The role of autoantibody-producing B-cells in connective tissue diseases (CTD) has recently been highlighted by the successful treatment with CD19-targeting CAR T cells. Detrimental effects of autoantibodies are linked to the formation of deposited IgG complexes and the activation of immune cells via Fcγ-receptors (FcγRs). The role of circulating immune complexes (cICs) as link between adaptive and innate immunity has remained understudied. Clinical testing of cICs was hindered by the lack of reliable detection methods. The aim of this study was to determine the potential of IgG-containing cICs to activate FcγRs (their bioactivity) using a new detection method. A reporter cell platform was used to assess the presence and bioactivity of cICs in IgG-autoantibody-positive CTD patients (cross-section analysis) and in patients treated with CD19.CAR T cells (longitudinal analysis). The bioactivity of cICs in the cohort of patients with CTDs was significantly higher compared with healthy controls and patients with IgG-autoantibody-negative systemic inflammatory disease (psoriatic arthritis). Analyses of individual diseases revealed the presence of cICs in the sera of all CTDs, including systemic sclerosis (SSc) and primary Sjögren's syndrome (SjS), although there was significant heterogeneity among individuals. Within SSc, patients positive for anti-topoisomerase-I (Scl70) autoantibodies, diffuse cutaneous and lung involvement had significantly enhanced cIC bioactivity. Finally, the bioactivity of cICs was significantly reduced in CTD patients after CD19-CAR T cell therapy. Our study reveals the presence of FcyR-engaging cICs in CTDs and demonstrates that the bioactivity of cICs is correlated with clinical phenotypes and treatment outcomes.

Abstract Background: The COVID-19 pandemic has created a critical global situation, causing widespread challenges and numerous fatalities due to severe respiratory complications. Since lung involvement is a key factor in COVID-19 diagnosis and treatment, accurate identification of infected regions in lung images is essential. Methods: We propose a multiphase segmentation method based on the level set framework to determine lunginvolved areas. The shearlet transform, a high-precision directional multiresolution transform, is employed to guide the gradient flow in the level set formulation. Additionally, the phase stretch transform (PST) is applied to enhance the contrast between infected and healthy regions, improving convergence speed during segmentation. Results: The proposed algorithm was tested on 500 lung images. The method accurately identified infected areas, enabling precise calculation of the percentage of lung involvement. The use of the shearlet transform also allowed clear delineation of ground-glass opacity boundaries. Conclusion: The proposed multiphase level set method, enhanced with shearlet and phase stretch transforms, effectively segments COVID-19–infected lung regions. This approach improves segmentation accuracy and computational efficiency, offering a reliable tool for quantitative lung involvement assessment.

A 55-year-old woman was diagnosed with anti-melanoma differentiation-associated gene 5 (anti-MDA5) positive dermatomyositis (DM) in 2020, having low grade fever, weight loss, arthritis in small joint of hands, erythematous-desquamative lesions on hands, cuticle dystrophy and severe skin ulcerations. Firstly, she was treated with cyclosporine (CsA), soon discontinued due to gastrointestinal intolerance. She was subsequently treated with steroid pulses, hydroxychloroquine (HCQ) and mycophenolate (MMF), without improvement. In March 2021 she started therapy with intravenous immunoglobulins (IVIG) and prostanoids, leading to ulcer improvement, but stopped due to gastrointestinal intolerance. A chest high resolution computed tomography (HRCT) done in June 2021 showed interstitial lung disease (ILD). In September 2021 rituximab (RTX) was stopped at the first infusion due to gastrointestinal intolerance. From January 2022 the patient also started to walk with difficulty due to development of deep asthenia. Therapy with various Jak inhibitors was started (first tofacitinib, then baricitinib, finally upadacitinib), leading to improvement of cutaneous ulcers, but stopped every time after a few months due to gastrointestinal intolerance and dizziness. In August 2023 during hospitalization a spirometry showed reduction of diffusion capacity of the lung for the carbon monoxide (DLCO). In June 2024, in consideration of poor disease control and refractoriness of the disease (loss of appetite and weight, worsening of asthenia which forced the patient into a wheelchair, persistence of polyarthritis, skin ulcers, alopecia, Gottron’s signs, radiological progression of ILD), she was hospitalized again and Anifrolumab (ANI) was started in July 2024 (300 mg IV every four weeks). After four infusions the patient reported improved appetite with significant weight gain, resolution of arthritis and disappearance of cutaneous ulcers, Gottron’s sign and alopecia. In February 2025, after seven ANI infusions, a HRCT demonstrated a significant radiological improvement of the ILD compared to 2024, and spirometry showed significant improvement of DLCO compared to 2023. In this period, no adverse effects were observed from the new therapy. After twelve total infusions, constitutional, articular and cutaneous involvement remained in good control. This case suggests the potential efficacy of ANI in refractory anti-MDA5-positive DM not only on skin manifestation, but also on articular and lung involvement.

