Use of ruxolitinib in COPA syndrome manifesting as life-threatening alveolar haemorrhage.

COPA (coatomer subunit α) syndrome is a newly recognised cause of interstitial lung disease in children and adults, frequently associated with arthritis and renal dysfunction. We report a 11-year-old girl with disease limited to major pulmonary haemosiderosis manifesting at the age of 2 years, due to a heterozygous p.(Arg233His) mutation in COPA Her interferon (IFN) signature was elevated (10.312 and 12.429, healthy <2.466), as was the level of serum IFNα (211 fg/mL, healthy <10 fg/mL). STAT1 phosphorylation in T lymphocytes and monocytes was increased as compared with healthy controls. Based on these results she was treated with the JAK1/2 inhibitor ruxolitinib, which resulted in reduction in IFN signalling and appeared to be associated with partial though incomplete decrease in the severity of her pulmonary disease. Patients with alveolar haemorrhage of unknown origin should be considered for COPA screening. Functional tests can help to personalise patient therapy.

Large bronchial artery-pulmonary artery fistula due to cavitating tuberculosis.

Bronchial artery-pulmonary artery fistulae are rare vascular malformations most commonly caused by infection. Our case presents a 57-year-old male who presented to the Emergency Department with a symptomatic bronchial artery-pulmonary artery fistula due to cavitating pulmonary tuberculosis (TB). The diagnosis was made with multiphase CT angiography of the thorax (including pulmonary and systemic arterial phases). The patient was brought to interventional radiology for further investigation and management. The left upper lobe bronchial artery-pulmonary artery fistula was successfully identified and treated with endovascular embolization. Bronchial artery-pulmonary artery fistulae can pose a diagnostic and therapeutic challenge. Our case demonstrates endovascular embolization as an effective method of treating symptomatic bronchial artery-pulmonary artery fistulae.

Bronchial occlusion with endobronchial Watanabe spigots using a two-scope technique for massive haemoptysis.

Massive haemoptysis is a life-threatening condition whose cause needs to be identified rapidly so that prompt interventions can ensue. Bronchial occlusion with endobronchial Watanabe spigots (EWSs) may be useful when endovascular treatment or surgery proves to be difficult. An 84-year-old woman developed massive haemoptysis during percutaneous mitral valve repair for refractory heart failure due to severe mitral regurgitation (MR). Interventional radiology (IVR) and surgery were contraindicated, and bronchial occlusion with EWSs was attempted to control bleeding. The bleeding was so persistent that it was difficult to secure the visual field without aspiration with a bronchoscope. Herein, we report a two-scope technique, also used in cryobiopsy of peripheral lung lesions, to control bleeding and perform bronchial occlusion with EWSs.

CIRSE Standards of Practice on Bronchial Artery Embolisation.

This CIRSE Standards of Practice document is aimed at interventional radiologists and provides best practices for performing bronchial artery embolisation to effectively treat haemoptysis. It has been developed by an expert writing group established by the CIRSE Standards of Practice Committee.

Coil embolisation for massive haemoptysis in cystic fibrosis.

INTRODUCTION: Massive haemoptysis is a life-threatening event in advanced cystic fibrosis (CF) lung disease with bronchial artery embolisation (BAE) as standard of care treatment. The aim of our study was to scrutinise short-term and long-term outcomes of patients with CF and haemoptysis after BAE using coils. METHODS: We carried out a retrospective cohort study of 34 adult patients treated for massive haemoptysis with super selective bronchial artery coil embolisation (ssBACE) between January 2008 and February 2015. Embolisation protocol was restricted to the culprit vessel(s) and three lobes maximum. Demographic data, functional end-expiratory volume in 1 s in % predicted (FEV1% pred.) and body mass index before and after ssBACE, sputum colonisation, procedural data, time to transplant and time to death were documented. RESULTS: Patients treated with ssBACE showed significant improvement of FEV1% pred. after embolisation (p=0.004) with 72.8% alive 5 years post-ssBACE. Mean age of the patients was 29.9 years (±7.7). Mean FEV1% pred. was 45.7% (±20.1). Median survival to follow-up was 75 months (0-125). Severe complication rate was 0%, recanalisation rate 8.8% and 5-year-reintervention rate 58.8%. Chronic infection with Pseudomonas aeruginosa was found in 79.4%, Staphylococcus areus in 50% and Aspergillus fumigatus in 47.1%. DISCUSSION: ssBACE is a safe and effective treatment for massive haemoptysis in patients with CF with good results for controlling haemostasis and excellent short-term and long-term survival, especially in severely affected patients with FEV<40% pred. We think the data of our study support the use of coils and a protocol of careful and prudent embolisation.

