Current Issue : July-September Volume : 2026 Issue Number : 3 Articles : 5 Articles
Background: Common variable immunodeficiency (CVID) is the most frequent symptomatic primary antibody deficiency, associated with recurrent infections, immune dysregulation, and non-infectious complications. Amyloidosis is a rare but severe complication with pulmonary involvement being exceptional. Objective: The aim of this study was to review reported cases of amyloidosis complicating CVID and present a unique case of pulmonary involvement. Methods: A literature research identified observational studies and case reports linking amyloidosis with CVID. Additionally, we describe a patient with CVID complicated by pulmonary and gastrointestinal amyloidosis. Results: Fifteen cases were identified, mostly amyloid A (AA) with multiple organ involvement. Only one case of pulmonary amyloidosis was reported. To date, no cases of pulmonary light-chain amyloidosis (AL) have been described in CVID patients without an underlying plasma cell dyscrasia. Our patient initially presented with AA amyloidosis but evolved to systemic AL type with rapid progression and fatal outcome despite therapy. Conclusions: Amyloidosis should be considered in CVID patients with atypical symptoms. Accurate amyloid typing is essential as treatment differs between AA and AL types. Early recognition may improve outcomes....
Mucoepidermoid carcinoma of the lung is a rare salivary gland-type tumor with heterogeneous clinical behavior and the potential to mimic neoplasms arising in other organs. The purpose of this report is to describe an exceptionally uncommon presentation of pulmonary mucoepidermoid carcinoma manifesting as a breast metastasis in a male patient, a scenario that poses significant diagnostic challenges due to its rarity and its morphological resemblance to primary breast carcinoma. We evaluated the patient through clinical examination, cross-sectional imaging, endobronchial procedures, ultrasound-guided biopsy, immunohistochemistry, and molecular analysis, integrating these data to establish the diagnosis. Imaging revealed a primary lung mass and a second lesion in the left breast infiltrating the pectoralis muscle. Biopsy of the breast mass showed high-grade salivary gland-type mucoepidermoid carcinoma, clinically and radiologically suggestive of pulmonary origin. Because the lesion showed signs of impending ulceration, palliative surgical debulking was performed with good postoperative recovery. The patient subsequently began systemic therapy with gemcitabine. This case underscores the need for careful clinicopathologic correlation when evaluating atypical breast lesions and highlights the diagnostic value of molecular testing in distinguishing primary from metastatic salivary gland-type tumors. Recognizing such rare metastatic patterns is essential for appropriate therapeutic planning....
Introduction: Bronchiectasis is a chronic, heterogeneous airway disease characterised by irreversible bronchial dilatation, recurrent infections, and persistent inflammation, leading to progressive lung damage, frequent exacerbations, and impaired quality of life. Neutrophildriven inflammation, largely mediated by excessive activity of neutrophil serine proteases such as neutrophil elastase, represents a central pathogenic mechanism and an important therapeutic target. Methods: Brensocatib, a first-in-class, selective, and reversible inhibitor of dipeptidyl peptidase-1 (DPP-1), prevents the activation of neutrophil serine proteases during neutrophil maturation in the bone marrow. By reducing downstream protease activity, brensocatib modulates aberrant neutrophilic inflammation without broadly suppressing immune function. Results: Clinical studies, including the Phase-2 WILLOW trial and the Phase-3 ASPEN trial, have demonstrated that brensocatib significantly reduces exacerbation frequency, prolongs time to first exacerbation, and lowers sputum neutrophil protease activity, with a favourable safety profile. Importantly, these benefits were observed across multiple patient subgroups and in addition to standard-of-care therapies. Conclusions: As the first FDA-approved (12 August 2025) mechanism-based therapy for non–cystic fibrosis bronchiectasis, brensocatib represents a paradigm shift toward targeted, precision treatment of neutrophil-mediated airway disease. Its clinical efficacy, biomarkerdriven rationale, and potential to reduce antibiotic dependence highlight brensocatib as a cornerstone therapy in bronchiectasis management and a promising strategy for other neutrophil-driven inflammatory conditions....
