Abstract Background/Aims There is an increased mortality rate associated with RA. Previously, cardiovascular disease (CVD) has been identified as the major cause of mortality in patients with RA. The management of CVD has markedly improved over recent decades, with the recognition of acute coronary syndrome using high sensitive troponin assays and the early use of coronary artery stenting and bypass surgery. Enhanced therapies for cardiac failure have further reduced cardiovascular mortality. Consequently the causes of death in patients with RA may have shifted over the past two decades. Methods The data in this study were collected as part of project IRAS ID 194833, approved by South West regional ethical committee (UK). The cohort consisted of men attending routine rheumatology clinics at the Royal Cornwall Hospital, Cornwall, UK, from February 2015 to August 2016. All patients fulfilled 2010 American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) RA criteria at diagnosis. Data were anonymised at source. The men were followed up for 10 years and mortality was recorded; where possible the cause of mortality was ascertained from hospital notes or death certificates. Results A total of 247 deaths were recorded over a 10-year period in a cohort of 667 males with RA. Therefore 37% of the cohort of RA males died over a 10-year period. The annualised death rate was 3.7 %/year. Two hundred deaths were analysed further. The single most common type of death was respiratory in origin. There were 67/200 (33.5%) respiratory deaths. In addition, there were 15 deaths due to lung cancer 15/200 (7.5%). Any other type of cancer accounted for 35/200 (17.5%) deaths. CVD only accounted for 12/200 (6%) deaths. The rest of the deaths (71/200 [35.5%]) were because of frailty, dementia, fracture of the femur and sepsis. Conclusion Respiratory disease and lung cancer account for 41% of all the deaths. CVD only accounted for 6% of deaths. Greater emphasis should be placed on the respiratory system by routinely inquiring about respiratory symptoms at each consultation and performing regular chest auscultation to detect any basal crepitations. The use of hand held spirometers in clinic should be encouraged, along with a strong focus on joint rheumatology and respiratory clinics, smoking cessation programmes and ensuring that Influenza, pneumococcal, and COVID -19 vaccinations are up to date. Disclosure M. Dissanayake: None. D. Hutchinson: None.

Systemic sclerosis (SSc) is associated with substantial morbidity and mortality, prompting the development of internationally endorsed quality indicators (QIs). However, real-world adherence to these standards remains incompletely characterized. We evaluated adherence to baseline and longitudinal SSc QIs and identified predictors of adherence in a specialized clinical practice. We conducted a retrospective cohort study including all patients with SSc managed at a tertiary SSc clinic between January 2016 and December 2024. Adherence to QIs was assessed across baseline screening (interstitial lung disease and pulmonary hypertension), longitudinal monitoring (pulmonary function testing, echocardiographic follow-up, skin assessment, and clinical documentation), and treatment and referral practices (digital ulcer management and rehabilitation). Multivariable logistic regression identified independent predictors of adherence. Among 66 patients (mean age 46.2 [SD 12.9] years, 92% female), adherence to baseline screening was high, including high-resolution computed tomography (86%) and transthoracic echocardiography (TTE; 84%). Baseline treatment adherence was also high, with vasodilator therapy for all patients with active digital ulcers (100%). In contrast, longitudinal follow-up adherence was suboptimal, including annual pulmonary function testing (35%), follow-up TTE after a new decline in diffusing lung capacity for carbon monoxide (36%), dyspnea documentation (63%), chest auscultation (27%), annual modified Rodnan skin score (19%), and physical therapy referral (37%). Older age, longer disease duration, diffuse cutaneous subtype, and pulmonary hypertension independently predicted lower adherence. Despite strong baseline adherence, major gaps persist in longitudinal monitoring, documentation, and rehabilitation, highlighting key targets for quality improvement.

