Pulmonary manifestations and outcomes in paediatric ANCA-associated vasculitis: a single-centre experience.

OBJECTIVES: ANCA-associated vasculitis (AAV) usually involves the renal and respiratory systems, but the paediatric literature on pulmonary manifestations and outcomes is limited. We aimed to describe pulmonary manifestations and outcomes after therapy in a cohort of paediatric AAV (pAAV) patients. METHODS: A retrospective chart review of all patients <19 years presenting to our institution with AAV between 1/2008 and 2/2018 was conducted. Patient demographics, clinical presentation, diagnostic testing, therapy and pulmonary outcomes over the first 3 years after presentation were evaluated. RESULTS: A total of 38 patients were included; all had ANCA positivity by immunofluorescence. A total of 23 had microscopic polyangiitis (MPA), 13 had granulomatosis with polyangiitis and 2 had eosinophilic granulomatosis with polyangiitis. A total of 30 (79%) had pulmonary manifestations, with cough (73%) and pulmonary haemorrhage (67%) being the most common. Abnormalities were noted in 82% of chest CT scans reviewed, with nodules and ground-glass opacities being the most common. At 6, 12 and 36 months follow-up, respectively, 61.8%, 39.4% and 29% of patients continued to show pulmonary manifestations. Five MPA patients with re-haemorrhage are described in detail. CONCLUSION: MPA was more common than granulomatosis with polyangiitis, with pulmonary involvement being common in both. MPA patients had more severe pulmonary manifestations. Chest CT revealed abnormal findings in a majority of cases. A subgroup of young MPA patients experienced repeat pulmonary haemorrhage. Treatment modality and response were comparable in different subtypes of AAV, except for this young MPA group. Additional prospective studies are needed to better understand the different phenotypes of pAAV.

Associated factors of co-existent pulmonary tuberculosis and lung cancer: A case-control study.

BACKGROUND: Although the incidence of co-existent pulmonary tuberculosis (PTB) and lung cancer in China is increasing, risk factors related to its development are still poorly understood. We aimed to investigate which clinical factors were associated with the odds of co-existent PTB and lung cancer (PTB-lung cancer) cases in a case-control study. METHOD: A total of 125 PTB-lung cancer patients were enrolled by Beijing Chest Hospital as the case group between January 2012 and December 2016. Age- and sex-matched PTB-only (N = 125) and lung cancer-only (N = 125) patients were selected as the control groups. Data were collected from the medical records and computed tomography (CT) reports. The case group was further categorized into three sub-groups according to the diagnosis intervals between previous PTB and lung cancer (<1 year, 1-10 years, and > 10 years). RESULT: Compared with both controls of PTB-only and lung cancer-only patients, the PTB-lung cancer case group had significantly higher proportions of patients with irritant cough, expectoration, hemoptysis, fever and CT features of irregular mass and pleural thickening. For PTB patients, fibrous calcification (OR, 2.193; 95%CI, 1.168-4.117) was associated with higher odds of lung cancer (P-value < .05). CONCLUSION: Distinct clinical symptoms and CT tests may help with the early diagnosis of PTB-lung cancer cases. PTB patients with fibrous calcification may have a higher risk of lung cancer. Further multicenter prospective studies are required to validate our findings.

Right-sided infective endocarditis in association with a left-to-right shunt complicated by haemoptysis and acute renal failure: a case report.

BACKGROUND: Infective endocarditis has a relevant clinical impact due to its high morbidity and mortality rates. Right-sided endocarditis has lower complication rates than left-sided endocarditis. Common complications are multiple septic pulmonary embolisms, haemoptysis, and acute renal failure. Risk factors associated with right-sided infective endocarditis are commonly related to intravenous drug abuse, central venous catheters, or infections due to implantable cardiac devices. However, patients with congenital ventricular septal defects might be at high risk of endocarditis and haemodynamic complications. CASE PRESENTATION: In the following, we present the case of a 23-year-old man without a previous intravenous drug history with tricuspid valve Staphylococcus aureus endocarditis complicated by acute renal failure and haemoptysis caused by multiple pulmonary emboli. In most cases, right-sided endocarditis is associated with several common risk factors, such as intravenous drug abuse, a central venous catheter, or infections due to implantable cardiac devices. In this case, we found a small perimembranous ventricular septal defect corresponding to a type 2 Gerbode defect. This finding raised the suspicion of a congenital ventricular septal defect complicated by a postendocarditis aneurysmal transformation. CONCLUSIONS: Management of the complications of right-sided infective endocarditis requires a multidisciplinary approach. Echocardiographic approaches should include screening for ventricular septal defects in patients without common risk factors for tricuspid valve endocarditis. Patients with undiagnosed congenital ventricular septal defects are at high risk of infective endocarditis. Therefore, endocarditis prophylaxis after dental procedures and/or soft-tissue infections is highly recommended. An acquired ventricular septal defect is a very rare complication of infective endocarditis. Surgical management of small ventricular septal defects without haemodynamic significance is still controversial.

