Fabry disease, also known as Anderson-Fabry disease, is a rare, X-linked lysosomal storage disease caused by mutations in the GLA (galactosidase alpha) gene, which causes a deficiency in the action of the alpha-galactosidase A enzyme. This often leads to an accumulation of globotriaosylceramide and globotriaosyl-sphingosine in various tissues, allowing clinical presentations of Fabry disease to be quite diverse among patients. This report demonstrates a case of a 31-year-old woman with a unique presentation of Fabry disease. The patient presented to the emergency room with two days of cough and hemoptysis. A CT scan revealed a 2.5 cm x 2.6 cm x 2.4 cm lung nodule in the right middle lobe. This nodule was eventually removed and sent for pathological examination, where it was determined to be a likely manifestation of Fabry disease. Genetic testing confirmed the diagnosis of a GLA c.988C>G, p. Q330E variant classified as likely pathogenic. Due to the absence of other signs or symptoms of Fabry disease after further workup, the patient was recommended for biannual follow-up. This case demonstrates a unique presentation of Fabry disease that may be helpful for clinicians when determining the etiology of lung nodules, especially in patients with a family history of the disease. Furthermore, recognition of nodule formation as a potential presentation of Fabry disease is important for effective diagnosis and long-term management of patients with the disease as a whole.

Symptomatic pulmonary aspergilloma and chronic cavitary pulmonary aspergillosis (CCPA) are typically managed with surgical resection or prolonged triazole therapy, but many patients are poor surgical candidates, and many cannot tolerate voriconazole. Our centre has previously described bronchoscopic debridement combined with antifungal therapy as a minimally invasive alternative for patients with accessible intracavitary fungal balls. Isavuconazole, a newer triazole with a more favourable adverse-event and drug-drug interaction profile, has not been well characterised in this setting. We performed a retrospective single-centre review of nine adults with pulmonary aspergilloma or CCPA referred to our interventional pulmonology service who received isavuconazole between September 2022 and September 2025. Of these, six underwent attempted bronchoscopic debridement (four successful, two with limited or unsuccessful access), one required surgical resection after bronchoscopy failed, one was managed medically because the cavity was inaccessible, and one declined further procedure. Three patients had previously received voriconazole; two were switched to isavuconazole because of intolerance and one was re-treated with isavuconazole at recurrence. One patient developed isavuconazole-associated drug-induced liver injury requiring discontinuation. Three patients died during follow-up: two from causes unrelated to aspergillosis (acute coronary event during dialysis; progression of metastatic colorectal cancer) and one from refractory hemoptysis after isavuconazole was stopped for hepatotoxicity. In this small heterogeneous cohort, isavuconazole appeared well tolerated and was a feasible alternative to voriconazole when prolonged adjunctive therapy was required alongside bronchoscopic or medical management of pulmonary aspergilloma and CCPA. Larger comparative studies are needed to define its place in multimodal protocols.

Purpureocillium lilacinum (P. lilacinum) is a rare opportunistic mold that predominantly affects immunocompromised hosts and exhibits intrinsic resistance to several conventional antifungal agents. Pulmonary infection is uncommon, and only a limited number of cases have been reported in patients with autoimmune connective tissue diseases receiving immunosuppressive therapy. We report a 37-year-old male hospital social worker with anti-Jo-1-positive myositis-associated interstitial lung disease receiving corticosteroids and mycophenolate mofetil who presented with hemoptysis and dyspnea. Computed tomography of the thorax demonstrated bilateral ground-glass opacities, multilobar consolidation, tree-in-bud nodularity, and mediastinal lymphadenopathy. Bronchoalveolar lavage fungal culture yielded P. lilacinum, with species identification confirmed by matrix-assisted laser desorption/ionization time-of-flight mass spectrometry (MALDI-TOF MS). Because second-generation triazoles were unavailable at the time of diagnosis, treatment was initiated with oral itraconazole (200 mg twice daily) together with temporary cessation of immunosuppressive therapy. The patient experienced rapid clinical improvement, with resolution of the acute pulmonary infiltrates on follow-up computed tomography after three months and a negative follow-up sputum fungal culture after six months of therapy. Immunosuppressive treatment was subsequently reintroduced without recurrence of infection or progression of interstitial lung disease during follow-up. This case highlights the diagnostic challenge of probable pulmonary P. lilacinum infection in immunosuppressed patients with myositis-associated interstitial lung disease. It demonstrates a favorable clinical course during prolonged oral itraconazole therapy when preferred antifungal agents were unavailable. However, treatment outcomes should be interpreted with caution, as this is a single case, and concomitant antibacterial therapy, along with temporary modification of immunosuppressive therapy, may also have contributed to recovery.