Evaluate the impact of lung ultrasound (LUS) compared to current chest physiotherapy (CPT) outcome measures on clinical decision-making in the Neonatal Intensive Care Unit (NICU). Single-centre prospective observational study at the NICU, Mater Mothers Hospital (MMH), Brisbane. Infants admitted to the NICU who had a chest x-ray (CXR) within the preceding 24 h. Infants were excluded if LUS was not possible, were expected to be discharged from MMH in less than 24 h or had a plan for redirection of care. Infants were assessed using CXR and auscultation by a physiotherapist, who recorded a diagnosis and intervention plan. An examiner blinded to the initial assessment then performed a LUS assessment. After receiving the LUS results, the initial physiotherapist recorded a re-evaluated intervention plan. The primary outcome was the net reclassification improvement (NRI), which quantified how frequently physiotherapy diagnosis and treatment was reclassified after LUS. Secondary outcomes included diagnostic concordance between CXR/auscultation and LUS, and physiotherapist confidence in diagnoses. From 30 included episodes, NRI was -41% (SE 0.38; 95% CI -116% to 33%), indicating that LUS did not significantly result in reclassification of physiotherapy diagnosis and treatment. Diagnostic concordance was moderate at 0.595 (p < 0.001) observed in 21 (70%) cases, resulting in treatment alteration in nine instances. After LUS, physiotherapy confidence in diagnosis increased by 1.16 on a 7-point Likert scale (p < 0.001). Although LUS did not result in significant reclassification of physiotherapy diagnosis and treatment, it did result in treatment alteration and improved physiotherapy confidence in diagnosis. ACTRN12624000290594. CONTRIBUTION OF THE PAPER.

Anaphylaxis is a severe, potentially life-threatening systemic hypersensitivity reaction. Anaphylactic reactions commonly present with hypotension. However, the incidence of hypertensive anaphylaxis is relatively uncommon. Only a few literary works are available on hypertensive anaphylaxis. A 68-year-old woman arrived to the emergency room with complaints of hoarseness of voice and breathing difficulty after being stung by a wasp an hour back. On arrival in the emergency room, she was tachypnoeic, with a saturation of 89% in room air, blood pressure (BP) of 180/110mmHg, and heart rate (HR) of 110 bpm. There was hoarseness of voice, swollen tongue, and bilateral rhonchi on chest auscultation. A diagnosis of anaphylaxis was made and managed with intramuscular adrenaline, intravenous (IV) hydrocortisone, IV pheniramine, and IV adrenaline. Her BP remained persistently high till the anaphylaxis started to resolve. Once the patient started showing improvement in her symptoms, her blood pressure came down to 160/80mmHg, and her blood pressure on discharge was 130/70mmHg. It's unusual for anaphylaxis to present with hypertension. Anaphylaxis can present with hypertension also as compared to the usual hypotension. Adrenaline remains the first line in anaphylaxis. In a scenario with high BP with anaphylaxis, adrenaline can be given under strict haemodynamic monitoring. Do not hesitate to administer adrenaline in anaphylaxis, and always keep in mind the possibility of hypertensive anaphylaxis.

Early detection of cardiovascular and pulmonary diseases is essential for improving patient outcomes, but traditional methods like auscultation are often subjective and require significant resources. While deep learning has shown potential in analyzing audio, it usually lacks a comprehensive, patient-focused interpretation. In this paper, we introduce a new hybrid AI system that combines three different data sources: chest auscultation audio, analysis by specialized deep learning models, and symptoms reported by the user. Our approach starts by converting standard 5-second audio clips into 2D Mel spectrograms. These spectrograms are then analyzed by a 'Committee of Experts'—two separate Convolutional Neural Networks (CNNs) specialized in heart and lung sounds. We tested our method using a custom CNN and a pre-trained EfficientNetV2-B0 model with transfer learning. The EfficientNetV2-B0 model performed better, achieving 92.15% accuracy for heart sounds and 90.5% for lung sounds. The unique final step in our system uses an LLM-based synthesizer to combine the technical results from both specialists with the user's described symptoms. In a qualitative study with 15 participants, 93% found the report generated by the LLM much clearer and more useful than the raw technical data. This hybrid, multi-modal system offers a reliable, accurate, and easy-to-use framework for e-Health screening.

Larsen syndrome is a rare congenital skeletal dysplasia characterized by multiple joint dislocations and distinctive craniofacial features, which can complicate neonatal airway management. We report a term female neonate born at 40 weeks of gestation, weighing 2200 grams, delivered spontaneously in a peripheral facility with thick meconium-stained amniotic fluid. The infant did not cry at birth and exhibited severe respiratory depression (Apgar scores 3–4–5). Initial resuscitation, including airway clearance and positive pressure ventilation without a self-inflating bag, was performed to achieve effective lung aeration, resulting in gradual improvement in oxygen saturation from 93% to 100%. Clinical examination revealed congenital knee dislocation and cephalhematoma, while anthropometric assessment indicated intrauterine growth restriction. Chest auscultation demonstrated no adventitious sounds despite persistent tachypnea. This case highlights the complexity of neonatal stabilization when meconium aspiration syndrome coexists with syndromic skeletal anomalies. In resource-limited settings, structured resuscitation protocols and careful handling of the airway and limbs are essential to optimize outcomes and prevent further injury.