Lung involvement in patients with leptospirosis is associated with a more complicated disease course. However, the demographic and clinical associations of lung involvement are incompletely defined, and its optimal management is uncertain. This retrospective study examined consecutive patients admitted to a referral hospital in tropical Australia, with laboratory-confirmed leptospirosis between January 2015, and June 2024. Lung involvement was defined as new lung parenchymal changes on chest imaging at any point during the patients’ hospitalisation. The demographics, clinical findings and clinical course of the patients with and without lung involvement were compared. The median (interquartile range (IQR)) age of the 109 patients was 39 (24–56) years; 93/109 (85%) were male. Lung involvement was present in 62/109 (57%), 55 (89%) of whom had no documented comorbidities. Patients with lung involvement received antibiotics later in their disease course than those without lung involvement (after a median (IQR) of 5 (4–6) versus 3 (2–5) days of symptoms, p = 0.001). Lung involvement was frequently associated with multi-organ failure: patients with lung involvement were more likely to require intensive care unit admission than patients without lung involvement (41/62 (66%) versus 15/47 (32%), p < 0.001). Overall, 30/109 (28%) satisfied criteria for acute respiratory distress syndrome (ARDS) and 26/109 (24%) developed pulmonary haemorrhage. Patients with lung involvement received cautious fluid resuscitation, vasopressor support and prompt initiation of additional supportive care—including mechanical ventilation, renal replacement therapy and extracorporeal membranous oxygenation—guided by the patients’ physiological parameters and clinical trajectory. All 109 patients in the cohort were alive 90 days after discharge. Life-threatening lung involvement was identified in the majority of individuals in this cohort and occurred in young and otherwise well individuals. However, in Australia’s well-resourced health system excellent outcomes can be achieved using a standard contemporary approach to the management of a patient with undifferentiated infection while a confirmed diagnosis of leptospirosis is awaited.

Background. Systemic Lupus Erythematosus (SLE) is a chronic autoimmune disease that can significantly affect the lungs leading to irreversible damage, resulting in reduced life quality and varied mortality rates. It can impact all parts of the lung although respiratory symptoms display later. Despite the high frequency, lung involvement in SLE remains understudied. Objectives. To evaluate the extent of pulmonary involvement in SLE patients and identify factors associated with its occurrence and severity. Materials and Methods. A retrospective cohort study from January 2024 to January 2025 was conducted at our hospital, including SLE patients >18 years old, fulfilling the ACR and/or SLICC diagnostic criteria. Data regarding demographics, disease, pulmonary involvement, and laboratory markers were extracted from the hospital records. R software version 4.2 was used to carry out statistical analysis. Results. 45 SLE patients were included; 44.4% (n = 20) were aged 41 years and older, 93.4% (n = 42) females. Around 17.7% had Kidney Disease (KD), 11.1% had hypertension, 4.4% had diabetes and 2.2% had hypothyroidism and deep vein thrombosis. Pulmonary involvement was present in 22 (48.8%) SLE patients, in the form of pleural effusion (45.5%), interstitial lung disease (13.6%), lupus pneumonitis (13.6%), pleuritic (13.6%), pulmonary embolism (9.3%) and pulmonary hemorrhage (4.5%). The most frequent respiratory symptoms reported by SLE patients were: dyspnea (25%), chest pain (20%), and cough (20%) while fever (16.7%), palpitation (13.8%), and hemoptysis (4.5%) were less frequent. KD and hypertension were significantly associated with pulmonary involvement; (χ2=3.307, p=0.026) and (χ2=7.762, p=0.02), respectively. Even the seropositivity for ANA, anti-DNA (ds), and low C4 were significantly associated with pulmonary involvement, (χ2=3.237, p=0.021), (χ2=4.672, p=0.023) and (χ2=8.2467, p=0.01), respectively. Conclusions. More than one-fifth of SLE patients develop pulmonary complications with pleural effusion being the most common manifestation. Dyspnea, chest pain, and cough are the most frequent respiratory symptoms reported in these patients. KD, hypertension, seropositivity for ANA and anti-DNA (ds) with low C4 were significantly associated with lung involvement. This study provides valuable insights into factors linked to pulmonary involvement in SLE further contributing to a better understanding of this complex autoimmune disease toward proactive management to improve patient outcomes.