BET 1: Does inhaled tranexamic acid reduce morbidity in adults with haemoptysis?

A short cut review was carried out to establish whether inhaled tranexamic acid is more effective than placebo at controlling bleeding in patients with haemoptysis. Thirty-four papers were found using the reported searches, of which one presented the best available evidence to answer the clinical question. The author, date and country of publication, patient group studied, study type, relevant outcomes, results and study weaknesses of this paper is tabulated. It is concluded that in patients with non-massive haemoptysis, management with nebulised TXA leads to fast resolution.

Mechanisms of recurrent haemoptysis after super-selective bronchial artery coil embolisation: a single-centre retrospective observational study.

OBJECTIVES: In recognition of the significant impairment caused by haemoptysis on a patient's quality of life, bronchial artery embolisation has been introduced worldwide as one of the first-line treatment options. Since little evidence is available on the mechanisms of recurrent haemoptysis after super-selective bronchial artery coil embolisation (ssBACE), the purpose of the present study is to evaluate these. METHODS: We retrospectively evaluated the mechanisms of recurrent haemoptysis using both enhanced computed tomography and cineangiography following ssBACE by reviewing 299 haemoptysis-related arteries (HRAs) in 57 consecutive patients who underwent 2nd series ssBACE for the management of recurrent haemoptysis between April 2010 and December 2015. RESULTS: Median age of patients was 69 (interquartile range 64-74) years, and 43.9% were men. This study revealed that (1) recanalisation was the most common mechanism (45.2%) followed by development of new HRA (38.5%), bridging collaterals (14.7%) and conventional collaterals (1.7%); (2) these trends could be modified in several situations such as with antiplatelet or anticoagulant medications; (3) relatively large-diameter HRAs were more likely to recanalise compared with small-diameter HRAs and (4) recurrent haemoptysis could be managed by 2nd series ssBACE with a procedural success rate of 97.7% without any major complications. CONCLUSIONS: Recanalisation was the most common mechanism of recurrent haemoptysis after ssBACE. Our results provide interventionists with indispensable insights. KEY POINTS: • Recanalisation was the most common mechanism of recurrent haemoptysis after super-selective bronchial artery coil embolisation, followed by development of new haemoptysis-related arteries • These trends could be modified in several situations such as with antiplatelet or anticoagulant medications • Recurrent haemoptysis could be managed by 2nd series super-selective bronchial artery coil embolisation with a procedural success rate of 97.7% without any major complications.

Spinal Arterial Blood Supply Does Not Arise from the Bronchial Arteries: A Detailed Analysis of Angiographic Studies Performed for Hemoptysis.

PURPOSE: To determine the angiographic prevalence of spinal arteries originating directly from the bronchial arteries in the setting of embolization for hemoptysis. MATERIALS AND METHODS: Over a 14-year interval, 205 patients underwent angiography for hemoptysis. Twenty-five patients were excluded because their bronchial arteries were not visualized. The remaining 180 patients underwent a total of 254 angiographic procedures (range, 1-8 per patient). Images were reviewed jointly by 2 interventional radiologists with formal fellowship training in both peripheral and neurological interventional radiology. All catheterized arteries were evaluated for arterial contribution to the spinal cord. For patients with multiple studies, each unique artery was reported only once. Embolization was performed during at least 1 procedure in 158 patients (88%). Electronic record review was used to assess neurological sequelae after the procedure. RESULTS: One or 2 bronchial arteries originating from the aorta were identified in 57 patients (32%) on the right and in 75 patients (42%) on the left. Conjoined bronchial arteries were found in 76 patients (42%). Spinal arterial supply was absent in all. A total of 102 patients (57%) had at least 1 right and 11 patients (6%) at least 1 left intercostobronchial artery. Spinal arterial supply from the intercostal portion of an intercostobronchial artery was found in 6 patients (5 right, 1 left). Medical record review revealed no postprocedure symptoms referable to spinal cord injury in any patient. CONCLUSIONS: Spinal arterial supply does not originate directly from the bronchial artery but can originate from the intercostal portion of an intercostobronchial artery.

