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Research advances in Dieulafoy's disease of the bronchus (Review)

  • Authors:
    • Xiqian Xing
    • Jie Liu
    • Shuanglan Xu
    • Yishu Deng
    • Jiao Yang
  • View Affiliations

  • Published online on: December 1, 2021
  • Article Number: 100
  • Copyright: © Xing et al. This is an open access article distributed under the terms of Creative Commons Attribution License.

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Dieulafoy's disease is characterized by abnormal submucosal arteries and results in acute luminal hemorrhage. Dieulafoy's lesions can also be found in the submucosa of the bronchus. Due to its low incidence rate and non‑specific clinical symptoms, Dieulafoy's disease is easy to overlook, but can lead to massive bleeding and high rates of mortality. Therefore, improvements in the understanding of the disease are necessary. The awareness of the disease and associated diagnostic and treatment techniques have continued to improve, and thus, an increasing number of cases of Dieulafoy's disease of the bronchus have been reported. In the present review, 74 cases of Dieulafoy's disease are summarized. New technologies such as endobronchial ultrasound, narrow‑band imaging, angiography and argon plasma treatment have been found to be increasingly applied to diagnose and treat Dieulafoy's disease of the bronchus. Therefore, the primary focus of this systematic review is to highlight advances in the diagnosis and treatment of bronchial Dieulafoy's disease.

1. Introduction

Dieulafoy's disease, initially described as ‘exulceratio simplex’ (1) in 1897, is an acute luminal hemorrhage caused by the rupture of a feeding artery under the actions of external factors, or spontaneous rupture owing to vascular malformations of the gastrointestinal, biliary or bronchial wall; the feeding artery does not taper to capillaries after entering the submucosa, but remains constant in diameter and protrudes from the intestinal lumen. After >100 years of revisions and improved understanding, the disease was renamed as Dieulafoy's disease.

Dieulafoy's disease of the bronchus was first described by Sweerts et al (2) in 1995, and to date, <100 cases have been reported worldwide. However, as the condition is under-recognized and frequently underdiagnosed, the actual incidence is likely to be much higher. Therefore, it is necessary to increase our awareness of Dieulafoy's disease of the bronchus, which is considered to be one of the primary causes of massive hemoptysis (3). Although the natural history, diagnosis and preferred treatment of the disease are still unclear, Dieulafoy's disease of the bronchus is believed to be caused by both congenital (2) and acquired factors (4). In previous years, the awareness of the disease and associated techniques of diagnosis and treatment have continued to improve, and novel diagnostic technologies have been increasingly applied. The present review summarizes the research advances in Dieulafoy's disease of the bronchus.

2. Methods

The terms ‘bronchial OR bronchus’ AND ‘dieulafoy OR dieulafoy's’ were searched in the PubMed and Embase databases, covering the period between January 1985 and December 2019. Literature associated with a definitive diagnosis or a high suspicion of Dieulafoy's disease of the bronchus was screened, with abstracts of meetings excluded. There were no limitations on the language of the publications. A manual search was then conducted according to the reference lists of the published articles.

3. Pathogenesis

Dieulafoy's disease of the bronchus is pathologically characterized by the rupture and bleeding of a dilated or abnormal artery in the bronchial submucosa. The dilated or abnormal artery passes through the bronchial wall next to the bronchial cavity, and is surrounded by a thin mucosal epithelial layer. Abnormal vessels predominantly branch from the bronchial artery system and rarely from the pulmonary artery (5).

The pathogenesis of bronchial Dieulafoy's disease remains to be clarified. Most researchers believe that the disease is congenital, while others believe that it is acquired or is simply an abnormality of normal blood vessels (2,4-6). The etiology and pathogenesis of Dieulafoy's disease of the bronchus may be associated with congenital abnormalities of bronchial and pulmonary arteries, chronic inflammation or injury of the airway, and is also considered to be associated with long-term heavy smoking (7). Almost half of the patients are smoking or had smoked in our study (31 of the 74 patients had a history of smoking). Parrot et al (7) reported the cases of seven patients with Dieulafoy's disease of the bronchus, all of whom were long-term heavy smokers (mean smoking capacity, 49 packs/year), which supports the aforementioned findings. Moreover, some patients had a history of tuberculosis (6 of the 74 patients developed tuberculosis), indicating a possible association with inflammatory injury in tuberculosis or stretching and dilation of the bronchial artery (7). In addition, 15 patients developed other respiratory diseases and two patients had a history of cardiovascular disease (8). These findings indicate that Dieulafoy's disease of the bronchus may be associated with a history of basic diseases, especially respiratory diseases such as tuberculosis (9-12), pneumonia (13,14) and bronchiectasis (15).

