|
 
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| REVIEW ARTICLE |
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| Year : 2023 | Volume
: 7
| Issue : 1 | Page : 2-12 |
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Approach to pulmonary haemorrhage in children: What could it be?
Anna Marie Nathan1, Hng Shih Ying1, Eg Kah Peng1, Nadia Fareeda Muhammad Gowdh2, Jessie de Bruyne1
1 Department of Paediatrics, University Malaya, Kuala Lumpur, Malaysia
2 Department of Biomedical Engineering, University Malaya, Kuala Lumpur, Malaysia
| Date of Submission | 07-Feb-2023 |
| Date of Decision | 02-Mar-2023 |
| Date of Acceptance | 03-Mar-2023 |
| Date of Web Publication | 07-Apr-2023 |
Correspondence Address:
Prof. Anna Marie Nathan
Department of Paediatrics, University Malaya Medical Centre, 59100 Lembah Pantai, Kuala Lumpur
Malaysia
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/prcm.prcm_4_23
Pulmonary haemorrhage is rare in children but can be life-threatening. It is recognised as a triad of haemoptysis, drop in haemoglobin or iron-deficiency anaemia and radiographic evidence of pulmonary infiltrates. Although there can be a myriad of causes, careful history with consideration of co-morbid factors and radio imaging of the chest can help determine the most likely cause. This review will illustrate how to determine the aetiology, types of investigations to perform and management through real-life clinical vignettes. Keywords: Blood, child, haemoptysis, management, pulmonary haemorrhage
How to cite this article:
Nathan AM, Ying HS, Peng EK, Gowdh NF, de Bruyne J. Approach to pulmonary haemorrhage in children: What could it be?. Pediatr Respirol Crit Care Med 2023;7:2-12 |
How to cite this URL:
Nathan AM, Ying HS, Peng EK, Gowdh NF, de Bruyne J. Approach to pulmonary haemorrhage in children: What could it be?. Pediatr Respirol Crit Care Med [serial online] 2023 [cited 2023 Jun 1];7:2-12. Available from: https://www.prccm.org/text.asp?2023/7/1/2/373841 |
| Introduction | |  |
Pulmonary haemorrhage (PH), which is the extravasation of blood into the airways and alveoli, is recognised by the triad of a drop in haemoglobin/iron-deficiency anaemia, pulmonary infiltrates and haemoptysis.[1],[2] In children with PH, haemoptysis is seen only in 56.2% of cases, resulting in delayed diagnosis.[3],[4] A decline in blood haemoglobin levels over a few days without haemolysis or any haemorrhage elsewhere should alert a physician to the possibility of PH.[5]
PH presents insidiously or suddenly: common symptoms are poor growth, reduced effort tolerance, chronic iron deficiency or sudden cyanosis with cardiorespiratory arrest/failure from massive haemorrhage. Massive haemorrhage is defined as blood loss of >8mL/kg within 24 h or if associated with cardiorespiratory arrest/failure.[6] Massive haemoptysis is usually seen in patients with a bleeding from the bronchial circulation, whereas mild bleeds may be seen in pulmonary bleeds.[7] Diffuse disease is usually from a low-pressure, high-volume circulation, for example, within the alveoli, like in diffuse alveolar haemorrhage (DAH). In contrast, the focal disease is usually seen in a high-pressure, low-volume circulation, like from the bronchial circulation.[8]
| Case 1 | | |
A 16-year-old girl presented to the hospital with a sudden onset of haemoptysis. She was otherwise well with no weight loss or reduced appetite, no fever, no contact history of tuberculosis (TB), no reduced effort tolerance or shortness of breath or cyanosis, and no rash or symptoms to suggest connective tissue disease. All investigations were normal, including full blood count, chest radiograph (CXR), computer tomography (CT) and auto-immune screen. On further questioning, she had just gone to stay with her mother and stepfather and was stressed by the home situation. In the ward, blood was seen during her retching period. She was diagnosed with Mallory Weiss tear and discharged well.