Bilateral pulmonary vein stenting for treatment of massive hemoptysis caused by pulmonary vein stenosis following catheter ablation for atrial fibrillation.

BACKGROUND: Massive hemoptysis is a life-threatening condition. Massive hemoptysis caused by pulmonary vein stenosis (PVS) after radiofrequency catheter ablation for atrial fibrillation (AF) is rare. However, bilateral lung hemorrhage following bilateral PVS is extremely rare. CASE PRESENTATION: We herein describe a 62-year-old man with refractory massive hemoptysis after radiofrequency catheter ablation for AF, which was successfully controlled by surgical lobectomy and endovascular bilateral PV stenting. The hemorrhage was derived from the bilateral lungs following PV obstruction and bilateral PVS, which was definitively diagnosed by bronchoscopic examination. The patient had no recurrence of hemoptysis during a follow-up period of 30 months, and the PV stents had not narrowed as shown by computed tomography 30 months after stent placement. CONCLUSIONS: Massive hemoptysis can be caused by bilateral PVS after radiofrequency catheter ablation for AF, and hemorrhage from the bilateral lungs in such patients is extremely rare. Nevertheless, cardiologists, interventional radiologists, and pulmonologists should consider the potential for massive hemoptysis caused by PVS.

Pulmonary Hemorrhage in the Neonate.

A case study of a 37 weeks' gestational age male infant, presenting with bloody fluid orally shortly after birth. The presentation, etiology, diagnosis, and management of pulmonary hemorrhage are addressed.

Effectiveness of Nitroglycerin in Managing Subacute Lung Bleeding Induced by Balloon Pulmonary Angioplasty.

Lung bleeding (LB) and hemoptysis is a common but life-threating complication of balloon pulmonary angioplasty (BPA) for chronic thromboembolic pulmonary hypertension. LBs related to BPA mostly occur acutely during BPA session. Therefore, it can usually be managed with occlusion balloon or other catheter-based approaches. While LB also develops subacutely after BPA session, the pharmacological option to subacute LB is currently limited. Here, we present a case of subacute LB which can be managed with intravenous administration of nitroglycerin. Nitrate mediated venous dilation can be an effective therapeutic option in managing LB and hemoptysis after BPA session.

Symptomatology, Clinical Presentation and Basic Work up in Patients with Suspected Pulmonary Embolism.

Basic knowledge of pulmonary embolism is relevant to most practicing physicians. Many medical specialties care for patients with increased risk of pulmonary embolism, why recognition of relevant symptoms, a thorough medical history, assessment of the clinical condition of the patient and possibly referral to a relevant facility should be a part of the skills of all clinicians. Sudden onset dyspnea, chest pain, syncope and hemoptysis are essential symptoms of pulmonary embolism, and in most of these patients basic investigations like arterial blood gas analysis, electrocardiogram, chest x-ray and biochemical analyses are appropriate. In addition, lung ultrasound and echocardiography are indicated in many of these patients. The information available from the medical history, clinical assessment and basic investigation form the basis on which the decision about further diagnostic imaging and intensity of treatment and monitoring can be made. These decisions can be guided by clinical scoring systems like the Wells score, revised Geneva score and the PESI.

Invasive Aspergillus Pseudomembranous and Obstructive Tracheo-bronchitis in an Immuno-competent Patient.

A 19 year female, presented with life threatening haemoptysis and cough with minimum expectoration for 3 months. Bronchoscopy showed multiple nodules in airway. The direct microscopy and culture of sputum revealed fungal elements and Aspergillus flavus respectively. Serum Galactomannan was positive. Thus diagnosis of invasive aspergillus tracheo-bronchitis made. She responded to voriconazole. Aspergillus tracheo-bronchitis is a rare form of invasive pulmonary aspergillosis in immuno-competent host. Aspergillus spp in respiratory samples should not be routinely discarded as colonization.

[Hemoptysis in adults with bronchiectasis: correlation with disease severity and exacerbation risk].