4. Incidence

In total, 74 cases of bronchial Dieulafoy's disease have been identified in the past 20 years since the first reported case in 1995 (2,4-44). Owing to the substantially low incidence of Dieulafoy's disease of the bronchus, there have been no statistical reports of its exact incidence. Among the patients reported, the youngest was 5 years old, and the oldest was 85 years old. Subjects aged 30-70 years were at a high risk, accounting for 80% of the total patients (Table I). The male-to-female incidence ratio was ~2:1 (45 male vs. 24 female patients), although the reason for the sex differences in Dieulafoy's disease of the bronchus is not clear.

Table I

Clinical features of bronchial Dieulafoy's disease.

Table I

Clinical features of bronchial Dieulafoy's disease.

 Treatment type and outcome 
Authors, yearAge, yearsSexClinical manifestationsSmokerHistory of hemoptysisLocalization of hemoptysisEmbolizationSurgeryMedicineFollow-up periodRef.
Sweerts et al, 199535FemaleMassive hemoptysisNoYesRLLFailureSuccessNM4 months(2)
 59MaleHemoptysis and melaenaYesNoRLLNMSuccessNMNM 
Antoune et al, 199845MaleHemoptysisNMNMRULNMNMNMNM(42)
van der Werf et al, 199970FemaleAcute lobar pneumoniaYesNesLULFailureFailureFailureNM(16)
Stoopen et al, 200151MaleProfuse hemoptysis and epistaxisYesNMRML, RLLNMSuccessNMNM(17)
Hope-Gill and Prathibha, 200249MaleIncreasing hemoptysisYesNoRMLSuccessNMNM12 months(9)
Bhatia et al, 200342MaleRecurrent hemoptysisYesNoLULSuccessNMNMNM(10)
Kuzucu et al, 200528MaleMassive hemoptysisYesYesLLLNMSuccessNM12 months(11)
 45MaleMassive hemoptysisYesYesRMLNMSuccessNM5 months 
Pomplun and Sheaff, 200532MaleMassive hemoptysisNMNoRULFailureSuccessNMNM(5)
Loschhorn et al, 200647FemaleHemoptysisNoNoRMLNMSuccessNM6 years(18)
 52FemaleCoughed up several clots of bloodNoNoRLLSuccessNMNMNM 
Xie et al, 200628FemaleHemoptysisNoNoLLLNMNMSuccess5 years(25)
Savale et al, 200730NMHemoptysisNMYesNMFailureSuccessNM47±35 months(19)
 31NMHemoptysisNMYesNMFailureSuccessNM47±35 months 
 32NMHemoptysisNMYesNMFailureSuccessNM47±35 months 
 33NMHemoptysisNMYesNMFailureSuccessNM47±35 months 
 34NMHemoptysisNMYesNMFailureSuccessNM47±35 months 
Rennert et al, 200751MaleHemoptysisYesYesLLLNMSuccessNMNM(43)
Fields and De Keratry, 200847MaleHemoptysisYesYesLMBSuccessNMNM8 months(44)
Parrot et al, 200869MaleHemoptysisYesYesRULSuccessNMNM31 months(7)
 45MaleHemoptysisYesYesRULSuccessNMNM49 months 
 54MaleHemoptysisYesNoLLLSuccessNMNM96 months 
 57FemaleHemoptysisYesYesLULSuccessNMNM28 months 
 49MaleHemoptysisYesNoLULSuccessNMNM25 months 
 38MaleHemoptysisYesNoRLLSuccessNMNM6 months 
 68FemaleHemoptysisYesYesRULFailureFailureFailure12 months 
Gharagozloo et al, 200851MaleHemoptysisYesYesLLLNMSuccessNMNM(13)
D'Souza and Sharma, 201063FemaleBronchiectasis and chronic bronchitisNMNMRMLNMNMNMNM(20)
Gurioli et al, 201065MaleNMNoNMRLLNMNMNMNM(21)