| Discussion: Is It Really from the Lung? | | |
One of the first issues faced by a child with “haemoptysis” is to exclude haematemesis, Mallory Weiss tear, oesophagitis, and nose or throat bleeds versus true blood from the lower airway. [Table 1] delineates how we can differentiate blood from the upper airway, lower airway and the gastrointestinal system. A detailed history and even pictures are paramount in the initial approach to “haemoptysis” to avoid unnecessary investigations. | Table 1: Clinical differences between blood from the airway (haemoptysis) and blood from the gastrointestinal system (haematemesis)* or upper airway
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| Case 2: Haemoptysis in a Child with Down Syndrome | | |
A 3-year-old boy with Down syndrome presented with a massive PH requiring invasive ventilation. He was born term and was diagnosed with a patent ductus arteriosus (PDA) that was managed conservatively. Antenatally, he was diagnosed with a right middle lobe (RML) congenital pulmonary airway malformation. At 3 months of age, he had an elective video-assisted thoracoscopic RML lobectomy and right upper lobe (anterior segmentectomy) as this was deemed suspicious. Subsequently, he continued to have recurrent admissions for pneumonia with persistent changes seen in the right upper and middle zones of the CXR [Figure 1]. Bronchoscopy was performed at 2-years-old and confirmed the diagnosis of chronic suppurative lung disease. This was considered secondary to his severe respiratory syncytial virus pneumonia and silent aspiration due to suck-swallow incoordination. His parents, unfortunately, defaulted treatment and only reappeared when he had a massive haemoptysis with cyanosis and severe anaemia (Hb 47 g/dL). Extensive investigations finally revealed a focal bleed from the right upper and remnant middle lobe [Figure 2]. Computerised tomography angiogram (CTA) did not show the source of the bleed. Finally, cardiac catheterisation found abnormally dilated and tortuous aorta-pulmonary collaterals (APCs) supplying these areas. The tortuous APCs and the PDA were coiled. He remains well, nearly after the bleed. | Figure 1: Serial chest radiographs of the patient when he was admitted for pneumonia
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 | Figure 2: Bronchoscopy red dichromatic imaging (RDI) mode showing blood coming out from the right middle lobe [blue arrow]
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| Aetiology of Pulmonary Haemorrhage | | |
Determining the aetiology of PH is challenging, especially in the above case of Down syndrome, where there are so many possibilities. Numerous tests can be done to determine the aetiology [Table 2]. However, these should be prioritised based on the most likely diagnosis and differential diagnoses.
The first task is to determine if this is a local versus diffuse bleed. This can be assisted by looking at the CXR. As in case 2, the differential was narrowed down by looking at his serial CXRs [Figure 1] and noting persistent consolidation of the right upper and mid-zone. Flexible bronchoscopy (FB) also confirmed that the PH was focal.
[TAG:2]Role Bronchoscopy (Rigid or Flexible)[8][/TAG:2]
Bronchoscopy, when performed in a controlled setting, is very useful diagnostically and therapeutically. In a massive bleed, FB should be performed within <48 h of presentation as this will increase the yield to find the source of the bleed, especially if it is a local bleed. Using a scope that can flush and suck adequately, for example, a 2.0 mm working channel, would be ideal to allow clearance of blood clots and improve oxygenation. It will also allow better visualisation of the area to determine or exclude possible aetiologies, for example, tumour, foreign body and airway anomalies. Visualisation using the red diffusion index mode, which enhances the visibility of blood vessels and bleeding sources in deeper tissue by using narrow-band light at two centre wavelengths (600 and 630 nm) in the red band, is very useful, especially when there is a lot of blood[9],[10] [Figure 2].