Experts offer nursing students advice on chest auscultation, which is a required proficiency for NMC registration

Abstract Introduction Anti-Melanoma Differentiation-Associated gene 5 dermatomyositis (anti-MDA5 DM) is a distinct subtype of idiopathic inflammatory myopathy, often associated with clinically amyopathic features and rapidly progressive interstitial lung disease (RP-ILD). Early symptoms may resemble seronegative rheumatoid arthritis, complicating early diagnosis. COVID-19 infection adds another layer of complexity, both as a potential trigger for autoimmune phenomena and as a source of overlapping pulmonary pathology. This overlap can result in delayed recognition and treatment of potentially life-threatening autoimmune pathology. We present a case of anti-MDA5 DM with ILD in which an initial misdiagnosis of COVID-19 pneumonitis delayed appropriate intervention. Case description A 38-year-old male from Liberia presented in March 2022 with progressive shortness of breath, fatigue, arthralgia, and generalised weakness. He was initially evaluated by rheumatology for possible undifferentiated inflammatory arthritis and autoimmune ILD, and commenced on oral corticosteroids and methotrexate. In July 2022, he was hospitalised with worsening breathlessness and a non-productive cough. He tested positive for COVID-19. CT pulmonary angiogram (CTPA) revealed scattered airspace opacities and ground-glass changes, interpreted as COVID pneumonitis. Methotrexate was withheld, and he received dexamethasone. One month later, he was re-admitted with chest pain. CT imaging demonstrated diffuse reticular changes, suggestive of fibrotic lung disease. Multidisciplinary discussion in an ILD meeting concluded ongoing post-COVID interstitial changes. Pulmonary function testing revealed a restrictive defect. He remained on steroids. A repeat CT chest in September 2022 continued to show extensive basal fibrotic changes. Autoimmune screening, including rheumatoid factor, anti-CCP, ANCA, and ANA, returned negative. He was referred to an ILD specialist clinic, where he received intravenous methylprednisolone (1 g for 3 days). A myositis panel subsequently revealed anti-MDA5 antibody positivity. By March 2023, on rheumatology review, he described new dermatological symptoms including hyperpigmented, scaling rashes over the face, hands, and feet; tender palmar papules; and ulcerated lesions on the knuckles. He also reported polyarthritis with morning stiffness involving MCPs, PIPs, wrists, elbows, knees, and feet. Examination revealed erythematous, tender nailfolds; synovitis of multiple MCPs; bilateral Gottron’s papules; and healing rashes across the forehead, nasolabial folds, and cheeks. Fine basal crackles were noted on chest auscultation. A final diagnosis of anti-MDA5 positive dermatomyositis with associated ILD was made. He was treated with two cycles of rituximab, commenced on mycophenolate mofetil, and a steroid taper was initiated. Follow-up pulmonary function tests showed gradual improvement, alongside clinical and dermatological symptom resolution. Discussion Anti-MDA5 dermatomyositis is a distinct phenotype of dermatomyositis, typically presenting with minimal muscle involvement but prominent cutaneous features and a high risk of rapidly progressive interstitial lung disease. Without early diagnosis and aggressive treatment, MDA5-associated ILD can have high mortality. During the COVID-19 pandemic, there has been increasing recognition of phenotypic overlap between COVID-19 and autoimmune conditions like dermatomyositis, including respiratory symptoms, ground-glass changes on imaging, and cutaneous manifestations. This diagnostic ambiguity can delay timely immunosuppression in patients with underlying autoimmune disease. In this case, the initial assumption of post-COVID pneumonitis obscured the underlying MDA5-associated ILD. Despite treatment for presumed viral pneumonitis, the patient’s condition failed to improve. The eventual identification of MDA5 antibodies, in conjunction with cutaneous signs and progressive respiratory decline, confirmed the autoimmune diagnosis. This case reinforces the importance of maintaining a broad differential in post-COVID patients, especially those with persistent symptoms and atypical radiological features. Early use of myositis-specific panels, particularly in patients with subtle cutaneous or articular features, may help detect MDA5-positive dermatomyositis before irreversible pulmonary damage occurs. Key learning points 1. Anti-MDA5 dermatomyositis may present with non-specific symptoms, cutaneous findings, and interstitial lung disease, with little or no muscle involvement. 2. COVID-19 infection and anti-MDA5-associated ILD can present with similar respiratory and imaging features, making early differentiation difficult. 3. Delayed diagnosis of MDA5-positive DM due to misattribution of symptoms to COVID-19 pneumonitis can lead to missed treatment windows for aggressive immunosuppression. 4. Myositis-specific antibody testing should be considered in patients with ILD, skin rashes, and joint involvement, especially when standard treatment for viral or inflammatory conditions fails. 5. Early multidisciplinary input and immunosuppressive therapy are key to improving outcomes in MDA5-DM with RP-ILD.