Barisione et al, 201257FemaleMassive hemoptysisNoYesRML or RLLSuccessNMNMNM(6)
Kolb et al, 201244FemaleRecurrent hemoptysisNoYesRMLSuccessNMNMNM(22)
Trisolini et al, 201366FemaleRecurrent pneumoniasNoNoLLLNMSuccessNMNM(15)
Yang et al, 2013)41MaleRepeated hemoptysisNoYesRMLSuccessNMNMNM(26)
 36MalePulmonary infectionNMNMRML, RLLFailureFailureFailureNM 
 61MaleHemoptysis (4 days)NMNMRULNMSuccessNM5 years 
Fang et al, 201413MaleMassive hemoptysisNoYesRML, RLLNMSuccessNM5 months(23)
Smith et al, 201430MaleMassive hemoptysisNoYesRLLNMSuccessNM22 months(4)
Liu et al, 201418FemaleHemoptysisNoYesRLLNMSuccessNM60 months(39)
 31MaleCough and hemoptysisYesNoRLLSuccessNMNM12 months 
 33FemaleCough and hemoptysisNoYesRLLSuccessNMFailure13 months 
 36FemaleCough and hemoptysisNoYesRLLSuccessNMFailure14 months 
 47FemaleRight chest painNoNoRMLNMNMSuccess31 months 
Dalar et al, 201528MaleRecurrent hemoptysisYesYesLULSuccessNMNM2 years(24)
Xia et al, 201531MaleHemoptysisYesNMRMLFailureSuccessNM84 months(40)
 21MaleHemoptysisYesNMRMLFailureSuccessNM73 months 
 85MaleHemoptysisYesNMLULFailureSuccessNM20 months 
 63MaleHemoptysisNoNMLingulaNMSuccessNM70 months 
Padilla-Serrano et al, 201549FemaleCough and abundant blood expectorationYesYesRLLSuccessNMNMUntil 2015(41)
Hadjiphilippou et al, 201747MaleRecurrent hemoptysisYesYesRLLSuccessNMNMNM(28)
Madan et al, 201726MaleHemoptysisNoYesRLLSuccessNMNM6 months(29)
Niu et al, 20175FemaleHemoptysisNoYesRULNMNMSuccess12 months(30)
Wadji and Farahzadi, 201716FemaleMassive hemoptysisNoNoRLLNMSuccessNM18 months(31)
Yang et al, 201760FemaleFrequent hemoptysisNoYesNMSuccessNMNMUntil 2017(32)
Bonnefoy et al, 201866MaleMassive hemoptysis, acute respiratory failureNMNMRULNMSuccessNM6 months(33)
Minchole et al, 201867MaleDry cough and progressive dyspneaYesNoRMLNMNMSuccessNM(34)
Pan et al, 201876FemaleCoughNoNMRUL, RMLNMSuccessNMNM(12)
 66MaleHemoptysisYesNMRUL, RML, RLL, LLLNMNMSuccessNM 
 51MaleCough, sputum and feverYesNMRUL, RMLNMSuccessNMNM 
 36MaleFrequent lung infectionsNoNMRLLNMNMSuccessNM 
 41MaleHemoptysisYesNMRML, RLLNMNMSuccessNM 
Sheth et al, 201851FemaleHemoptysisNoNMLLLSuccessNMNM2 years(8)
 67MaleHemoptysisYesYesLLLSuccessNMNM6 months 
Wang et al, 201821FemaleMassive hemoptysisNoYesRLLSuccessNMNM13months(35)
Chen et al, 201918FemaleHemoptysisNoYesRLLSuccessNMNMUntil 2019(36)
 72FemaleCough and abundant blood expectorationNoYesRLLFailureFailureFailureNM 
 38MaleRecurrent hemoptysisNoYesLLSuccessNMNM2 years 
Tang et al, 201967MaleCough and hemoptysisNMNoRMLSuccessNMNMUntil 2019(37)
White et al, 201969MaleHemoptysisNoYesRLLSuccessNMNM6 months(38)
Zhou et al, 201962MaleIntermittent hemoptysisYesYesLLLSuccessNMNMNM(14)

[i] RUL, right upper lobe; RLL, right lower lobe; RML, right middle lobe; LUL, left upper lobe; LLL, left lower lobe; LL, left lung; LMB=left main bronchus; NM, not mentioned.