During bronchoscopy, the aims would be to (a) determine the site of bleeding if focal, (b) look for abnormal mucosa, tumour or masses, (c) flush 50–60 mL aliquots of saline × 3 within the same lobe, and inspect the colour of the effluent after each aliquot.[8] If more blood or persistent blood is coming out as you flush, this is more likely a DAH rather than a focal bleed. Finally, using cold saline or adrenaline: 1:10,000–1:20,000 can be therapeutic to control the bleeding.[11] Some have successfully used factor VIIa for massive bleeds.[12] Bronchoscopic alveolar lavage (BAL) samples should be sent for (a) cytology to look for haemosiderin-laden macrophages (including counts) and eosinophil count, (b) bacterial culture, (c) TB smear, culture and polymerase chain reaction/ GeneXpert, (d) fungus for microscopy and culture, (e) galactomannan antigen for Aspergillus and (f) multiplex polymerase chain reaction for viruses.
| Role of Imaging to Determine the Aetiology | | |
Radiographic investigations are vital as initial investigations, whether only CXRs or CT [Table 3]. If unable to perform FB immediately, multidetector CT with CTA can determine the source by identifying unusual or aberrant arteries, enlarged pathological bronchial arteries or pseudoaneurysms[13] [Figure 3]. A computed tomography pulmonary angiography (CTPA) is necessary if suspecting pulmonary embolism.[14] In DAH, CT will show diffuse ground glass with thickened septae increasing crazy paving [Figure 4].  | Figure 3: (A) Chest radiograph showed bilateral patchy consolidation and nodular air space opacity at left upper lobe in a patient with haemoptysis and smear positive TB. (B) CT angiography axial view and (C) CT angiography coronal view. Dilated bronchial vessels (blue arrows) adjacent to a cavitation with lack of tapering signifying presence of a pseudoaneurysm. This patient was treated with anti-TB medication and his haemoptysis improved
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 | Figure 4: Diffuse bilateral ground glass opacities predominantly in the right upper (A + B) and lower lobes (C) involving the subpleural and central regions with areas of consolidation in the central regions of the right upper lobe. Areas of ground glass opacities with thickened interlobular septa giving rise to a crazy paving pattern seen predominantly in the right upper lobe and apical segment of right lower lobe
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An Egyptian study of mainly adults found that CT helped detect the bleeding in 84% of patients.[15] CTA is not always successful, as in our case 2, where two CTAs were performed and had a negative yield. Angiography or cardiac catheterisation is the next step.[16]
| Role of Cardiac Catheterisation in the Management of Haemoptysis | | |
Cardiac catheterisation (CC) is not commonly performed for children with haemoptysis.[16],[17] However, it can help detect focal bleeding sources from various pathologies such as APCs, arteriovenous malformations (AVMs) and enlarged or pathological bronchial arteries when other conventional radiological techniques have failed. CC can also be therapeutic with the performance of transcatheter interventions. In a retrospective study from the cardiac centre at Children’s Hospital of Michigan, over 15 years, only 21 cases were performed for PH.[17] The patients’ median (range) age was 17 years (range 0.3–60). CC detected the cause of the bleeding in 81% of patients, and two-thirds had a transcatheter procedure: aortopulmonary collateral embolisation, aortopulmonary, venovenous collateral embolisation and pulmonary arteriovenous malformation embolisation.[17] Although recurrent haemoptysis was frequent (50%) postintervention, the final effectiveness of transcatheter interventions was 79%. In a smaller study published by Zaidi et al.,[16] they, too, found CC useful in identifying and treating APCs. Bronchial artery embolisation is also a well-accepted therapy in adults with massive or recurrent haemoptysis and children with cystic fibrosis.[13],[18],[19] However, this procedure can be more dangerous as inadvertent embolisation of the spinal arteries may cause paraplegia. Unfortunately, the expertise to perform CC in children is only available in some centres.