Introduction Systemic lupus erythematosus (SLE) is characterized by production of antibodies against various nuclear antigens with involvement of multiple organs. Pulmonary manifestations of SLE can include a wide spectrum of diseases such as pleuritis, pneumonia, pulmonary embolism, pneumothorax and pulmonary haemorrhage. Lupus pneumonitis (LP) has an incidence of 1-8% and may be difficult to distinguish from pulmonary infections. We report a case of LP mimicking community acquired pneumonia (CAP) and pulmonary tuberculosis (PTB) admitted to the Internal Medicine ward in Hospital National Guido Valadares, Dili, Timor Leste. Case A 43-year-old female school teacher presented with cough and shortness of breath. She also had a history of fever and malar rash along with significant hair loss. On examination she was febrile and dyspneic, with anaemia, hair loss, malar rash, tachycardia, tachypnoea, and coarse crepitations on chest auscultation. Sputum for Gene Xpert MTB/RIF Ultra test, bacterial culture, and fungal stains were negative. She was initially treated with broad-spectrum antibiotics for CAP, while being investigated for TB. However, as she failed to respond to intravenous antibiotics, further evaluation was done. Anti-nuclear antibodies (ANA) and ds-DNA were strongly positive. Urine analysis revealed nephrotic-range proteinuria. High resolution computed tomography (CT) showed bilateral ground glass changes suggestive of lupus pneumonitis (LP). A diagnosis of SLE with LP was made and the patient was commenced on corticosteroids (pulsed methylprednisolone for three consecutive days, followed by oral prednisolone) which led to a dramatic clinical and radiological response. Conclusion SLE has a wide range of presentations. Keeping this in mind, even in countries where tuberculosis is endemic, the differential diagnosis of SLE and LP should be considered. Many challenges exist in the diagnosis and management of patients with SLE and its complications in resource-limited settings.

Azithromycin-induced allergic reaction – A rare case report and review of literature

Background:Ensuring proper endotracheal tube (ETT) placement is crucial during general anesthesia. Traditional confirmation methods include capnography and chest auscultation, but each has limitations. Ultrasound (USG) offers real-time visualization of ETT placement, potentially improving confirmation speed and accuracy.Objectives:To compare the feasibility of USG for early detection of esophageal intubation against capnography and chest auscultation in trainees.Methodology:This comparative observational study assessed the time for ETT placement confirmation using USG, capnography, and chest auscultation in 90 patients undergoing general anesthesia. Patients were divided into three groups: USG confirmation (Group A), capnography confirmation using the first or sixth waveform (Group B), and chest auscultation confirmation (Group C).Results:Group A had the fastest confirmation time (32.1 s), followed by Group C (bilateral: 46.97 s), and Group B (6th waveform: 48.23 s). Statistically significant differences were observed between Group A and Group B (6th waveform), and Group A and Group C (bilateral). Hemodynamic parameters showed significant changes during and after intubation compared to baseline.Conclusions:USG emerged as a faster and potentially more reliable method for ETT placement confirmation compared to capnography and chest auscultation. The real-time visualization offered by USG is valuable for novice trainees, enabling rapid confirmation, and potentially improving patient safety by facilitating early detection of misplacement.

Thoracic trauma is an important cause of mortality in pediatric trauma, and most thoracic trauma in pediatrics is blunt trauma. Pediatric patients have important anatomic and physiologic differences from adults that must be considered in the setting of blunt thoracic trauma. Some of the more significant differences are related to chest wall structure, laxity of mediastinal fixation points, and increased sensitivity to ionizing radiation. When imaging pediatric blunt thoracic trauma, a chest radiograph is the initial test of choice. Further imaging is often not needed, especially if the initial chest radiograph is normal, and additional findings on computed tomography (CT) often do not impact management. Factors which have been found to increase the utility of chest CT in pediatric blunt thoracic trauma include age 15years or older, abnormal chest auscultation, tachycardia, chest pain, abnormal chest radiograph - especially mediastinal contour abnormality - and severe mechanism. Common injuries in pediatric blunt thoracic trauma include pulmonary contusion, pulmonary laceration, pneumothorax, hemothorax, and rib fractures. Rare but important injuries include cardiac and great vessel injuries, tracheobronchial injuries, esophageal injuries, and diaphragmatic injuries.