5. Lesion site

A total of 74 cases of Dieulafoy's disease of the bronchus were summarized, including 48 in the right bronchus (19 in the right lower lobe, 12 in the right middle lobe, 9 in the right upper lobe, 5 in the right lower and middle lobes, 2 in the right middle and upper lobes, and 1 in the entire right lung), 20 in the left bronchus (11 in the left lower lobe, 1 in the left main bronchus, 7 in the left upper lobe, and 1 in the left lung), 1 in the lingula, and 6 in an unspecified location. An analysis of the reported cases revealed that Dieulafoy's disease of the bronchus commonly occurs in the right bronchus, and that patients with lesions in the right bronchus account for approximately two-thirds of the total cases. These differences may be due to the anatomical characteristics of the bronchi. The right bronchus is short and thick so that foreign bodies are more likely to enter and cause infection, one of the potential causes of Dieulafoy's disease of the bronchus. Therefore, biopsies should be performed with caution to prevent hemorrhage in patients with cryptogenic hemoptysis if a lesion (especially in the right bronchus) with similar manifestations to Dieulafoy's disease is demonstrated by bronchoscopy.

6. Clinical manifestations

Recurrent hemoptysis is a common symptom of Dieulafoy's disease of the bronchus. The maximum amount of hemoptysis has been reported as 2,000 ml, and is often without an obvious cause (14). A previously reported patient with Dieulafoy's disease presented with chest pain and no hemoptysis; the latter only occurred after bronchoscopic biopsy, and a definitive diagnosis was established by bronchial angiography (39). Indeed, patients with Dieulafoy's disease frequently visit the hospital with a cough (12,36), infection (12,20) or respiratory failure (33,34). In conclusion, the clinical symptoms of Dieulafoy's disease of the bronchus are non-specific. Therefore, physicians should not only pay more attention to patients with massive hemoptysis, but also focus on patients with recurrent respiratory symptoms.

7. Auxiliary examination

In Dieulafoy's disease of the bronchus, chest X-rays and computed tomography (CT) scans are rarely positive for symptoms other than the manifestation of an intrapulmonary hemorrhage and the original lung disease. Only a few cases of endobronchial nodules have been identified by chest CT examination (17). By contrast, multi-slice CT angiography can clearly indicate the shape and direction of bronchial pulmonary vessels, which may detect a tortuous and dilated bronchial artery (22), and may also demonstrate well-enhanced endobronchial nodules (25).

Owing to cryptogenic hemoptysis, most patients who undergo bronchoscopic examination and bronchoscopy demonstrate massive endobronchial hemorrhage, which may even be accompanied by blood clot formation (12,36). Mucosal nodular projections can be a few millimeters in diameter and height with a smooth surface (36). The lesion may also be congested and rough, with slight vascular pulsation in specific cases. Sometimes the protruding surface is coated with yellow-white exudate forming a ‘little white hat’-like shape, easily misdiagnosed as an endobronchial tumor nodule (7) (Fig. 1). The nodule may show as a neoplasm-like granulation nodule, leading to local obstruction of the bronchial lumen and causing obstructive pneumonia. If the nodule is mistaken for a neoplasm, subsequent biopsy may lead to a large hemorrhage and death by suffocation. Abnormal vessels in the submucosa can be tortuous and dilated, and with a worm-like shape (Fig. 2), sometimes with fork-like ‘twigs’. This type of case is easily mistaken for submucosal tumor infiltration, and subsequent biopsies may lead to fatal hemorrhage. As the bronchial cavity is filled with blood and blood clots, it is difficult to find a small mucosal protrusion, or the mucosal protrusion is localized below the subsegmental bronchus and thus cannot be seen by conventional bronchoscopy.

Bronchial angiography contributes to the diagnosis of Dieulafoy's disease of the bronchus. Bronchial angiography shows a rich blood supply in the corresponding site of the lesion (23); the bronchial artery is tortuous, dilated and deformed, with signs of bleeding (Fig. 3).

An endobronchial ultrasound can be used to clarify the nature of any endobronchial protrusion. This technique helps to clarify the nature of the nodular lesion and provides clues for disease diagnosis. The major manifestation is a fluid echo-free zone in the submucosal lesion, and the Doppler mode can be used to detect blood flow (21) (Fig. 4).

