95% CI: 1.1-4.9; p = 0.029) as well as several minor DAEs compared to people with nrAIDs. Differences were observed between vaccines in terms of frequency of major DAEs and hospitalisations, with the latter seen more frequently in patients receiving the Moderna vaccine. People with SLE with no autoimmune multimorbidity less frequently reported overall minor DAEs compared to SLE patients with comorbid nrAIDs (OR: 0.5; 95% CI: 0.3-1.0; p = 0.036). CONCLUSION: Hospitalisations post-vaccination were more frequent in SLE patients than in HCs. Monitoring of SLE patients following COVID-19 vaccination can help in identifying DAEs early, informing patients about expected DAEs, and supporting patients, especially those with autoimmune multimorbidity.

Multimorbidity in rheumatoid arthritis: common mechanistic links and impact and challenges in routine clinical practice.

Early identification and management of multimorbidity in patients with rheumatic and musculoskeletal diseases (RMDs), such as RA, is an integral, but often neglected, aspect of care. The prevalence and incidence of conditions such as osteoporosis, cardiovascular disease, pulmonary disease and malignancies, often co-existing with RA, continues to have significant implications for the management of this patient group. Multimorbidity in RMDs can be associated with inflammatory disease activity and target organ damage. Lifestyle factors, such as smoking and inactivity, further contribute to the burden of disease. Inflammation is the underlying factor, not just in RA but also many comorbidities. The current framework of a treat-to-target approach focuses on achieving early remission and inflammatory activity suppression. We describe how the comorbidity burden in people with RMDs impacts on disease outcome and treatment response. The importance of addressing comorbidity at an early stage and adopting a patient centred approach is critical in modern practice.

Opportunistic optimization of inhaler technique in hospitalized adults with asthma: a two-phase educational study.

OBJECTIVE: To investigate effectiveness of two different educational methods to improve inhaler techniques in patients with prior diagnosis of asthma, hospitalized with a non-asthma-related diagnosis. METHODS: We undertook a real-world, opportunistic quality-improvement project. Inhaler technique in hospitalized patients with prior diagnosis of asthma was assessed in two cohorts over two 12-week cycles using a standardized device-specific proforma of seven-step inhaler technique, classed: "good" if 6/7 steps achieved; "fair" if 5/7 compliant; "poor" for others. Baseline data was collected in both cycles. Cycle one involved face-to-face education by a healthcare professional; cycle two involved additional use of an electronic device to show device-specific videos (asthma.org.uk). In both cycles, patients were reassessed within two days for improvements and the two methods compared for effectiveness. RESULTS: During cycle one 32/40 patients were reassessed within 48 h; eight lost to follow-up. During cycle two 38/40 patients were reassessed within 48 h; two lost to follow-up During cycle one, two and 12 had good/fair baseline technique respectively, and 26 poor. Most commonly missed steps were no expiry check/not rinsing mouth after steroid use. On reassessment 17% patients improved from poor to fair/good. During cycle two, initial technique assessment identified: 23 poor; 12 fair; five good. Post-videos, 35% of patients improved from poor to fair/good. Proportion of patients improving from poor to fair, or poor/fair to good increased in cycle two vs one (52.5% vs 33%). CONCLUSION: Visual instruction is associated with improved technique compared to verbal feedback. This is a user-friendly and cost-effective approach to patient education.

A systematic review of the incidence, management and prognosis of new-onset autoimmune connective tissue diseases after COVID-19.

A literature review on new-onset autoimmune connective tissue diseases (ACTDs) following COVID-19 is lacking. We evaluated potential associations between COVID-19 and the development of new-onset ACTDs. The "population" was adults with disease terms for ACTDs, including systemic lupus erythematosus (SLE), Sjogren's syndrome, systemic sclerosis (SSc), idiopathic inflammatory myositis (IIM), anti-synthetase syndrome, mixed CTD and undifferentiated CTD, and "intervention" as COVID-19 and related terms. Databases were searched for English-language articles published until September 2022. We identified 2236 articles with 28 ultimately included. Of the 28 included patients, 64.3% were female, with a mean age was 51.1 years. The USA reported the most cases (9/28). ACTD diagnoses comprised: 11 (39.3%) IIM (including four dermatomyositis); 7 (25%) SLE; four (14.3%) anti-synthetase syndrome; four (14.3%) SSc; two (7.1%) other ACTD (one lupus/MCTD overlap). Of eight, four (14.3%) patients (including that with lupus/MCTD) had lupus nephritis. The average time from COVID-19 to ACTD diagnosis was 23.7 days. A third of patients were admitted to critical care, one for treatment of haemophagocytic lymphohistiocytosis in SLE (14 sessions of plasmapheresis, rituximab and intravenous corticosteroids) and nine due to COVID-19. 80% of patients went into remission of ACTD following treatment, while three (10%) patients died-one due to macrophage activation syndrome with anti-synthetase syndrome and two from unreported causes. Our results suggest a potential association between COVID-19 and new-onset ACTDs, notably in young females, reflecting more comprehensive CTD epidemiology. The most common diagnosis in our cohort was IIM. The aetiology and mechanisms by which ACTDs emerge following COVID-19 remain unknown and require further research.

Pulmonary manifestations in VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome: a systematic review.