Abstract Background Tuberculosis (TB) remains a leading global cause of preventable death, with 10.8 million cases and 1.3 million deaths reported in 2023. Current methods for TB screening include symptom-based screening, and chest X-ray (CXR) with computer-aided detection (CAD-CXR). Each method has limitations related to cost, accessibility, and screening efficacy. As a result, an estimated 2.6 million TB cases were missed in 2023. AI-based TB screening using lung sounds captured by a digital stethoscope offers a potential solution to these challenges, enhancing access, efficacy, and cost-efficiency. Methods A dataset comprising 49,770 anonymized chest auscultation recordings from 1,659 participants (cases and controls) were collected by trained nurses in South Africa’s Western Cape province from June 2021 to November 2022 using AI Diagnostics’ prototype digital stethoscope. Consenting participants suspected to have TB that reported a recent sputum TB Xpert Ultra test were recruited from 34 primary care clinics. After stratification and data preparation, a final dataset of 1,169 participants was partitioned into an 80% training and 20% hold-out test set. A pre-trained transformer- based architecture was fine-tuned using K-fold cross-validation. The ensemble model’s ability to predict pulmonary TB was evaluated on the hold-out test set, with sputum Xpert Ultra as the reference standard. Results The AI model achieved a mean Area under the Receiver Operating Curve (AUC-ROC) of 0.79 (95% CI: 0.73-0.85). At a sensitivity of 89.9% (95% CI: 82.4%-94.4%), the ensemble model has a specificity of 50.4% (95% CI: 42.0%–58.7%) for predicting pulmonary TB using lung sounds. Conclusion AI-based digital chest auscultation for TB, with a sensitivity of 89.9% and specificity of 50.4% in this study, shows early promise as an alternative or adjunct to current TB screening methods. In addition, the method’s portability and low cost have the potential to significantly improve TB screening access. Future independent studies in diverse, unselected populations with high TB prevalence are required to validate model generalizability. Key messages What is already known on this topic Tuberculosis (TB) is a major global health issue, especially in low-resource areas. Existing screening methods like symptom checks, chest X-rays, and CAD tools are often costly, hard to access, or not sensitive enough. AI has shown promise in detecting other lung conditions using sound, but its use for TB screening has not been well studied. What this study adds This is the first large study showing early promise that AI can detect TB from lung sounds using a digital stethoscope. This technology could be further developed as a low-cost and portable screening tool which aligns well with the World Health Organization’s End TB Strategy. How this study might affect research, practice, or policy This study would encourage further research into AI-based auscultation in different populations and settings, helping build more the evidence base supporting the use of AI in disease screening. Further research would also support the development of more accurate and generalizable models. In clinical practice AI-based digital stethoscopes could be used for early TB screening, allowing faster diagnosis and treatment. This would be especially important in asymptomatic TB cases where symptom-based screening would miss all cases. From a policy perspective, the results of this study would support further research which may support the inclusion of this technology in national and global TB screening guidelines and WHO endorsement. This study was commercially funded by the technology provider, AI Diagnostics Pty (Ltd).

Optimal placement of an endotracheal tube (ETT) tip is necessary for safe and effective ventilation of the lungs. However, there is no consensus on the most accurate method for calculating the depth of ET insertion. To compare the accuracy (proportion of optimally placed ETT) of nasal-tragus length (NTL)-based formula, body weight-based formula, and gestational age (GA)-based method in estimating the depth of oral ETT insertion in neonates. Neonates of gestational age 250/7-41+6weeks undergoing oral endotracheal intubation during the first 28days of life were randomized into one of three study groups. The depth of ETT insertion was determined using gestational age-based (Kempley), weight-based (Tochen), or NTL-based methods. The optimal position of the ETT tip, assessed by a blinded radiologist on an anteroposterior chest radiograph, was considered to be between the upper border of T1 and the lower border of T2. In this study, 165 neonates (55 per group) were included. Birth weight, gestational age, proportion of small-for-gestational age neonates, and other baseline characteristics were similar among neonates in the three groups. The rates of optimal ETT placement were not significantly different between the NTL- (47.2%), GA- (45.4%) and weight-based (43.6%) methods (P = 0.911). The corresponding rates of ETT repositioning in the NTL-, GA- and weight-based method groups based on auscultation of chest was 58%, 49% and 29%, respectively (P < 0.001). No significant difference was observed in the accuracy of the three methods studied. Better methods are needed to calculate the depth of ETT insertion. Prospectively registered with the Clinical Trial Registry of India (CTRI/2022/12/048221).