8. Diagnosis

The possibility of Dieulafoy's disease of the bronchus should be considered in patients with hemoptysis when chest X-ray and CT examinations demonstrate no obvious abnormalities other than pulmonary hemorrhage. Dieulafoy's disease of the bronchus is largely diagnosed according to the presentations of bronchoscopy, bronchial angiography and pathology of surgical or autopsy specimens. Numerous researchers consider that pathological examination of biopsies, surgical or autopsy specimens is required for a definite diagnosis. However, there are no uniform diagnostic criteria, and since pathological biopsies can lead to fatal hemorrhages, the need for pathological diagnosis remains controversial. In some cases, the diagnosis is based on the manifestations revealed by bronchoscopy and bronchial angiography (24,25,39). Endobronchial ultrasonography (EBUS) is a new diagnostic method used to detect the lesions of Dieulafoy's disease (21).


Under bronchoscopy, a mucosal protrusion a few millimeters in diameter and height is visible in the corresponding site of the bleeding bronchus. On the top of the protrusion, the mucosa turns white without a pulsating sensation; the surrounding mucosa may be normal or slightly congested. In some cases, abnormal blood vessels within the submucosa are tortuous and dilated in an earthworm-like pattern, sometimes presenting with a purple nodular shape (39). To prevent uncontrollable hemorrhage, caution must be taken during biopsy if Dieulafoy's disease is suspected (based on the results of bronchoscopy). Yang et al (26) analyzed the clinical data of 22 patients with Dieulafoy's disease, including 12 with hemorrhage during biopsy. Bleeding stopped in eight patients after local hemostasis, and in two after lobectomy of the diseased lobe; another two patients died of massive hemorrhage. The remaining 10 patients did not undergo bronchoscopic biopsy. Our previous study reported the cases of six patients, including two undergoing bronchoscopic biopsy, both of whom suffered hemorrhage; the maximum amount of hemorrhage was ~1,000 ml, and bleeding stopped in both cases after selective bronchial artery embolization (39). Since 2014, with an improvement of the understanding of Dieulafoy's disease, biopsy has been avoided for nodules suspected to be caused by Dieulafoy's disease in the trachea (23,41), which has effectively reduced the probability of massive hemorrhage in Dieulafoy's disease.

Bronchial angiography

As aforementioned, bronchial angiography can be used to indicate the rich blood supply to the corresponding site of the lesion. The bronchial artery is tortuous, dilated and deformed, with signs of bleeding (39).

Multi-slice CT angiography of the bronchial or pulmonary artery

This technique is used to visualize an abnormal bronchial artery associated with tortuosity and dilation (22), and sometimes detects well-enhanced endobronchial nodules (25). A CT value of >100 for the enhanced lesion should be considered to indicate a vascular lesion.

Endobronchial ultrasound

When convex probe EBUS is used to detect blood flow within a lesion, it often shows a fluid echo-free zone in submucosal lesions; the blood flow can be displayed in the color or energy Doppler mode (21). Owing to its large diameter, convex probe EBUS cannot reach the upper lobe bronchus or segmental bronchus. Alternatively, radial probe EBUS can be used to examine the lesion. However, the latter approach has no Doppler mode and thus cannot determine blood flow within the lesion. Nonetheless, radial probe EBUS can indicate an echo-free zone, which may be considered for vascular lesions in patients with hemoptysis.

Narrow-band imaging

Wang et al (45) reported that narrow band imaging (NBI) can display endobronchial lesions such as bronchial artery-pulmonary artery fistulae. Thickened blood vessels and capillaries are tortuous and disordered in the submucosa of the lesion site. However, there have been no studies to assess the diagnostic value of NBI in Dieulafoy's disease.

Pathological examination

Pathology or autopsy pathology presents with arterial malformation in the bronchial submucosa. The tortuous, dilated, deformed artery forms small nodules coated with bronchial mucosa, protrudes from the bronchial lumen, and is only a few millimeters in diameter and height. In some cases, deformed blood vessels have an opening within the bronchial lumen, or the diseased bronchus is surrounded by rich blood vessels, some of which invade the bronchial wall and directly reach the submucosa (5). A diagnosis of Dieulafoy's disease depends on the results of pathological examination. However, since this can easily lead to massive bleeding, its use has been limited. Therefore, the incidence of Dieulafoy's disease may have been underestimated.

9. Differential diagnosis

Dieulafoy's disease and endobronchial hemorrhagic lesions are primarily distinguished by differential diagnosis. Additionally, the disease must be distinguished from early endobronchial cancer to avoid misdiagnosis.