| Case 3: TB or Not TB? | | |
A 14-year-old girl presented with a dry cough for 2 weeks and haemoptysis. There was no fever, but she had lost 2 kg over the past 3 months. Her father had been treated for TB 2 years ago. Initial CXR showed mild infiltration of the right upper lobe. Her CRP was 23 mg/L (normal < 5 mg/L), and she had microcytic hypochromic anaemia. Her sputum acid-fast baccilli smear was negative. She had three other visits to the emergency department with haemoptysis over the next 4 weeks. Her repeat CXR showed lung cavities, and she was referred to us for further management. Her TB culture was positive for Mycobacteria tuberculosis.
| Discussion: Infection in Haemoptysis | | |
Whereas there are many causes of haemoptysis, infection is a common cause. TB is the most common infection associated with haemoptysis in Asia.[20] It is surprising that TB can even be forgotten by many as a cause of haemoptysis, even in the setting of a positive contact history, like the patient above. In a recent review article looking at haemoptysis, TB was not even mentioned as a cause of haemoptysis.[21] de Silva et al.[1] looked at 44 children managed in their centre and found TB to be the most common infection associated with PH. In a systematic review by Simon et al.,[22] TB was the 3rd most common infectious aetiology. TB can also be associated with complications like bronchial artery pseudoaneurysms, which can cause haemoptysis.[23]
In a review of infections that can cause DAH, the following were identified as possible causes: cytomegalovirus, adenovirus, invasive aspergillosis, Mycoplasma, Legionella, and Strongyloides in the immunocompromised during influenza A (H1N1) infection, dengue, Leptospirosis, Malaria and Staphylococcus aureus infection in the immunocompetent patient.[24]
| Case 4: Vascular Anomalies | | |
A 5-year-old girl was referred for recurrent bouts of haemoptysis (blood clots) since the age of 2 years. She is a second twin, born at 33 weeks gestational age, complicated with poor weight gain and necrotising enterocolitis. On examination, she was not clubbed, not cyanosed, and tachypnoeic with reduced breath sounds and dullness to percussion over the left lower zone. CTPA revealed an absent left pulmonary vein. Bronchoscopy revealed dilated blood vessels that easily bled, over the carina, and left the main bronchus and left lower lobe [Figure 5]. She was managed conservatively due to parental refusal of treatment and remains well. | Figure 5: (A) Dilated sub mucosal blood vessels at the carina and leading into the left main bronchus. (B) Dilated blood vessels at the submucosal region of the left main bronchus. Endobronchial changes with dilation of the dense submucosal venous plexus can often be seen in bronchoscopy
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| Vascular Malformations of the Heart-lung | | |
These can cause both diffuse as well as focal bleeding [Table 3].
Pulmonary vein stenosis
Haemoptysis with a history of mediastinal masses, granulomatous diseases, interventional procedures for atrial fibrillation therapy, lobectomy or right-sided pneumonectomy, pulmonary hypertension, recurrent respiratory infections or after lung transplantation should prompt the suspicion of pulmonary venous stenosis.[25]
The pulmonary and bronchial circulation drain via the pulmonary veins into the left atrium. Thus, in pulmonary vein stenosis, the drainage systems of both lung circulations are blocked. Typical consequences include distended pleural-hilar bronchial veins, alveolar haemorrhage, a friable endobronchial mucosa, reduced lymphatic drainage, interstitial pulmonary oedema, enlarged hilar lymph nodes, enlarged lymph vessels and sometimes a pleural effusion.[25],[26],[27],[28]
In pulmonary venous stenosis, the expectorated blood is deoxygenated and, therefore, usually darker compared to haemoptysis with offspring of the systemic bronchial arteries.[29] Other presentations include tussive irritation, exertional dyspnoea, recurrent pulmonary infections and signs of pulmonary venous hypertension. Endobronchial changes with dilatation of the dense submucosal venous plexus can often be seen in bronchoscopy. The alveolar haemorrhage will result in a bloody bronchoalveolar lavage or, if occult, an increased number of haemosiderin-laden macrophages in the cell differentiation.[29] Due to the dense network between the pulmonary and bronchial circulation, extensive collaterals between both circulations may develop, with the possible occurrence of secondary bronchial and pulmonary venous varices in the long run. Misinterpretation of these varices and collaterals as pulmonary arteriovenous malformations has been reported. Bronchial artery embolisation may be deleterious in cases of hindered pulmonary venous drainage.