Randomised trial of first-line bronchial artery embolisation for non-severe haemoptysis of mild abundance.

BACKGROUND: Whereas first-line bronchial artery embolisation (BAE) is considered standard of care for the management of severe haemoptysis, it is unknown whether this approach is warranted for non-severe haemoptysis. RESEARCH QUESTION: To assess the efficacy on bleeding control and the safety of first-line BAE in non-severe haemoptysis of mild abundance. STUDY DESIGN AND METHODS: This multicentre, randomised controlled open-label trial enrolled adult patients without major comorbid condition and having mild haemoptysis (onset <72 hours, 100-200 mL estimated bleeding amount), related to a systemic arterial mechanism. Patients were randomly assigned (1:1) to BAE associated with medical therapy or to medical therapy alone. RESULTS: Bleeding recurrence at day 30 after randomisation (primary outcome) occurred in 4 (11.8%) of 34 patients in the BAE strategy and 17 (44.7%) of 38 patients in the medical strategy (difference -33%; 95% CI -13.8% to -52.1%, p=0.002). The 90-day bleeding recurrence-free survival rates were 91.2% (95% CI 75.1% to 97.1%) and 60.2% (95% CI 42.9% to 73.8%), respectively (HR=0.19, 95% CI 0.05 to 0.67, p=0.01). No death occurred during follow-up and no bleeding recurrence needed surgery.Four adverse events (one major with systemic emboli) occurred during hospitalisation, all in the BAE strategy (11.8% vs 0%; difference 11.8%, 95% CI 0.9 to 22.6, p=0.045); all eventually resolved. CONCLUSION: In non-severe haemoptysis of mild abundance, BAE associated with medical therapy had a superior efficacy for preventing bleeding recurrences at 30 and 90 days, as compared with medical therapy alone. However, it was associated with a higher rate of adverse events. TRIAL REGISTRATION NUMBER: NCT01278199.

Leptospirosis with pulmonary haemorrhage and multiple organ failure: a case report and literature review.

Pulmonary haemorrhage is an important complication of leptospirosis. We herein report an uncommon case of severe pulmonary haemorrhage and multiple organ failure caused by leptospirosis in a 49-year-old man who was previously healthy. He was a farm worker who was admitted to the hospital because of haemoptysis. He had worked in a paddy field 4 days prior to admission. Chest computed tomography revealed pulmonary haemorrhage, which rapidly deteriorated into haemorrhagic shock and multiple organ failure. Based on the patient's possible history of contact with contaminated water and the DNA sequence of Leptospira detected in his bronchoalveolar lavage fluid, the patient was diagnosed with pulmonary haemorrhagic leptospirosis. Despite the administration of a fluid bolus, norepinephrine, broad-spectrum antibiotics, and haemostatics, and even with administration of a blood transfusion and extracorporeal life support, the pulmonary haemorrhage could not be controlled effectively. The patient eventually died of haemorrhagic shock. Leptospirosis can be a life-threatening disease despite aggressive treatment, even with extracorporeal life support. Next-generation sequencing can provide important diagnostic clues for patients with atypical leptospirotic symptoms.

Thoracic endovascular aortic repair for aortobronchial fistula: a case series.

BACKGROUND: Aortobronchial fistula (ABF) formation following the rupture of thoracic pseudoaneurysm is a rare clinical entity. Its aetiology includes atherosclerosis, infections, trauma, post-surgery, and post-endovascular aortic repair. The clinical presentation of ABF includes intermittent or massive haemoptysis, acute respiratory distress, hypotension, and even death. These patients require an emergency aortic intervention to stop active haemorrhage. Thoracic endovascular aortic repair (TEVAR) is a less invasive, safe, and effective treatment compared to conventional open surgical repair. CASE SUMMARY: We hereby report three cases of ruptured descending thoracic aortic pseudoaneurysms resulting in a fistula formation. The first two cases had tuberculosis as their underlying aetiology, while the third case was the result of previous open post-aortic surgery. All patients presented with massive haemoptysis and were successfully treated by emergency TEVAR and had favourable outcomes. DISCUSSION: Thoracic endovascular aortic repair is a rapid, less invasive, and effective treatment for emergency management of ABF. It has more than 85% technical success rates in the reported literature. We had procedural success in all three cases. The short and midterm outcome of ABF following TEVAR is favourable and encouraging.