Bronchiectasis and pulmonary tuberculosis (TB) remain common causes of chronic respiratory morbidity worldwide, despite major advances in antimicrobial therapy and structured airway clearance strategies. In a subset of patients, medical treatment alone is insufficient, and surgical intervention may be required when disease is anatomically localised or when serious complications develop. From a surgical perspective, resection of non-functional, chronically infected lung segments in bronchiectasis can lead to sustained symptom improvement, fewer exacerbations, and effective control of haemoptysis when patients are appropriately selected. In pulmonary TB, surgery continues to serve both diagnostic and therapeutic purposes. Operative intervention may be necessary when noninvasive investigations fail to establish a diagnosis, or when persistent cavitary disease, drug-resistant infection, or structural complications such as destroyed lung, chronic empyema, or bronchopleural fistula are present. Current respiratory guidelines emphasise careful medical optimisation and multidisciplinary assessment before surgical referral, highlighting the importance of appropriate patient selection. This narrative educational review focuses on practical principles that guide surgical decision-making in bronchiectasis and pulmonary TB. Indications for surgery, contraindications to operative intervention, and criteria for referral for lung transplantation are discussed, with the aim of supporting clinicians involved in the care of patients with complex structural lung disease.

Bronchial artery embolization (BAE) is an established first-line treatment for life-threatening hemoptysis in adults; however, evidence regarding its safety and efficacy in pediatric patients remains limited, particularly in children with idiopathic pulmonary hemosiderosis-associated pulmonary hemorrhage.

Tuberculosis (TB) caused by acid-fast bacilli is one of the leading causes of morbidity and mortality in developing countries. Pakistan has the world's fifth-largest prevalence of TB, with 276/100,000 cases in prevalence and a mortality of 34/100,000. Delayed diagnosis, disruptions in treatment continuity and a lack of awareness are the key issues leading to multidrug-resistant TB. The current study aimed to find out the determinants of delayed pulmonary TB in a rural district of Khyber Pakhtunkhwa Province, Pakistan. The cross-sectional study was conducted in six TB control program centers designated by the National TB Control Program from March to December 2024. Data were collected from 250 eligible patients, and after removal of incomplete questionnaires, the data of 229 patients were analyzed. The study was approved by the district TB officer, and participants' consent was obtained before data collection. A delay of >21 days was considered the cutoff point for delayed TB diagnosis. Among 229 patients, 51.1% were male and 48.9% female participants, with a mean age of 33.31±21.32 years. The mean diagnostic delay was 23.66±14.94 days. Descriptive analysis of data showed 52% of delayed TB diagnosis cases; 92.2% of the studied patients were from rural areas, in which 80% of delayed TB diagnosis cases were reported. Male gender [odds ratio (OR)=2.31], getting treatment from public hospitals (OR=4.13), age group 31-45 years (OR=3.10), presence of acute extrapulmonary symptoms of cough, fever, and weight loss (OR=2.84), perception that the symptoms will be relieved by oneself (OR=9.99), economic constraints (OR=6.58), and perception of the poor quality of healthcare services at hospitals (OR=7.32) were found to be significant factors of delayed TB diagnosis. Primary school- (OR=0.20), college- or university-level education (OR=0.42) and high-income groups (OR=0.39) were significant protective factors against delayed TB diagnosis. Marital status, occupation, residence, comorbidities, distance from healthcare facility, previous exposure to TB patients, fever, weight loss, chronic cough, hemoptysis, fear of diagnosis, fear of social isolation, and bad experience of the healthcare staff behavior were found to be non-significant factors for delayed TB diagnosis. The study only focused on delayed diagnosis and did not report healthcare system-related or total delay in tuberculosis management. The study affirmed the significant impact of patient perceptions and socioeconomic status on delayed TB diagnosis.