Introduction: Systemic sclerosis (SSc) is a connective tissue disease affecting the skin and internal organs, particularly the lungs, leading to significant morbidity and mortality. The objective of our study was to estimate the frequency of interstitial lung disease (ILD) in SSc and to investigate its characteristics. Patients and methods: This retrospective study analyzed 329 patient records of individuals hospitalized in the rheumatology department between 1986 and March 2018 for systemic sclerosis (meeting ACR and/or LeRoy-Medsger criteria) with interstitial lung disease. SSc cases associated with other connective tissue diseases were excluded. Results: One hundred and seventy-eight patients (54%) had ILD. The majority were women (approximately 85%), with a mean age of 47.5 years. SSc was limited cutaneous in 60% of cases, diffuse cutaneous in 39%, and sine scleroderma in 2 cases. ILD was asymptomatic in 31% of patients. High-resolution chest CT (HRCT), performed in all patients, revealed ground-glass opacities in 87.64% of cases, a honeycomb pattern in 32.46%, reticular opacities in 22.5%, bronchiectasis in 28%, nodules in 19.66%, silicosis in 6.2%, and pleural effusion in 3 patients. ILD was classified as nonspecific interstitial pneumonia (NSIP) in 85% of cases and usual interstitial pneumonia (UIP) in 9.55%. Pulmonary function testing showed a restrictive pattern in 64% of cases. Antinuclear antibodies were positive in 92% of patients, with antitopoisomerase I antibodies in 56% and anti-centromere antibodies in 5%. Immunosuppressive therapy was initiated in 40% of patients, with three patients receiving rituximab. Additionally, 77% of patients received low-dose corticosteroid therapy (<15 mg/day), and 53% were vaccinated against pneumococcus. Conclusion: Interstitial lung disease (ILD) is common in systemic sclerosis, most frequently presenting as nonspecific interstitial pneumonia (NSIP). It can be asymptomatic, highlighting the need for systematic screening using HRCT and pulmonary function testing.

Background: The use of prone positioning to treat patients with ARDS due to COVID-19 (C-ARDS) is well documented, being valid its use in a widespread manner and as a rescue strategy in catastrophic respiratory failure. However, the evidence is scarce in those where prone positioning (PP) is later than 36 hours after admission to the intensive care unit (ICU). Objectives: To compare the sociodemographic and clinical characteristics of patients with C-ARDS who required prone positioning after 36 hours from admission to the ICU until hospital discharge. Methodology: Retrospective observational cohort study, with a final sample of 23 patients (59.6 ± 11.2 years, 65.2% men). Who met the inclusion and exclusion criteria. The data were obtained from the electronic Clinical Administration System (SAC) and from the Kinesthetic care registry. The following information was obtained; socio demographic, clinical characteristics, imaging and laboratory tests, pulmonary severity, ventilatory mechanics, time prior to PP, days on mechanical ventilation (MV), in ICU, in hospital and mortality. Conclusions: Patients with C-ARDS who required PP after 36 hours were mostly obese adults who presented more lung involvement in posterior and basal segments. After PP, there was favorable oxygenation in most patients without the need for initial mechanical ventilator intervention. Most patients required tracheostomy and connection to MV after ICU stay, with a hospital stay of more than 30 days.