CT is superior in detecting pulmonary venous stenosis/atresia compared to transthoracic echocardiogram. Phase contrast magnetic resonance imaging also has its advantages of absent radiation, reduced invasiveness and visualisation of mediastinal structures and being able to provide information regarding blood flow. However, CT can give additional information about lung parenchyma.[29]
Arteriovenous malformations
These are defined as abnormal pulmonary arteries to venous connections without interconnecting capillaries.[30] Pulmonary AVMs occur with an incidence of 1 in 2500, with 80% occurring in patients with Hereditary Haemorrhagic Telangiectasia (HHT).[31] Besides haemoptysis, asymptomatic hypoxaemia, stroke, or brain abscess are other presentations of AVMs. AVMs can be simple or complex. Simple AVMs make up 80% of AVMs. Complex AVMs have multiple feeding blood vessels, while diffuse AVMs occupy all lung segments and are associated with cyanosis and clubbing.
HHT is usually inherited in an autosomal dominance trait. Patients present with nose bleeds, iron-deficiency anaemia and an increased risk of pulmonary hypertension.[32]
Aorta-pulmonary collaterals
These are usually blood vessels from the descending aorta, subclavian artery, and bronchial arteries and supply the terminal respiratory unit or bronchioles. It is commonly seen in conditions associated with reduced pulmonary blood flow, for example, tetralogy of Fallot and pulmonary atresia. They have also been identified in premature infants with BPD. Non-bronchial systemic arteries commonly supply the lungs in patients with chronic lung disease.[13] These usually regress and may not require treatment. A review of seven patients, mainly infants with APCs without cardiac disease, presented at a mean age of 3 months and six out of seven required embolisation, which was highly successful. They found that helical CT scans were not useful in detecting these APCs. However, cardiac catheterisation was far superior as diagnostic and therapeutic if embolisation or coiling was performed in one setting.[16]
APC vessels have been reported as a frequent source of bleeding in congenital heart disease (CHD) patients.[33] It is hypothesised that APCs develop due to the need for a secondary source of pulmonary blood flow in CHD patients because of chronic hypoxia and altered pulmonary vasculature.[12] Patients after the Fontan operation are likely to develop significant APCs with an estimated haemoptysis rate of 4%.[33] Interestingly, APCs have also been reported as the source of haemoptysis in children without CHD.[8],[13]
Bronchial artery malformations, for example, dieulofoys disease
Bronchial artery malformations are well known for massive bleeds and require treatment in 90% of cases, that is, bronchial artery embolisation or surgical resection.[13] Worldwide, TB is the most common cause of non-massive haemoptysis.[12],[33] These malformations are also seen in diseases with chronic inflammation, for example, cystic fibrosis, bronchiectasis and fibrotic scarring, aspergillomas or invasive mycotic infections and fibrotic scarring.[12],[34] These can be diagnosed via contrast-enhanced CT, but angiography is more sensitive. This is similar to a pseudoaneurysm.
Another rare but very interesting disease is Dieulofoy’s disease.[33],[35] These appear small (usually < 1 cm in diameter), smooth and elevated nodules, with white pointed caps and a ridge-like bulge between the nodules. These sessile nodules are usually covered with normal-appearing bronchial mucosa and appear benign. However, they are fed by tortuous dysplastic bronchial arteries in the submucosa, from which vascular branches derive that can be located in the mucosa and biopsy of these lesions can be fatal. Diagnosis is confirmed via angiography.[35] Bronchial artery embolisation can be performed in the same setting and is said to be successful in 96.1% of cases.[33],[36]
| Case 5: Pulmonary Hemosiderosis | | |
A 14-month-old girl presented with iron-deficiency anaemia requiring multiple blood transfusions. Initially, she did not have any respiratory tract symptoms. At 4-years-old, she presented with haemoptysis, cough, fever and shortness of breath. The initial diagnosis of pneumonia. However, her CT chest showed DAH. All investigations were negative, including her connective tissue screen. No lung biopsy was performed. On the basis of her previous history of persistent iron-deficiency anaemia and CT changes, she was diagnosed with idiopathic pulmonary haemosiderosis and treated with pulses of IV methylprednisolone. Whereas she did seem to respond, her disease relapsed three times over a 15-year period. This was usually associated with the cessation of prednisolone. A lung biopsy was then performed, which showed evidence of capillaritis. Her diagnosis was changed to idiopathic pulmonary capillaritis, and she was treated with pulse cyclophosphamide.