Objective: To investigate the association between hemoptysis and disease severity and risks of acute exacerbations in patients with bronchiectasis. Methods: Between September 2012 and January 2014, we recruited 148 patients (56 males, 92 females, mean age: 44.6 years) with clinically stable bronchiectasis, who were classified into hemoptysis group (36 males, 70 females, mean age: 45.6 years) and non-hemoptysis group (20 males, 22 females, mean age: 41.8 years). We inquired the past history, and evaluated chest imaging characteristics, lung function, cough sensitivity assessed using capsaicin cough challenge tests, and airway inflammation. We also performed a 1-year follow-up to evaluate whether patients with hemoptysis would have greater risk of having acute exacerbations. Results: In the hemoptysis group, median 24-hour sputum volume was 20.0 ml, median Bronchiectasis Severity Index (BSI) was 7.0, median bronchiectatic lobes was 4.0, median chest CT score was 7.0, the geometric mean for eliciting 5 coughs following capsaicin cough sensitivity (C5) was 77 µmol/L, 67 cases (63%) had cystic bronchiectasis and 52 cases (49%) had pulmonary cavity shown on chest CT, and 35 cases (33%) had Pseudomonas aeruginosa colonization. In the non-hemoptysis group, median 24-hour sputum volume was 5.0 ml, median BSI was 4.0, median bronchiectatic lobes was 3.0, median chest CT score was 5.0, 15 cases (36%) had cystic bronchiectasis and 10 cases (24%) had pulmonary cavity, the geometric mean for C5 was 212 µmol/L, and 4 cases (10%) had Pseudomonas aeruginosa colonization. All the above parameters differed significantly between the hemoptysis and the non-hemoptysis group (P<0.05). In the hemoptysis group, 29 patients with pulmonary cavity (27%) had reported the use of intravenous antibiotics, and 44 cases (42%) had at least one hospitalization within the previous 2 years. In the non-hemoptysis group, 8 cases (19.0%) had reported the use of intravenous antibiotics, and 8 cases (19.0%) reported hospitalization within 2 years. A prior history of hemoptysis was associated with a greater risk of experiencing bronchiectasis exacerbations during follow-up, after adjusting for age, sex, smoking status and BSI (62 cases in the hemoptysis group, 18 cases in the non-hemoptysis group, χ(2)=16.06, P=0.03). In a multivariate model, cystic bronchiectasis was the sole risk factor for hemoptysis; 67 cases which accounted for 63% of patients in the hemoptysis group and 15 cases which accounted for 36% of patients in the non-hemoptysis group, odds ratio: 2.84, 95% confidence interval: 1.00-8.14, P=0.05 . Conclusions: In this study, 72% of bronchiectasis patients had experienced hemoptysis, which was associated with the severity of bronchiectasis. Patients with a prior history of hemoptysis had a greater risk of acute exacerbations during follow-up than those without.

[The features of diagnosis and treatment of traumatic and non-traumatic pulmonary bleeding]. / Osobennosti diagnostiki i lecheniia legochnogo krovotecheniia travmaticheskoi i netravmaticheskoi étiologii.

AIM: To analyze an efficacy of differentiated approach for pulmonary bleeding of different etiology and severity. MATERIAL AND METHODS: The study included 134 cases of pulmonary bleeding for the period 2006-2015 including 53 patients with traumatic and 81 with non-traumatic etiology. Men/women ratio was 2.7:1, mean age was 43 years. Comparative retrospective analysis of X-ray and CT data in diagnosis of bleeding source was performed. Bronchoscopy was used to confirm these data. There were 43 endovascular examinations including 40 cases of bronchial arteriography (BAG). RESULTS AND DISCUSSION: X-ray and CT-signs of bleeding source were detected more often in case of traumatic pulmonary bleeding (62.3% and 93%) compared with non-traumatic (27.2 and 54%; p<0.05). Bronchoscopy revealed ongoing pulmonary bleeding in 40 (30%) patients, completed - in 94 (70%) patients. Indirect angiographic signs were the most frequent for pulmonary bleeding origin: hypervascularization (32.6%), bronchial-pulmonary shunts (23.2%) and bronchial artery dilatation (20.9%). 17 patients with ongoing bleeding underwent bronchial obstruction with adequate hemostasis in all cases. Endovascular interventions included bronchial arteries embolization (EBA) (33), occlusion of intercostal arteries (3) and segmental branches of low-lobar pulmonary artery (1), aortic stenting (1). Early efficacy of EBA was 97% with 2 month recurrence of pulmonary bleeding near 12.5%. Ongoing traumatic pulmonary bleeding was an indication for emergency thoracotomy in 18.9% compared with 12.3% for non-traumatic bleeding when surgery was made after endoscopic and endovascular hemostasis. CONCLUSION: Differentiated approach depending on etiology and severity of pulmonary bleeding improves outcomes and reduces the number of operations for ongoing severe bleeding.

Clinical Relevance of Computed Tomography Pulmonary Venography.