Background. Fibromyalgia (FM) is a complex syndrome characterised by chronic pain, fatigue and functional symptoms; it is often accompained by mood disorders. FM affects, on average, 2.3% of the European population with a profound impact on both physical and social aspects of quality of life (QoL). FM is known to be a frequent comorbidity in Connective Tissue Diseases (CTDs). Few data are available at present about the prevalence of FM in Idiopathic Inflammatory Myopathies (IIMs) and about the profile of IIM patients with FM. The objective of the study was to determine the prevalence of FM in a monocentric cohort of IIMs patients, evaluating possible correlations with clinical features, disease activity and parameters of QoL. Materials and Methods. We retrospectively analyzed medical records of consecutive patients diagnosed with IIM according to the EULAR/ACR 2017 criteria and regularly followed at our specialistic outpatient Myositis Clinic. We collected data about demography, subset and disease duration, organ involvement, autoantibody profile and comorbidities. Patients’ QoL was investigated through the following Patient Reported Outcomes (PROs): Short Form 36 (SF36), Hospital Anxiety and Depression Scale (HADS), Health Assessment Questionnaire (HAQ). The diagnosis of FM was confirmed following the 2016 American College of Rheumatology Fibromyalgia Diagnostic Criteria. Intergroups comparisons were assessed by using Chi-square, t-test and ANOVA. P values <0.05 were considered significant. Results. A total of 184 patients were enrolled, 119 (64,7%) female, with a mean age of 66,5±11.2 years and a mean disease duration of 9.9±5.2 years. Twenty-three patients (12,5%) had FM, all of them were female. Taking into account epidemiological and clinical characteristics of patients, a diagnosis of FM was significantly associated with female sex (p<0.001), muscular involvement (p=0.015), lung involvement (p=0.036), sicca syndrome (p=0.004). Regarding comorbidities, a significant correlation was highlighted between FM and obesity (p=0.024), osteopenia (p=0.026) and thyroid dysfunction (p=0.05). PROs analysis showed patients with FM had significantly higher values of HAQ (p=0.005) and significantly lower values on different items of SF36 (Physical Function p=0.019, Bodily Pain p=0.006, Vitality p=0.026). Conclusions. Our data showed, as expected, that the prevalence of FM in IIMs patients was higher than in the general population and similar to that of other CTDs (4,5). Moreover, these results confirm the deep relationship between FM and both female gender, sicca syndrome and thyroid dysfunctions. However, they also showed a higher risk of FM in IIMs patients with muscle or pulmonary involvement, or with a reduced bone mineral density, or in obese patients. PROs’ analysis confirmed a significant impact of FM on both physical and mental outcomes of IIM patients. Although preliminary, these results could help rheumatologists in profiling the characteristics of FM patients in IIMs in their routinary assessment, thus aiming at improving the management of this potentially disabling condition.

Background. Anti-synthetase syndrome (ASS) is a systemic autoimmune disease frequently associated with lung involvement. This single-center retrospective study aimed to longitudinally assess the clinical and instrumental evolution of patients with ASS and Interstitial Lung Disease (ILD) and to evaluate the rate of disease progression. Materials and Methods. Clinical and instrumental data, including spirometric parameters (%pFVC and %pDLCO), radiological patterns, and fibrotic extent according to HRCT visual scoring, were collected at three time points: baseline (first visit at our center), 12 ± 3 months, and 24 ± 3 months. Progressive ILD at 12 and 24 months was defined according to the ATS/ERS/JRS/ALAT 2022 guidelines (presence of at least two among worsening respiratory symptoms, decline in %pFVC ≥ 5% or in %pDLCO ≥ 10%, or radiological progression) and the INBUILD trial criteria (at least one among %pFVC decline ≥ 10%, 5–10% decline in %pFVC with worsening symptoms or radiological progression, or worsening of symptoms and radiological progression). Predictors of progression were analyzed. Results. Thirty-three patients with ASS-ILD were included. Patients and disease characteristics are summarized in Table 1. Twenty-five patients (76%) presented pulmonary involvement at disease onset. The most frequent radiological pattern was NSIP, observed in 23 patients (70%), followed by UIP in 6 patients (18%), OP in 2 patients (6%), and mixed NSIP-OP in 2 patients (6%). Fibrotic extent on HRCT was ≤10% in 15 patients (46%), 10–20% in 10 patients (30%), and >20% in 8 patients (24%). The distribution of NYHA functional class for dyspnea across the three time points is shown in Graphic 1. We observed an improvement from baseline in NYHA functional class for dyspnea (2, 4, and 2 patients moved from class IV, III, and II to class I, respectively, at both 12 and 24 months), as well as an improvement in exertional dyspnea at 24 months [24 (73%) vs 15 (45%); p = 0.0242], together with a progressive increase in %pFVC values at the two time points (Graphic 2, Table 2). Five (15%) and four (12%) patients were classified as progressors at 12 and 24 months, respectively. The characteristics of patients with INBUILD-defined progression at 24 months compared with those without progression are summarized in Table 3. No predictor of ILD progression was identified. Conclusions. In our cohort of patients with ASS-ILD, the %pFVC and NYHA functional classes improved over 12 and 24 months of follow-up. The proportion of progressors identified according to the two criteria was reduced (15% and 12%). No predictor of progression was identified.