Bronchial arteriovenous malformation (46)

Bronchial arteriovenous malformation can manifest as an endobronchial vascular lesion. The presence of abnormal blood vessels in the lesion can be demonstrated by EBUS or NBI, but it is difficult to distinguish via bronchoscopy. Bronchial angiography can be used to clarify the communication between the bronchial artery and the pulmonary circulation or cavernous hemangioma.

Bronchial artery aneurysm (47)

Bronchial artery aneurysm can manifest as an endobronchial vascular lesion. This condition can be distinguished from Dieulafoy's disease by bronchial angiography or multi-slide CT angiography as an aneurysm-like dilation of the bronchial artery.

Lobular capillary hemangioma (48)

This condition has no typical symptoms other than hemoptysis. Intraluminal neoplasms can be seen under bronchoscopy. Ulcers and bleeding are visible on the surface and are difficult to distinguish from Dieulafoy's disease of the bronchus. The differential diagnosis primarily relies on pathological examination.

Tracheal capillary hemangioma (49)

In general, tracheal capillary hemangioma is similar to tracheal lobular capillary hemangioma and Dieulafoy's disease of the bronchus in terms of clinical manifestations, laboratory examination results, imaging and endoscopy. The differential diagnosis primarily relies on pathological examination.

Early cancer

If Dieulafoy's disease is suspected based on bronchoscopy, and the possibility of early cancer cannot be ruled out, NBI and EBUS may be used to determine the presence of thickened and abnormally tortuous and disordered vessels within the lesion. Fluorescence bronchoscopy preliminarily determines the malignancy of the lesion.

10. Treatment and prognosis

Existing methods for the treatment of Dieulafoy's disease of the bronchus include conservative internal medication, selective bronchial artery embolization (SBAE), pulmonary lobectomy and argon plasma coagulation via bronchoscopy. Currently, SBAE is the preferred surgical approach, and lobectomy of the diseased lobe is used following embolization failure or recurrent post-embolization hemoptysis. Only one case of argon plasma coagulation via bronchoscopy has been reported (24).


As the condition presents with bleeding caused by the rupture of the bronchial or pulmonary artery, internal treatment with hemostatic agents often has poor efficacy for Dieulafoy's disease of the bronchus (39). However, for individual patients, pituitrin and thrombin may occasionally demonstrate good therapeutic effects (30).

Bronchoscopic treatment

Topical application of hemostatic drugs under bronchoscopy also has poor efficacy. To date, only Dalar et al (24) has reported the success of treating one patient with Dieulafoy's disease of the bronchus by argon plasma coagulation via bronchoscopy. However, the patient underwent no bronchial angiography or pathological diagnosis, and Dieulafoy's disease of the bronchus was diagnosed based solely on the presentations under bronchoscopy. Thus, the diagnosis of that patient remains controversial. In Dieulafoy's disease, once blood vessels rupture and bleed, the hemorrhage is fast and massive, resulting in an unclear field of view under bronchoscopy. Hence, it is our belief that bleeding from Dieulafoy's disease is not suited to dotted electrocoagulation and superficial hemostasis by argon-beam-coagulator burning, laser treatment and freezing under bronchoscopy. Nevertheless, this approach is feasible to remove blood clots that block the bronchial lumen and to clarify the bleeding site under bronchoscopy, thereby determining the lesion site for bronchial angiography or surgery. The use of a Dumon silicone stent for compression was reported in a patient with Dieulafoy's disease of the left main bronchus, and the patient was followed up for 8 months without recurrent hemorrhage (44). This study indicated that hemostasis by stent compression may be an alternative treatment option. Additionally, a bronchoscopic balloon can be used to compress the bronchus at the bleeding site to provide preparation time for SBAE. For the treatment of gastrointestinal Dieulafoy's disease, coagulant injection under digestive endoscopy can be used for hemostasis. However, no study has reported the use of this method for Dieulafoy's disease of the bronchus, thus the feasibility, safety and efficacy of the method are currently undetermined.


The SBAE procedure is effective in most patients with Dieulafoy's disease of the bronchus; however, hemoptysis may recur after surgery. Bhatia et al (10) reported the case of one patient with recurrent hemorrhage who had undergone SBAE seven times. Patients with abnormal blood vessels from the pulmonary artery are often non-responsive to SBAE. In our previous repor (39), all six patients with Dieulafoy's disease of the bronchus underwent SBAE at the corresponding site of hemorrhage. Hemorrhage was stopped in one patient following SBAE, who then underwent a pulmonary lobectomy; the other five patients all underwent SBAE and were followed up for 1 to 5 years. Furthermore, hemoptysis was not recurrent in four of the patients, though one patient did experience relapse.