BACKGROUND: In clinical routine, the pulmonary contrast-enhanced chest computer tomography (CT) is usually focussed on the pulmonary arteries. The purpose of this pictorial essay is to raise the clinicians' awareness for the clinical relevance of CT pulmonary venography. CASE PRESENTATION: A pictorial case series illustrates the clinical consequences of different pulmonary venous pathologies on systemic, pulmonary and bronchial circulation. CONCLUSION: Computed tomography pulmonary venography must be considered before atrial septal defect (ASD) closure and pulmonary lobectomy. Computed tomography pulmonary venography should be considered for patients with right ventricular overload and pulmonary hypertension, as well as for patients with unclear recurrent pulmonary infections, progressive dyspnoea, pleural effusions, haemoptysis, and for patients with respiratory distress after lung-transplantation.

[GOODPASTURE'S SYNDROME--CASE REPORTS]. / GOODPASTUREOV SINDROM--PRIKAZI BOLESNIKÂ.

Goodpasture's syndrome is a rare clinical entity characterized by rapidly progressive glomerulonephritis, diffuse pulmonary hemorrhage and the presence of circulating autoantibodies to the glomerular basement membrane (GBM). Autoantibodies bind to reactive epitopes of noncollagenous domain of the collagen type IV alpha-3 chain in glomerular and alveolar basement membranes. Autoantibodies activate the complement cascade resulting in tissue injury by the type II hypersensitivity reaction according to the Coombs and Gell classification of antigen-antibody reactions. Prognostic factors include the renal excretory function and the degree of renal and lung damage at the time of presentation. Prompt diagnosis and early and adequate medical treatment is vital for patients. Clinical treatment must be aggressive in order of achieving better outcome. This article describes three patients who clinically presented with renopulmonary syndrome, renal failure, hematuria, proteinuria and hemoptysis. Kidney biopsy diagnosis was crescentic glomerulonephritis due to antibodies against GBM. In all three patients we started therapy with glucocorticoids and cyclophosphamide combined with plasma exchange therapy. In two patients who initially had severe impairment of renal function and high percentage of crescents in the renal biopsy, kidney function recovery was not achieved. In one patient, who at the time of clinical presentation showed milder renal failure and lower percentage of crescents in renal biopsy, the full recovery of renal function was obtained.

Complications of tuberculosis.

PURPOSE OF REVIEW: Diagnosis and management of tuberculosis (TB) remains challenging and complex because of the heterogeneity of disease presentations. Despite effective treatment, TB disease can lead to significant short-and long-term health consequences. We review potential acute and chronic complications of TB disease and current management approaches. RECENT FINDINGS: Acute and subacute complications of TB disease are attributable to structural damage or vascular compromise caused by Mycobacterium tuberculosis, as well as metabolic abnormalities and host inflammatory responses. TB-related sepsis is a life-threatening acute complication for which current diagnostic and management approaches are likely inadequate. Therapeutic intensification and usage of immunomodulators are areas of ongoing research. Paradoxical reaction or symptom worsening during TB treatment may benefit from corticosteroids. Despite successful cure of TB, chronic complications can arise from anatomic alterations at disease sites. Examples include mycetomas developing within residual TB cavities, impaired pulmonary function, or focal neurologic deficits from tuberculomas. SUMMARY: Effective management of TB requires attention to potential structural, metabolic, vascular, and infectious complications. In some instances, individualizing treatment regimens may be necessary. Imunosuppression and other host factors predispose to complications; others occur despite adequate treatment. Public health TB programs and health systems require additional resources to provide comprehensive TB and post-TB care.

Multidetector computed tomography in life-threatening hemoptysis.

Life-threatening hemoptysis is a severe condition that requires rapid diagnosis and treatment. One of the treatments of choice is embolization. The initial assessment aims to locate the origin and cause of bleeding. The technological advance of the development of multidetector computed tomography (MDCT) has changed the management of patients with life-threatening hemoptysis. MDCT angiography makes it possible to evaluate the cause of bleeding and locate the vessels involved both rapidly and noninvasively; it is particularly useful for detecting ectopic bronchial arteries, nonbronchial systemic arteries, and pulmonary pseudoaneurysms. Performing MDCT angiography systematically before embolization enables better treatment planning. In this article, we review the pathophysiology and causes of life-threatening hemoptysis (including cryptogenic hemoptysis) and the MDCT angiography technique, and we review how to systematically evaluate the images (lung parenchyma, airways, and vascular structures).