Background. Abatacept has been suggested to be a safe and effective drug for the treatment of rheumatoid arthritis (RA) complicated by interstitial lung disease (ILD), one of the most severe extra-articular manifestations of RA. Objectives. In this prospective multicentre observational study, we aimed to evaluate the effectiveness and safety of abatacept on RA-ILD. Concurrently, the study aimed to evaluate the incidence of new diagnosis of ILD in RA patients treated with abatacept. Methods. We enrolled all consecutive RA patients already under treatment with abatacept for at least three months referred to 12 rheumatological centres in Italy. ILD patients were followed according to the current clinical practice: forced vital capacity (FVC%) was repeated every six months, while chest high resolution computed tomography (HRCT) was performed annually or when respiratory symptoms worsened. In patients without ILD at baseline, a careful assessment for respiratory symptoms and velcro crackles was performed at enrolment and every six months. HRCT was requested in case of new-onset dyspnoea, persistent dry cough, or detection of velcro crackles. Results. We enrolled 325 RA patients treated with abatacept. Among them, ILD was already present in 20.6% of cases. A usual interstitial pneumonia (UIP) pattern was detected 25.4% of cases. The two-year retention rate of abatacept was 79.6% ±3.8 and 76.8%±10.6 in patients without and with ILD, respectively, without difference between the two groups. Two cases of infectious pneumonia were reported in ILD group, requiring a transient discontinuation of abatacept. No patients discontinued abatacept for a worsening of lung function, and no episodes of acute exacerbation of ILD were observed during the study. At the end of follow-up, the radiological picture remained stable in 71.6% of subjects, improved in 13.4%, and worsened in 14.9%. A worsening at HRCT was recorded in 29.4% of cases of UIP, and only in 10% of patients with other ILD patterns (p not significant). Regarding lung function tests, FVC improved in 13.4% of cases, remained unchanged in 74.6%, and worsened in 11.9%. During the 24-month period of follow-up, 2 patients developed ILD, with an incidence of 0.39 new ILD cases/100 patients/year. Conclusions. Our study confirmed the safety and the effectiveness of abatacept in this population of difficult to treat RA patients. Of interest, ILD didn’t affect the retention rate of the drug. Moreover, lung function and HRCT features improved or remained stable in about 85% of patients, suggesting a role for abatacept in the treatment of RA-ILD. Concurrently, we observed a low rate of new cases of ILD, with an incidence rate of 0.39 new ILD cases/100 patients/year. Controlled studies, specifically developed in RA-ILD population, will be necessary to define the efficacy and safety of abatacept compared to other biologic DMARDs.

Pneumonia is as an acute respiratory infection that affects lung tisuue and stands as one of the primary causes of mortality among children worldwide.. According to the World Health Organization (WHO, 2022), pneumonia is caused by infectious agents such as Streptococcus pneumoniae, Haemophilus influenzae type b (Hib), and Respiratory Syncytial Virus (RSV). UNICEF (2024) reports that more than 700,000 children under the age of five die annually due to pneumonia. Radiological chest X-ray examination plays an essential role in detecting pulmonary abnormalities such as infiltrates, consolidation, and bronchopneumonia (Az et al., 2021). It also assists in distinguishing between bacterial and viral pneumonia (Aprilia et al., 2024). Objective to determine the radiological characteristics of chest X-ray findings in pediatric patients with pneumonia at Royal Prima Ayahanda Hospital Medan. Methods this research employed a descriptive retrospective study design using a cross sectional approach. Secondary data were collected from the medical records of pediatric pneumonia patients at Royal Prima Ayahanda Hospital, Medan, during 2022–2023. A total of 57 pediatric patients who met the inclusion and exclusion criteria were included. Data were analyzed univariately and presented in frequency and percentage distributions based on variables such as age, gender, infiltrate, and consolidation findings. The results showed that the most common age group was 0–1 years (42.1%), with females predominating (61.4%). Chest X-ray examination revealed that 75.4% of patients had abnormal radiological findings. The most frequent feature was pulmonary infiltrate (77.2%), while consolidation was found in 21.1% of patients. Conclusion most pediatric pneumonia patients demonstrated abnormal radiological chest X-ray findings, predominantly alveolar infiltrates. Chest X-ray examination remains an important diagnostic tool in assessing lung involvement and determining the severity of pneumonia in children.