Surgical treatment

In cases of SBAE failure, SBAE for hemoptysis relapse or no SBAE treatment, a lobectomy may be performed at the corresponding site of the lesion. Hemoptysis is unlikely to recur following resection of the diseased lung lobe. In a report by Yang et al (26), 13 patients underwent lobectomy of the diseased lobe; 12 did not experience recurrent hemoptysis, and one died due to hemorrhage after bronchoscopic biopsy, but not due to surgery.

11. Conclusions

Dieulafoy's disease of the bronchus lacks specificity in clinical symptoms and primarily presents as massive hemoptysis, although other respiratory or cardiovascular symptoms may also occur. Due to its relative rarity, respiratory physicians currently lack sufficient awareness of Dieulafoy's disease of the bronchus, thus the disease is likely to be missed or misdiagnosed. Serious consequences may result if attention is not paid to the diagnosis and treatment process. In particular, when local protrusion changes are found in the lumen during routine bronchoscopy, physicians perform routine biopsies which can cause fatal bleeding. Therefore, to rule out the possibility of Dieulafoy's disease of the bronchus, bronchial angiography, multi-slice CT angiography, EBUS and NBI examinations must be considered for patients undergoing bronchoscopy who present with cryptogenic hemoptysis and/or smooth protrusion within the bronchial lumen resembling Dieulafoy's disease (even those who do not experience hemoptysis). Biopsy must be prohibited if bronchial angiography demonstrates: i) The presence of a bronchial artery with abnormal tortuosity and dilation, as well as rupture and bleeding at the lesion site; ii) an enhanced CT value >100 for the lesion; or iii) EBUS and NBI results of abnormal blood flow within the lesion. Caution must be taken during bronchoscopic operations, such as biopsy and brushing, to prevent asphyxial hemorrhage. At present, SBAE and pulmonary lobectomy are the primary treatment methods for Dieulafoy's disease of the bronchus, although conservative drug treatment and flexible bronchoscope argon plasma coagulation have also been successfully used. SBAE can retain part of the function of the diseased lung, but may result in relapse following treatment. Lobectomy is a radical cure for Dieulafoy's disease, although complete removal of the diseased lung may affect the patient's quality of life. Thus, clinicians must assess the advantages and disadvantages, and select the most appropriate treatment method depending on the physical manifestations of each patient.


Not applicable.


Funding: The present study was supported by the Special and Joint Program of the Yunnan Provincial Science and Technology Department and Kunming Medical University [grant no. 2018FE001(-206)], the Yunnan Health Training Project of High Level Talents (grant no. and H-2018095), the Young Academic and Technical Leaders of Yunnan Province (grant no. 2017HB053), the Science and Technology Program for Public Wellbeing of Yunnan Province (grant no. 2014RA020).and the Famous Doctors of High-level Talent Training Support Program of Yunnan Province (grant nos. YNWR-MY-2020-013 and YNWR-MY-2020-027).

Availability of data and materials

Not applicable.

Authors' contributions

XX and JY were the primary contributors to the writing of the manuscript. SX and JL contributed to the diagnosis and differential diagnosis of bronchial Dieulafoy's disease. YD designed the study and made important revisions to the manuscript. Data authentication is not applicable. All authors read and approved the final manuscript.

Ethics approval and consent to participate

Not applicable.

Patient consent for publication

The patients provided consent for publication of the figures included in the manuscript.

Competing interests

The authors declare that they have no competing interests.



Dieulafoy G: Exulceratio simplex: Surgical intervention in overwhelming haematemesis following simple exulceration of the stomach. Bull Acad Med. 49:49–84. 1898.