BACKGROUND: VEXAS (vacuoles, E1 enzyme, X-linked, auto-inflammatory, somatic) syndrome is a newly described auto-inflammatory disease. Many cases feature pulmonary infiltrates or respiratory failure. This systematic review aimed to summarize respiratory manifestations in VEXAS syndrome described to date. METHODS: Databases were searched for articles discussing VEXAS syndrome until May 2022. The research question was: What are the pulmonary manifestations in patients with VEXAS syndrome? The search was restricted to English language and those discussing clinical presentation of disease. Information on basic demographics, type and prevalence of pulmonary manifestations, co-existing disease associations and author conclusions on pulmonary involvement were extracted. The protocol was registered on the PROSPERO register of systematic reviews. RESULTS: Initially, 219 articles were retrieved with 36 ultimately included (all case reports or series). A total of 269 patients with VEXAS were included, 98.6% male, mean age 66.8 years at disease onset. The most frequently described pulmonary manifestation was infiltrates (43.1%; n = 116), followed by pleural effusion (7.4%; n = 20) and idiopathic interstitial pneumonia (3.3%; n = 9). Other pulmonary manifestations were: nonspecific interstitial pneumonia (n = 1), bronchiolitis obliterans (n = 3), pulmonary vasculitis (n = 6), bronchiectasis (n = 1), alveolar haemorrhage (n = 1), pulmonary embolism (n = 4), bronchial stenosis (n = 1), and alveolitis (n = 1). Several patients had one or more co-existing autoimmune/inflammatory condition. It was not reported which patients had particular pulmonary manifestations. CONCLUSION: This is the first systematic review undertaken in VEXAS patients. Our results demonstrate that pulmonary involvement is common in this patient group. It is unclear if respiratory manifestations are part of the primary disease or a co-existing condition. Larger epidemiological analyses will aid further characterisation of pulmonary involvement and disease management.

Assessment and diagnosis of the acute hot joint: a systematic review and meta-analysis.

OBJECTIVES: Prompt diagnosis of septic arthritis (SA) in acute native hot joints is essential for avoiding unnecessary antibiotics and hospital admissions. We evaluated the utility of synovial fluid (SF) and serum tests in differentiating causes of acute hot joints. METHODS: We performed a systematic literature review of diagnostic testing for acute hot joints. Articles were included if studying ≥1 serum or SF test(s) for an acute hot joint, compared with clinical assessment and SF microscopy and culture. English-language articles only were included, without date restriction. The following were recorded for each test, threshold and diagnosis: sensitivity, specificity, positive/negative predictive values and likelihood ratios. For directly comparable tests (i.e. identical fluid, test and threshold), bivariate random-effects meta-analysis was used to pool sensitivity, specificity, and areas under the curves. RESULTS: A total of 8443 articles were identified, and 49 were ultimately included. Information on 28 distinct markers in SF and serum, differentiating septic from non-septic joints, was extracted. Most had been tested at multiple diagnostic thresholds, yielding a total of 27 serum markers and 156 SF markers. Due to heterogeneity of study design, outcomes and thresholds, meta-analysis was possible for only eight SF tests, all differentiating septic from non-septic joints. Of these, leucocyte esterase had the highest pooled sensitivity [0.94 (0.70, 0.99)] with good pooled specificity [0.74 (0.67, 0.81)]. CONCLUSION: Our review demonstrates many single tests, individually with diagnostic utility but suboptimal accuracy for exclusion of native joint infection. A combination of several tests with or without a stratification score is required for optimizing rapid assessment of the hot joint.

2022 EULAR recommendations for screening and prophylaxis of chronic and opportunistic infections in adults with autoimmune inflammatory rheumatic diseases.

OBJECTIVES: To develop EULAR recommendations for screening and prophylaxis of chronic and opportunistic infections in patients with autoimmune inflammatory rheumatic diseases (AIIRD). METHODS: An international Task Force (TF) (22 members/15 countries) formulated recommendations, supported by systematic literature review findings. Level of evidence and grade of recommendation were assigned for each recommendation. Level of agreement was provided anonymously by each TF member. RESULTS: Four overarching principles (OAP) and eight recommendations were developed. The OAPs highlight the need for infections to be discussed with patients and with other medical specialties, in accordance with national regulations. In addition to biologic/targeted synthetic disease-modifying antirheumatic drugs (DMARDs) for which screening for latent tuberculosis (TB) should be performed, screening could be considered also before conventional synthetic DMARDs, glucocorticoids and immunosuppressants. Interferon gamma release assay should be preferred over tuberculin skin test, where available. Hepatitis B (HBV) antiviral treatment should be guided by HBV status defined prior to starting antirheumatic drugs. All patients positive for hepatitis-C-RNA should be referred for antiviral treatment. Also, patients who are non-immune to varicella zoster virus should be informed about the availability of postexposure prophylaxis should they have contact with this pathogen. Prophylaxis against Pneumocystis jirovecii seems to be beneficial in patients treated with daily doses >15-30 mg of prednisolone or equivalent for >2-4 weeks. CONCLUSIONS: These recommendations provide guidance on the screening and prevention of chronic and opportunistic infections. Their adoption in clinical practice is recommended to standardise and optimise care to reduce the burden of opportunistic infections in people living with AIIRD.