Background. Interstitial lung disease (ILD) is a major complication in systemic sclerosis (SSc) patients, associated with substantial morbidity and mortality. Functional, imaging, and clinical measures of lung involvement could be biased in SSc due to its multiorgan nature and extra-articular involvement (e.g., cardiac, musculoskeletal). Artificial intelligence (AI) reading of high-resolution computed tomography (HRCT) has emerged as a novel tool for the objective and reliable assessment of pulmonary diseases. The aim of this study is to correlate AVIEW measures, an Deep learning based software for HRCT image assessment, with ILD-progression and disease-related mortality in SSc patients. Materials and Methods. The AVIEW software (Coreline Soft, South Korea) was employed to analyze HRCT images from a cohort of consecutive SSc-ILD patients at baseline and after 24±3 months. Quantitative analyses included lung volume, texture, airways, and vascular anatomy. Baseline metrics were assessed for their association with ILD progression, defined by clinical, functional, and imaging criteria based on the INBUILD study parameters over 24 months. Furthermore, changes in AVIEW-derived measurements between consecutive HRCT evaluations over the 24-month period were analyzed for their association with SSc-related mortality during the subsequent 36 months. All absolute measurements were normalized to body surface area. Results. A total of 146 HRCT scans from 73 SSc-ILD patients were assessed (mean age 58.4±14.3 years; male 16.4%; diffuse skin variant 49.3%). Thirty-one patients (42.4%) experienced ILD progression, which was predicted at baseline by higher percentages of ground glass opacities (GGO) (p=0.05) and reticulation (p=0.05), higher subpleural vessel volumes (p=0.017), and a tendency toward larger distal airways (p=0.066). Serial evaluations demonstrated that INBUILD progression was associated with a reduction in the percentage of normal lung (p=0.044) and absolute volumes (p=0.009), without significant changes in reticulation, GGO, vessels, or airways when considered individually. Twelve patients died due to SSc within 36 months following the second HRCT evaluation. Patients in the upper quartile for changes in reticular score and airway volume exhibited a higher mortality risk, independent of INBUILD progression (reticular score: OR 3.30, 95% CI 1.03–10.61, p=0.045; airway volume: OR 3.37, 95% CI 1.08–10.51, p=0.036) Conclusions. Deep learning-based assessment in SSc-ILD identified distinct modifications and prognostic significance in lung anatomical components, offering potential improvements in patient evaluation and stratification beyond conventional clinical tools.

Background. Pulmonary hypertension (PH) is one of the most severe and potentially life-threatening complications of systemic sclerosis (SSc), with a major impact on prognosis and patient quality of life. It is often related to microvascular dysfunction and, in some cases, to interstitial lung involvement. Early diagnosis is essential but remains challenging, as initial symptoms are frequently nonspecific and may overlap with other manifestations of the disease. In this context, the use of multimodal screening strategies combining clinical, functional, imaging, and microvascular assessments may significantly improve early identification of patients at risk. Objectives. To assess the prevalence of PH in a cohort of patients with SSc, and to analyze its correlations with pulmonary function parameters, echocardiographic findings, and microvascular abnormalities observed through nailfold videocapillaroscopy. Materials and Methods. We conducted a retrospective analysis of 120 patients with SSc followed at our center. Pulmonary hypertension was defined echocardiographically as a systolic pulmonary artery pressure greater than 30 mmHg. All patients underwent high-resolution computed tomography (HRCT) of the chest, pulmonary function tests, echocardiography, and nailfold videocapillaroscopy. Clinical, serological, and instrumental data were collected and analyzed. Statistical analysis included t tests, chi-square tests, and logistic regression. Results. Twenty-seven percent of patients met criteria for PH. Subjects with PH exhibited a significant reduction in DLCO (mean 47.2 ± 13.3 vs 58.8 ± 11.8; p<0.001), a lower FVC/DLCO ratio, and signs of right ventricular dysfunction (mean TAPSE: 19.5 ± 3.8 mm vs 23.1 ± 3.1 mm; p<0.001). Capillaroscopic findings showed a significantly higher prevalence of the “late” pattern in patients with PH (62% vs 23%; p<0.01), characterized by avascular areas, giant capillaries, and loss of microvascular architecture. sPAP was inversely correlated with DLCO (r = –0.56) and positively correlated with capillaroscopic damage score (r = 0.42). A significant association between anti-centromere antibody positivity and PH was also observed (p=0.03). Conclusions. Our findings confirm a high prevalence of PH among patients with SSc and highlight its association with impaired pulmonary function, right-heart dysfunction, and advanced microvascular damage on videocapillaroscopy. The integration of pulmonary function tests, echocardiography, and nailfold videocapillaroscopy may represent a non-invasive and effective approach for the early identification of patients at risk of developing PH. These results support the adoption of integrated, multidisciplinary screening models in clinical practice to ensure early diagnosis and monitoring of pulmonary hypertension in systemic sclerosis. Future prospective studies will be essential to confirm these data and to evaluate their long-term prognostic impact.