| Discussion: Diffuse Alveolar Haemorrhage | | |
DAH occurs due to the disruption of the alveolar-capillary basement membrane in the lung, resulting in bleeding into the alveolar spaces.[37] Injury can be immune- or non-immune mediated, with subsequent involvement of the blood vessels and the alveolar septae.
DAH is best diagnosed when diffuse changes in CT scan plus bronchoscopic evidence of haemosiderin-laden macrophages. Classically, persistent or increasing blood on three sequential lavage aliquots from one affected area of the lung supports the diagnosis of DAH.[8],[38] The causes of DAH are shown in [Table 3]. The majority listed is immune-mediated.
| Worth a Mention: Idiopathic Pulmonary Hemosiderosis | | |
Idiopathic pulmonary hemosiderosis (IPH) would be a diagnosis of exclusion in children with an unknown cause of DAH. This would be one of the most common causes of haemorrhage.[1],[5] Lung biopsies show bland alveolar haemorrhage, with large amounts of haemosiderin-laden macrophages in the alveoli. There is a distinct absence of inflammation, capillaritis and vasculitis in histology. A review on IPH found it more commonly in girls, with only one-quarter of them having positive auto-antibodies: anti-nuclear antibody, 20.3%; anti-neutrophil cytoplasmic antibody, 17%; anti-dsDNA, 9.1%; RF, 12%; anti-smooth muscle antibody, 23.2%; and celiac antibodies, 25.9%.[39] Cow’s milk protein allergy was present in 16.2% of the children.[39] The significance of an association between IPH and the presence of autoantibodies is unclear, as the autoantibodies could be suggestive of an overall immune dysregulation rather than causation. However, limited evidence suggests that the presence of anti-nuclear antibody may be associated with a higher risk of recurrence and worse outcomes.[39]
Immune dysregulation diseases associated with PH. There is mounting evidence of the role of genetic immune dysregulation diseases that cause systemic vasculitis associated with haemoptysis, for example, coatomer associated protein subunit alpha[40],[41] and sting-associated vasculopathy of infancy (SAVI)[42],[43] [Table 4]. A review of 14 patients with coatomer associated protein subunit alpha syndrome, all had symptoms before 12 years, had a positive family history of disease, and had both diffuse lung disease and arthritis.[40] Symptoms were either respiratory and/or joint manifestations. Only two patients presented with anaemia and fatigue secondary to PH. Eventually, 50% had a PH. All subjects were positive for anti-neutrophil cytoplasmic antibody, antinuclear antibody, or both and 71% of patients were rheumatoid factor positive. The most common pulmonary findings included cysts on chest computed tomography and evidence of follicular bronchiolitis on lung biopsy. All were treated with immunosuppressive medication, that is, methylprednisolone, cyclophosphamide and/or rituximab. Longitudinal data demonstrated improvement in chest radiology but an overall decline in pulmonary function despite chronic treatment.[41]
SAVI, on the other hand, typically manifests as neonatal-onset systemic inflammation, interstitial lung disease (ILD), and severe cutaneous vasculopathy in acral regions, including fingers, toes, ears and nose. The majority present with interstitial lung disease rather than PH and other autoimmune signs.[42],[43]
| Authors Indications for Lung Biopsy | | |
- Suspect IPH or autoimmune disease
- Female
- Negative CTD markers
- Poor response to first line immunomodulators for example, steroids
- Recurrent or relapsing disease > 2 times
- Inability to wean off steroids due to relapse
- Need to use steroid sparing medication due to complications from steroids.