Emergency intubation was done with an endotracheal tube 7.5 mm and put on mechanical ventilation in AC-VC mode, FiO 2 : 60%, TV: 320 mL, and positive end-expiratory pressure (PEEP): 6 cm H 2 O.The patient was shifted to the intensive care unit (ICU) for further management.After a few hours of treatment, neurological examination showed improvement with GCS of E2VTM5 with no plantar reflex, while bilateral pupils were normal and sluggishly reactive to light.On chest auscultation, bilateral air entry with diffuse crepitations was present.Arterial blood gas (ABG) analysis showed respiratory acidosis, and chest X-ray showed diffuse lung opacity (Fig. 1).Baseline measurements were hemoglobin 15.1 gm/dL, total leukocyte count 10.61/mm 3 , prothrombin time 18.6 seconds, international normalized ratio (INR) 1.5, and activated partial thromboplastin time 27.4 seconds.Her serum urea and creatinine were 29.3 and 0.43 mg/dL, respectively.

PV280 / #741Case Report Poster Topic:AS03 - Antiphospholipid SyndromeLate-Breaking AbstractIntroductionCardiac manifestations in primary antiphospholipid syndrome (APS) range from mild valvular disease, to more devastating disorders associated with morbidity and mortality. Cardiomyopathy has seldom been reported as the initial manifestation of the disease. Therefore, we present the case of a 47 year-old lady with dilated cardiomyopathy (DCM) as the initial presentation of APS.Case Presentation With InvestigationA 47-year-old lady, previously healthy with no known comorbidities, presented with acute dyspnea. This started hours prior to presentation. She described orthopnoea as well as gradual worsening of lower limb edema in the preceding days. The patient had no cardiac family history, and was a nonsmoker and teetotal. Chest auscultation revealed crackles up till the lung apices. Further examination confirmed an elevated jugular venous pulse, as well as pitting lower limb edema extending to the thighs bilaterally. Oxygen saturations were 89% on room air, correcting with oxygen. Inspection revealed livedo reticularis of the thighs and arms. A bedside echocardiogram revealed severe diastolic dysfunction. An NT-proBNP assay was elevated at 35,000 pg/mL. The patient was admitted under the cardiologists for diuresis and investigation. The patient underqent a coronary angiogram, which showed fully patent coronary arteries. A Cardiac MRI confirmed the presence of an advanced DCM, with thickening of the mitral and tricuspid valvular apparatus. Rheumatology was consulted in view of these findings. The patient tested positive for lupus anticoagulant, anti-β2 glycoprotein antibodies (IgG and IgM &gt; 200 IU/mL) and anticardiolipin antibodies (IgG and IgM &gt; 120 IU/mL). A double-stranded DNA antibody assay was negative, with normal complement protein levels. She denied a history of thrombotic episodes and miscarriages. A diagnosis of primary APS causing DCM was made, and warfarin, corticosteroids, mycophenolate mofetil and heart failure optimization were initiated. The patient subsequently improved and was discharged home.Literature ReviewThe etiological basis of DCM in APS is hypothesized to be microvascular thrombotic insults,[1] as evidenced by autopsy studies. Recurrent microthrombotic inflammatory activity has been associated with a risk of progression to DCM, with contributions from myofibroblasts and fibromuscular remodeling of the myoendocardium. Tumor necrosis factor alpha and transforming growth factor beta are the main cytokines implicated in this process. APS patients, particularly primary APS patients and those with strongly positive serology, have been shown to exhibit asymptomatic diastolic dysfunction in up to 20% of cases.[1] This suggests that the remodeling changes in the myocardium are asymptomatic and subclinical, yet capable of having catastrophic consequences. Immunosuppression and anticoagulation have shown success in the sparse case reports of APS-DCM in the literature, particularly with mycophenolate and IVIG.[1]DiscussionThe key learning points from this case are: 1. All women of middle-age and younger, presenting or having been diagnosed with DCM, should be screened for APS. 2. Cardiac MRI was, in this case, a reliable surrogate to endomyocardial biopsy. Further studies are needed to validate this. 3. Female sex, younger age and strongly positive serology are associated with an increased risk of DCM in APS patients. 4. Up to 20% of APS patients can have subclinical diastolic dysfunction. The authors recommend echocardiographic screening in view of the events of this case. 5. The etiology of APS-DCM is related to microvascular thrombosis, and therefore coronary angiography and macrovascular imaging will be normal in such cases. 6. Management involves warfarinization and immunosuppression with steroids and DMARDs.Reference:[1.] Coletto L. Autoimmun Rev 2022;21:102990.