The rarity of myositis-specific antibodies (MSAs) presents challenges in the assessment of idiopathic inflammatory myopathy (IIM) subsets. We leveraged a national database to identify MSA-positive veterans and compare all-cause mortality across antibody subsets in a singular population. A retrospective analysis of veteran adults with MSA testing between 1/1/2011 and 12/31/2021 was performed. A minimum of two outpatient International Classification of Disease 9 or 10 visit codes from specialty clinics at least 30days apart with one creatine phosphokinase level above the upper limit of normal was used to assign a presumptive IIM diagnosis. Current Procedural Terminology codes were used to identify lung computerized tomography imaging studies. Kaplan-Meier survival and Cox proportional regression analyses were performed for the primary outcome of mortality from any cause with a censor date of 12/10/2023. A total of 1749 veterans met inclusion criteria. Jo-1 was the most common MSA (75.0%), followed by PL-7 (8.9%) and HMGCR (3.5%). Our cohort was male-predominant (83.4%) with a robust representation of non-White veterans (47.3%). Jo-1 individuals had longer survival compared to non-Jo-1 (75% survival at 6.5years, 95% CI 5.8-7.1 vs 3.4years, 95% CI 2.7-5.2, log rank p < 0.001). Univariate and multivariable Cox proportional hazard analyses showed that non-Jo-1 status was associated with higher mortality, irrespective of antibody titer. There were no sex differences in mortality or lung disease prevalence. Age, lung involvement, and non-Jo-1 MSA status are associated with increased veteran mortality in this large, male-predominant cohort. Key Points • Jo-1 antibody positivity was associated with the best survival among MSA subsets. • The incidence of lung involvement was equal among Jo-1-positive men and women. • Veteran survival was chiefly determined by MSA subtype, irrespective of antibody titer.

Rationale:Systemic vasculitis is one of the most challenging, complex and difficult diseases in the field of rheumatic diseases and even in the field of internal medicine, which can involve multiple systems of the whole body. Owing to their rich vascular beds, the lungs and kidneys are among the most commonly affected organs in systemic vasculitis. Lung involvement can manifest as interstitial lung disease, diffuse alveolar hemorrhage, pulmonary nodules, pulmonary arterial hypertension, pulmonary aneurysms, pulmonary arteriovenous thrombosis, etc, which are easily confused with infections, tumors, and other diseases. Renal involvement in systemic vasculitis presents with a diverse spectrum of clinical manifestations. Given its high prevalence, complex clinical presentation, significant diagnostic challenges, and generally poor prognosis, achieving a thorough understanding of its various phenotypes, establishing an early diagnosis, and initiating prompt and aggressive treatment are crucial for substantially improving patient outcomes.Patient concerns:A 68-year-old man presented with a cough. His condition deteriorated despite antibiotic treatment for suspected pneumonia, followed by the development of worsening renal function evidenced by progressively elevated serum creatinine and proteinuria. Subsequent serological testing was positive for antineutrophil cytoplasmic antibody antibodies.Diagnoses:Laboratory tests and pulmonary computed tomography scan.Interventions:The patient was started on a regimen of oral cyclophosphamide and high-dose glucocorticoids.Outcomes:The patient’s renal function has deteriorated and requires maintenance hemodialysis.Lessons:This case emphasizes the necessity of maintaining vigilance when distinguishing ANCA-associated vasculitis lung damage from lung infection in patients.

ObjectivesThe primary aim of this study was to describe the prevalence and characteristics of lung ultrasound abnormalities six months after hospital discharge for COVID-19 pneumonia.MethodsThis prospective observational study included patients hospitalised with COVID-19 pneumonia between March 2020 and May 2022. Participants attended a follow-up visit between one and six months after discharge. A standardised 14-zone ultrasound protocol was applied in all cases. Clinical variables and symptoms, including dyspnoea graded using the modified Medical Research Council (mMRC) scale, were recorded.ResultsThe median time from discharge to follow-up assessment was 75 days. Lung involvement on ultrasound was frequent, observed in 79.3%. The most common findings were pleural line irregularities with B-lines (75.9%) and subpleural consolidations (35.6%). A B-line count ≥ 3 per segment was noted in 38.3%. Abnormalities were more common in the basal lung regions. Dyspnoea was the main respiratory symptom, reported by 67% with varying degrees on the mMRC scale.ConclusionsCOVID-19 pneumonia remains a significant public health concern, particularly among high-risk groups susceptible to severe disease and post-COVID sequelae. Efficient screening methods are needed for evaluating potential sequelae. The technical features of lung ultrasound—its accessibility, safety, reproducibility, and ability to detect pulmonary abnormalities during post-COVID follow-up—may render it a valuable tool for patient monitoring. In our series, lung ultrasound findings (pleural line irregularities, B-lines, and subpleural consolidations) were frequent. However, further studies correlating sonographic findings with associated factors and persistent symptoms are required to confirm and validate its role in this setting.