| Management of Pulmonary Haemorrhage | | |
Management of PH can be divided into general and specific management strategies.[21]
The general management includes the following:
- Reducing hypoxia: May require intubation with adequate PEEP if it is a massive bleed. Can consider non-invasive ventilation if not massive bleeding and the patient is stable.
- Correcting the anaemia: Blood transfusion
- Correcting any coagulopathy: Suggested to correct if platelet count is <50,000 × 106/L or PT > 1.5
- In patients with significant/massive bleeding: Can consider use of factor VIIa[12] or tranexamic acid (TXA)[44] or IVI vasopressin[12]
Factor VIIa acts via the extrinsic pathway. It can be used either via the intrapulmonary or intravenous route. It has been reported to be useful for the general management of DAH, whether immune or not.[21] However, it may be less effective in children < 1-year-old and those with haematological malignancies.[12]
Both systemic and local administration of TXA have been used in the prophylaxis and treatment of bleeding diathesis, whether the bleeding is congenital or acquired.[25],[26] TXA acts by binding to plasminogen, which in turn inhibits its binding to fibrin. Activation to plasmin is thus impaired. TXA has been used in the intravenous, aerosolized and intrapulmonary form to treat PH.[45]
| Specific Management Depends on the Aetiology | | |
1. Treating infections: anti-bacterial, antifungal or antiviral
2. If suspected or autoimmune or idiopathic pulmonary haemosiderosis:
a. IVI methylprednisolone (MTP) 30 mg/kg 3–5 days then taper over 4 weeks with oral prednisolone or prednisolone 30 mg/kg/day then taper. Studies have shown that lower doses of methylprednisolone may be sufficient.[46]
b. Oral hydroxychloroquine (6–10 mg/kg/OD) as a maintenance therapy.[46],[47]
3. For resistant or recurrent haemoptysis from an autoimmune cause:
a. Pulse cyclophosphamide (CYC) has been used in the management of PH associated with SLE and oral azathioprine as an immunosuppressant.[48]
4. Rituximab (RTX) has gained interest, since its success in connective tissue disease or autoimmune vasculitis. RTX is a chimeric monoclonal antibody that targets CD20. Concurrent use of MTP, CYC and RTX has been shown to improve remission compared to use of MTP and CYC alone in patients with anti-neutrophil cytoplasmic antibody +ve connective tissue disease[49],[50] as well as in SLE.[48] RTX has also been shown to result in improved survival and long-term remission.[51]
5 .Life threatening haemoptysis secondary to autoimmune cause of haemoptysis:
a. IV Ig G at or plasmapheresis[21]
6. Abnormal vasculature for example, AVM, APCs and Aberrant/dilated bronchial arteries
a. Ideally embolisation and/or ligation of these abnormal bleeding vessels are essential to stop the bleeding. However, it may be difficult to do it small and require expertise.[13],[36]
| Summary | | |
Managing a child with haemoptysis is challenging and requires critical analyses of the case to avoid unnecessary tests as well as not to delay the diagnoses. Important steps are first to determine if (a) the blood is from the lung or elsewhere, (b) if from the lung, is it focal or diffuse bleed, (c) exclude infection, as this is common and easily treatable, (d) if this is a DAH, what autoimmune problem could it be and (e) treat the problem depending on the severity of the bleed and cause.
Financial support and sponsorship
This article was partially sponsored by the Malaysian Thoracic Society, 2018.
Conflicts of interest
There are no conflicts of interest.
Authors contribution
AMN, HSY, EKP, NFMG and JdB were involved in writing and editing this article.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]
[Table 1], [Table 2], [Table 3], [Table 4]
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