Abstract Rationale: Guidelines for screening for interstitial lung disease (ILD) in patients with systemic autoimmune rheumatic diseases (SARDs) have recently been published by the American College of Rheumatology and American College of Chest Physicians. We assessed practices for screening for SARD-ILD among physicians in central and Eastern Europe. Methods: Pulmonologists and rheumatologists from central and Eastern Europe who were treating patients with lung fibrosis were surveyed using Survey Monkey between February and April 2024. Physicians were asked whether they proactively screen patients with SARDs at risk for ILD and if so, which tools they usually use. Responses were analyzed according to specialty (pulmonologist or rheumatologist) and level of healthcare (secondary care [private practice, private hospital, community hospital] or tertiary care [tertiary hospital, university clinic]). The average use of screening tools per specialty and per level of healthcare was calculated across countries with ≥9 respondents. Results: A total of 1160 physicians (654 pulmonologists, 506 rheumatologists) from 13 countries (Austria, Bulgaria, Croatia, Czech Republic, Estonia, Hungary, Israel, Kazakhstan, Latvia, Poland, Romania, Serbia, Slovakia) provided responses to at least one question about screening tools. Use of all screening tools was higher among physicians working in tertiary care than secondary care (Figure). In secondary care, among pulmonologists and rheumatologists, respectively, 46% and 52% screened for SARD-ILD using chest auscultation, 45% and 48% using forced vital capacity (FVC), and 43% and 47% using HRCT. In tertiary care, among pulmonologists and rheumatologists, respectively, 65% and 73% screened for SARD-ILD using chest auscultation, 67% and 72% using FVC, and 64% and 71% using HRCT. Lung ultrasound was the least frequently used screening tool, used by fewer than 30% of physicians in both secondary and tertiary care. Conclusions: A survey of pulmonologists and rheumatologists from central and Eastern Europe suggested that guideline-recommended screening tools for SARD-ILDs, such as lung function tests and HRCT, are underused, especially in secondary care. Lung auscultation was only used for screening for SARD-ILDs by about half of the respondents working in secondary care.

Abstract Introduction: Lutetium Lu 177 dotatate (Lu-177) is a peptide receptor radionuclide therapy (PRRT) that is used in the treatment of neuroendocrine tumors (NETs). There have been reports of Lu-177 hypersensitivity reactions including angioedema during post-marketing surveillance. However, Lu-177 related pulmonary toxicity has not previously been described. Case: A 71-year-old female with metastatic pancreatic NETs presented to the emergency department with severe dyspnea. The patient received an initial dose of Lu-177 the day prior. The patient reported that, within hours of receiving Lu-177, she felt rigors, fatigue, and dyspnea. Upon presentation to the emergency department, the patient appeared in respiratory distress. She was hypoxemic with an SpO2 of 81%. Chest auscultation was notable for fine bibasilar crackles. She was not edematous. CT scan of the chest revealed diffuse ground glass opacities, nodular consolidation, and interlobular septal thickening (figure 1). Diagnostic evaluation yielded no evidence of bacterial or viral pneumonia, Pneumocystis pneumonia, cardiogenic pulmonary edema, or pulmonary embolism. The patient was treated with high flow nasal oxygen at a rate of 40 liters per minute and an oxygen fraction of 90%. Corticosteroids were administered with rapid improvement in respiratory impairment over the following 72 hours. She was discharged from the hospital after 7 days of treatment. Discussion: Here we identify a potentially novel case of acute pulmonary hypersensitivity reaction to Lu-177. Post-marketing reports of hypersensitivity reactions to Lu-177 including angioedema exist, however frequency of these reactions and a causal relationship with Lu-177 have yet to be established. Additionally, no reports exist describing pulmonary toxicity following exposure to this medication. We hypothesize that the CT findings in this case are representative of increased vascular permeability secondary to a drug induced hypersensitivity reaction. We note that the patient had previously been treated with everolimus. Retrospective analysis of everolimus induced pneumonitis report a median onset of symptoms of 3.6 months, however our patient tolerated this medication for over 9 years and it was discontinued prior to starting Lu-177. Overall, the timing of the patient's illness and the rapid response to treatment favors a reaction to Lu-177 rather than pneumonitis due to everolimus.