Objective: The prevalence of olfactory dysfunction during COVID-19 (Coronavirus Disease-19) ranges from 4% to 80%, yet the duration of olfactory impairment and the factors influencing it are unclear. This study investigated the progression and influencing factors of olfactory dysfunction over a 24-month follow-up period in patients who developed olfactory impairment during COVID-19. Methods: This cross-sectional study was conducted at Konya Selçuk University Medicine of Faculty Hospital between 01.06.2020 and 31.12.2023. Fifty-one patients with olfactory dysfunction identified during COVID-19 infection were included. Demographics, clinical histories, and laboratory data were recorded for all patients. Olfactory function was evaluated using the Connecticut Chemosensory Clinical Research Center (CCCRC) test, and the test was repeated at the end of two years in 49 accessible patients. Changes in olfactory function during the two-year period were reassessed. The duration of olfactory and gustatory dysfunction, types of gustatory impairment, and recovery times were recorded. Results: A negative correlation was found between patient age and the duration of olfactory dysfunction. Patients with pulmonary involvement had significantly longer durations of olfactory dysfunction than those without pulmonary involvement. Conclusion: Although various factors influence the development of olfactory dysfunction during COVID-19, it has been concluded that olfactory dysfunctions are more common and longer, especially in young patients and patients with lung involvement.

Background: The Omicron variant of SARS-CoV-2 exhibits higher transmissibility compared to previous variants. This retrospective, single-center study aimed to evaluate the relationship between vaccination status and mortality among patients infected with the Omicron variant. Methods: This retrospective analysis included 584 patients diagnosed with the Omicron variant of SARS-CoV-2 between January and March 2022 at Bozyaka Training and Research Hospital, Turkey. Demographic characteristics, comorbidities, laboratory findings, computed tomography (CT) severity scores, vaccination status, and mortality outcomes were assessed. Multivariate logistic regression was used to adjust for potential confounding factors, including age, sex, and comorbidities. Results: Among the 584 patients, 280 were unvaccinated and 304 vaccinated. Mortality was 21% in unvaccinated patients (mean age 56±17) and 10% in vaccinated patients (mean age 62±15). After adjustment for age and Charlson Comorbidity Index, vaccination remained independently associated with reduced mortality (p&amp;lt;0.05). Unvaccinated patients demonstrated higher inflammatory markers, elevated CT severity scores, and greater ICU admission rates (34% vs. 16%). Conclusion: Vaccination significantly reduced mortality and lung involvement among patients infected with the Omicron variant. Despite reports of milder disease, Omicron remains clinically significant, particularly in unvaccinated individuals.

Chronic graft-versus-host disease (cGVHD) is a long-term, heterogeneous immunologic complication of allogeneic hematopoietic cell transplantation. A subset of patients with cGVHD develop progressive fibrosis with highly morbid manifestations, including sclerodermatous skin and lung involvement, classically considered bronchiolitis obliterans syndrome. The fibrosis observed with cGVHD involves a complex interplay between immune dysregulation and aberrant tissue repair that is incompletely understood, although the mechanisms of other fibrotic diseases inform our understanding of the pathways involved and likely hold key insights for future therapeutic targets. Current treatment options for cGVHD are broadly approved for all manifestations. Data regarding the efficacy of these therapies for specific fibrotic complications are limited, although more recent trials have begun to specifically evaluate the efficacy and safety of these treatments for lung and sclerotic cGVHD. Furthermore, current methods of evaluating treatment effectiveness in sclerotic or fibrotic disease are generally subjective, and partial responses can vary substantially. Given these challenges, new techniques and technologies are needed to improve assessments of clinical response. Prospective studies adapted and dedicated to these high-risk organ-specific manifestations of cGVHD should be a research priority.

Peripheral T-cell lymphoma (PTCL) is a rare tumor that originates in mature T cells or NK cells. PTCL is extremely rare in the lungs, either as a primary or secondary lesion, and the clinical course usually progresses rapidly, resulting in a poor prognosis. This article reported a case of a 35-year-old young female patient who was diagnosed with PTCL by percutaneous lung puncture pathology. The patient was admitted to the hospital due to cough and sputum. Chest CT scan revealed multiple patchy, high-density shadows and scattered nodules of varying sizes in both lungs. The patient was admitted to the hospital for suspected tuberculosis. The patient underwent enhanced chest CT scan and a percutaneous lung puncture biopsy. According to the results of histopathology and immunohistochemistry, the patient was finally diagnosed with PTCL. The patient eventually died of septic shock after chemotherapy. This result revealed the complexity of lung lesions. PTCL is difficult to diagnose in the early stage and is prone to misdiagnosis and missed diagnosis.