Kartagener syndrome (KS) is a rare autosomal recessive motile ciliopathy and a well-recognized subtype of primary ciliary dyskinesia, classically defined by the triad of situs inversus totalis, chronic sinusitis, and bronchiectasis, with frequent accompanying infertility. Because of its non-specific clinical manifestations overlapping with common respiratory disorders and limited availability of gold-standard diagnostic tools in primary care settings, KS is frequently misdiagnosed or diagnosed late. This study retrospectively analyzed the clinical data, diagnostic trajectories, and treatment outcomes of three adult patients with clinically diagnosed complete KS admitted to our institution between May 2021 and December 2022. All patients presented with long-standing recurrent cough and expectoration; two presented with hemoptysis and one with type II respiratory failure. Initial misdiagnoses included bronchiectasis, non-tuberculous mycobacterial infection, and chronic obstructive pulmonary disease. Clinical diagnosis of complete KS was established based on the classic triad on imaging combined with core clinical features, as confirmatory genetic or ultrastructural testing was not available. Pathogen testing detected Acinetobacter baumannii in one patient, while another patient had a coinfection of Pseudomonas aeruginosa and Enterobacter cloacae. One more patient received empirical antimicrobial therapy because their family declined bronchoscopy. After individualized treatment, clinical improvement was observed in all patients, with symptom resolution, normalized inflammatory markers, and stable disease during 6-12 months of follow-up. These findings underscore that the combined features of "situs inversus totalis + bronchiectasis + chronic sinusitis + childlessness/infertility (when formally confirmed)" are key diagnostic clues for KS in primary care. Primary care clinicians should strengthen awareness of this rare entity and use a multisystem diagnostic approach to prevent misdiagnosis and underdiagnosis. Prompt targeted antimicrobial therapy and sustained comprehensive long-term management were associated with favorable clinical outcomes in this small case series.

Primary pulmonary synovial sarcoma (PPSS) is a rare and aggressive malignancy accounting for 0.5% of lung cancers, with a 5-year survival rate of only 30%-50%. Diagnosis is challenging as it has no specific clinical or radiological features that distinguish it from other lung malignancies.We report a man in his 20s who presented with a 6-week history of cough and haemoptysis. Initial chest CT revealed a 2.2 cm endobronchial lesion in the left mainstem bronchus. Bronchoscopy confirmed the obstructing mass but initial biopsy was non-diagnostic. Positron emission tomography-CT scan demonstrated rapid tumour enlargement to 8.2 cm without evidence of distant hypermetabolic activity. CT-guided biopsy revealed spindle cell neoplasm, and immunohistochemistry along with SS18 fluorescence-in-situ-hybridisation confirmed a low-grade PPSS following a multidisciplinary team evaluation. Despite neoadjuvant chemotherapy, the tumour progressed; hence, he underwent sleeve pneumonectomy. This case highlights the importance of early referral to specialised lung centres and multidisciplinary care to enable timely diagnosis and improve outcomes in patients with rare pulmonary malignancies.

Behçet's disease (BD) is a rare systemic vasculitis. While pulmonary involvement occurs, severe intracardiac manifestations are exceptional. We report a 44-year-old male presenting with dyspnea and hemoptysis. Multimodality imaging revealed bilateral pulmonary thromboembolism, severe three-vessel and left main coronary artery disease, alongside massive thrombi on the ventricular surface of the tricuspid valve (right ventricle) and at the left ventricular apex. With negative blood cultures, he was diagnosed with BD. Refusing recommended surgical revascularization, he was successfully managed with immunosuppressants (glucocorticoids, cyclophosphamide, infliximab), rivaroxaban, and heart failure therapies. This case underscores the critical role of echocardiography and multimodality imaging in diagnosing life-threatening, multi-system cardiovascular complications of BD.

Hydatidosis, or cystic echinococcosis, is an anthropozoonosis caused by the larval form of Echinococcus granulosus. A ruptured pulmonary hydatid cyst occurring during pregnancy is rare and confronts the clinician with a dual maternal and fetal imperative. We report the case of a 31-year-old patient from a rural background, gravida 3, in the third trimester of a term pregnancy, admitted for hemoptysis following lower thoracic pain and a dry cough that had been present for two weeks. On examination, the patient was hemodynamically and respiratorily stable. Chest CT angiography revealed a cavitary lesion of the left lower lobe communicating with the bronchi, consistent with a ruptured hydatid cyst, associated with alveolar hemorrhage and without pulmonary embolism. As the pregnancy was already at term, a primary cesarean section was performed to avoid the expulsive efforts of a vaginal delivery, which were liable to aggravate the hemoptysis through the rise in intrathoracic pressure, and to secure the subsequent thoracic surgery; it resulted in the birth of a healthy newborn. A left lower lobectomy was performed two weeks later, and the maternal postoperative course was uneventful. This case illustrates the effectiveness of a sequential strategy involving a cesarean section followed by delayed surgical management in the treatment of a ruptured pulmonary hydatid cyst in a woman at term, with a favorable prognosis for both mother and child, provided that a multidisciplinary approach is adopted.