Background and Objectives: Idiopathic pulmonary fibrosis (IPF) is frequently accompanied by chronic cough, which may negatively affect sleep quality. However, the relationship between cough burden and sleep disturbances in patients undergoing antifibrotic therapy remains inadequately defined. This study aimed to investigate the association between cough and sleep quality in patients with IPF and to evaluate the potential effects of antifibrotic treatment on these outcomes. Materials and Methods: This crosssectional analytical study was conducted at a tertiary care center in Türkiye between January 2019 and December 2024. Patients with a diagnosis of IPF who were receiving antifibrotic therapy (nintedanib or pirfenidone) were consecutively recruited from the pulmonology outpatient clinic. Sleep quality was assessed using the Pittsburgh Sleep Quality Index (PSQI), and cough-related quality of life was evaluated with the Leicester Cough Questionnaire (LCQ). Pre-treatment data were obtained retrospectively based on patient recall, and follow-up assessments were conducted during ongoing therapy. Correlation analyses and multivariable linear regression models were used to identify factors associated with sleep quality. Results: The study included 74 patients, with a mean age of 68.6 ± 6.8 years. At baseline, 87.8% of patients had poor sleep quality (PSQI ≥ 5). During antifibrotic therapy, PSQI scores significantly improved (median 9 [IQR: 6–12] vs. 6 [IQR: 5–8], p < 0.001), accompanied by a clinically meaningful increase in LCQ total score (13.28 ± 2.86 vs. 16.06 ± 2.58, p < 0.001). Significant inverse correlations were observed between PSQI and LCQ scores at both baseline and follow-up. In multivariable analysis, LCQ score was an independent predictor of sleep quality during treatment (β = −0.453, p < 0.001), whereas demographic and physiological parameters showed no significant independent associations. No significant differences were detected between nintedanib and pirfenidone in terms of PSQI or LCQ outcomes. Conclusions: Cough-related quality of life is independently associated with sleep quality in patients with IPF receiving antifibrotic therapy. These findings highlight cough burden as a key determinant of patient-centered outcomes beyond traditional physiological measures. Targeted assessment and management of cough may represent an important strategy to improve sleep quality and overall quality of life in this population....
Chronic pulmonary regurgitation (PR) after the repair of tetralogy of Fallot (TOF) and other right ventricular outflow tract (RVOT) interventions leads to progressive right ventricular (RV) dilatation, altered ventricular–ventricular interaction, and an increased risk of arrhythmia and heart failure. Pulmonary valve replacement (PVR), whether surgical or transcatheter, effectively eliminates or reduces PR and is associated with short- and mid-term improvement in RV size, symptoms, and electrocardiographic markers. However, the optimal timing of intervention remains unresolved: operating late can result in irreversible myocardial damage and arrhythmogenic substrates, whereas operating early can lead to repeated reinterventions, the impact of which on hard outcomes is uncertain. This review summarizes contemporary evidence on ventricular remodelling after PVR, focusing on cardiovascular magnetic resonance (CMR) and echocardiographic markers, and critically appraises proposed criteria for timing PVR. Classic CMR-derived thresholds (RV end-diastolic volume index [RVEDVi] 150–170 mL/m2, RV end-systolic volume index [RVESVi] 80–90 mL/m2) and QRS duration cut-offs are discussed alongside emerging markers of risk, including the RV mass-to-volume ratio, diffuse myocardial fibrosis (extracellular volume fraction), strain imaging, and diastolic dysfunction. Meta-analyses show consistent reverse remodelling and symptomatic benefit after PVR, but no conclusive survival benefit has been demonstrated, and data on arrhythmic outcomes remain conflicting. Key gaps include (i) the lack of prospective randomized or carefully matched comparative studies of “early” versus “deferred” PVR; (ii) limited understanding of how myocardial fibrosis, RV hypertrophy, and diastolic dysfunction interact with volume load and timing to influence long-term outcomes; (iii) under-representation of adult and older adult TOF cohorts; and (iv) insufficient integration of multiparametric risk scores and machine-learning approaches into clinical decision-making. Future research should prioritize multicentre longitudinal cohorts with standardized imaging, electrophysiological and clinical endpoints, incorporate advanced imaging techniques (e.g., strain, 3D late gadolinium enhancement, and T1 mapping), and explore precision-medicine strategies to individualize PVR timing....
Loading....