Abstract Introduction: Birt-Hogg-Dubé syndrome (BHD) is a rare autosomal dominant genetic disorder characterized by skin fibrofolliculomas, lung cysts, increased risk of spontaneous pneumothorax, and a predisposition to renal tumors, primarily chromophobe renal cell carcinoma and oncocytomas. This condition results from mutations in the FLCN gene, a tumor suppressor gene. Case Presentation: A 38-year-old male presented to the emergency department with complaints of shortness of breath and palpitations. His medical history includes a congenital atrioventricular (AV) canal defect, repaired at age 3, and placement of a pacemaker at age 26 for a complete heart block. At age 33, he was diagnosed with atrial flutter, most likely induced by mitral valve regurgitation detected on echocardiogram, and anticoagulation therapy was subsequently initiated. At age 35, he was admitted for a spontaneous left-sided pneumothorax. During this presentation, his initial vitals showed a heart rate of 167 beats per minute, blood pressure of 122/83 mmHg, oxygen saturation of 97% on room air, and he was afebrile. An EKG showed atrial flutter and lab tests were unremarkable except for an elevated N-terminal pro-B-type natriuretic peptide of 6680 pg/mL. The patient was treated with diltiazem and furosemide. On physical examination, small, raised, whitish papules were noted on both cheeks. Chest auscultation was clear but he has a systolic heart murmur. A CT angiogram with a pulmonary embolism (PE) protocol excluded PE but identified a small right-sided pneumothorax and multiple small lung cysts. Due to the pneumothorax's size, no chest tube was inserted; instead, serial X-rays were used for monitoring. A skin biopsy of the facial lesions, performed by dermatology, confirmed fibrofolliculomas, characteristic of BHD syndrome. He was discharged pending genetic studies results. Following pneumothorax resolution, the patient will undergo a full pulmonary function test for risk stratification. Pleurodesis might be considered to prevent recurrence. He remains under surveillance for renal tumors with an annual renal ultrasound. Discussion: This case highlights the importance of considering BHD in patients presenting with recurrent pneumothoraces and dermatologic findings (fibrofolliculomas, trichodiscomas, and acrochordons), particularly fibrofolliculomas. Although pneumothorax and pulmonary cysts are hallmark features of BHD, dermatologic manifestations such as fibrofolliculomas can provide critical diagnostic clues. Early recognition of BHD is crucial, enabling timely surveillance for renal neoplasms and implementation of personalized management strategies aimed at minimizing morbidity and improving patient outcomes.

Abstract Introduction Invasive aspergillosis presenting as mediastinal mass and mimicking malignancy is rare. We describe a case of Apergillosis presenting as mediastinal mass in an immunocompetent male. Case report A 40-year-man presented with cough with scanty mucoid expectoration, low grade fever, hoarseness of voice, loss of appetite and weight for two months, and streaky hemoptysis for seven days. He had no chest pain, difficulty in swallowing, facial edema, or neck vein engorgement. There was no history of diabetes or diagnosed immunodeficiency. General examination and routine investigations were unremarkable. On chest auscultation, decreased intensity of vesicular breath sounds were heard in left infraclavicular and mammary areas. Other system examination including genital examination was unremarkable. Chest radiograph showed a homogenous opacity occupying left upper and mid zone. Contrast enhanced computed tomography (CECT) chest showed a heterogeneously enhancing soft tissue lesion in the prevascular mediastinal compartment causing invasion of the mediastinal vasculature and left main bronchus (Panel A). Tuberculin skin test was strongly positive. Tumor markers (alpha fetoprotein, lactate dehydrogenase) were negative. He received antitubercular treatment without any response. For further evaluation, flexible bronchoscopy performed showed left vocal cord palsy, left main bronchus narrowing due to extrinsic compression and mucosal infiltration. Endobronchial biopsy did not contribute to diagnosis. He lost follow up. At another hospital, a CT guided biopsy from the lesion showed few atypical cells, and he was started on Gefitinib. In view of worsening condition and new onset left sided chest pain and exertional breathlessness for last six months, he presented to us. Repeat endobronchial biopsy was non-contributory. We then performed CT guided biopsy from the mediastinal lesion which revealed cores of fibrocollagenous tissue with multiple epitheliod cell granulomas and foreign body giant cells (Panel C). Periodic acid Schiff highlighted septate fungal hyphae favouring Aspergillus (Panel D). Disseminated aspergillosis was ruled out. He received Voriconazole for twelve months following which his symptoms improved and CT showed almost complete resolution of the lesions(Panel B). Discussion Mediastinal aspergillosis presenting as a mass is rare, and can mimic malignancy. In tuberculosis endemic countries, it can be misdiagnosed as tuberculosis which can result in delayed diagnosis and treatment. This case highlights that aspergillosis can affect immunocompetent individuals and efforts should be made to establish pathological or microbiological diagnosis of mediastinal mass.