Sweerts M, Nicholson AG, Goldstraw P and Corrin B: Dieulafoy's disease of the bronchus. Thorax. 50:697–698. 1995.PubMed/NCBI View Article : Google Scholar


Qian X, Du Q, Wei N, Wang M, Wang H and Tang Y: Bronchial Dieulafoy's disease: A retrospective analysis of 73 cases. BMC Pulm Med. 19(104)2019.PubMed/NCBI View Article : Google Scholar


Smith B, Hart D and Alam N: Dieulafoy's disease of the bronchus: A rare cause of massive hemoptysis. Respirol Case Rep. 2:55–56. 2014.PubMed/NCBI View Article : Google Scholar


Pomplun S and Sheaff MT: Dieulafoy's disease of the bronchus: An uncommon entity. Histopathology. 46:598–599. 2005.PubMed/NCBI View Article : Google Scholar


Barisione EE, Ferretti GG, Ravera SS and Salio MM: Dieulafoy's disease of the bronchus: A possible mistake. Multidiscip Respir Med. 7(40)2012.PubMed/NCBI View Article : Google Scholar


Parrot A, Antoine M, Khalil A, Théodore J, Mangiapan G, Bazelly B and Fartoukh M: Approach to diagnosis and pathological examination in bronchial Dieulafoy disease: A case series. Respir Res. 9(58)2008.PubMed/NCBI View Article : Google Scholar


Sheth HS, Maldonado F and Lentz RJ: Two cases of Dieulafoy lesions of the bronchus with novel comorbid associations and endobronchial ablative management. Medicine (Baltimore). 97(e9754)2018.PubMed/NCBI View Article : Google Scholar


Hope-Gill B and Prathibha BV: Bronchoscopic and angiographic findings in Dieulafoy's disease of the bronchus. Hosp Med. 63:178–179. 2002.PubMed/NCBI View Article : Google Scholar


Bhatia P, Hendy MS, Li-Kam-Wa E and Bowyer PK: Recurrent embolotherapy in Dieulafoy's disease of the bronchus. Can Respir J. 10:331–333. 2003.PubMed/NCBI View Article : Google Scholar


Kuzucu A, Gürses I, Soysal O, Kutlu R and Ozgel M: Dieulafoy's disease: A cause of massive hemoptysis that is probably underdiagnosed. Ann Thorac Surg. 80:1126–1128. 2005.PubMed/NCBI View Article : Google Scholar


Pan F, Wang F, Liu Z, Yuan F, Sun KK, Gao ZC and Sun Y: The computed tomography angiography features of Dieulafoy disease of the bronchu]. Zhonghua Jie He He Hu Xi Za Zhi. 41:949–953. 2018.PubMed/NCBI View Article : Google Scholar : (In Chinese).


Gharagozloo F, Rennert D, Margolis M, Tempesta B, Schwartz A, Cole V and Wang KP: Dieulafoy lesion of the bronchus: Review of the literature and report of the 13th case. J Bronchol. 15:38–40. 2008.


Zhou P, Yu W, Chen K, Li X and Xia Q: A case report and review of literature of Dieulafoy's disease of bronchus: A rare life-threatening pathologic vascular condition. Medicine (Baltimore). 98(e14471)2019.PubMed/NCBI View Article : Google Scholar


Trisolini R, Cancellieri A and Patelli M: Life-threatening bleeding after endobronchial biopsy in a patient with bronchiectasis. Am J Respir Crit Care Med. 188:e9–e10. 2013.PubMed/NCBI View Article : Google Scholar


van der Werf TS, Timmer A and Zijlstra JG: Fatal haemorrhage from Dieulafoy's disease of the bronchus. Thorax. 54:184–185. 1999.PubMed/NCBI View Article : Google Scholar


Stoopen E, Baquera-Heredia J, Cortes D and Green L: Dieulafoy's disease of the bronchus in association with a paravertebral neurilemoma. Chest. 119:292–294. 2001.PubMed/NCBI View Article : Google Scholar


Löschhorn C, Nierhoff N, Mayer R, Zaunbauer W, Neuweiler J and Knoblauch A: Dieulafoy's disease of the lung: A potential disaster for the bronchoscopist. Respiration. 73:562–565. 2006.PubMed/NCBI View Article : Google Scholar


Savale L, Parrot A, Khalil A, Antoine M, Théodore J, Carette MF, Mayaud C and Fartoukh M: Cryptogenic hemoptysis: From a benign to a life-threatening pathologic vascular condition. Am J Respir Crit Care Med. 175:1181–1185. 2007.PubMed/NCBI View Article : Google Scholar


D'Souza F and Sharma R: Dieulafoy's disease of the bronchus. Pathology. 42:683–684. 2010.PubMed/NCBI View Article : Google Scholar


Gurioli C, Casoni GL, Gurioli C, Tomassetti S, Romagnoli M, Ravaglia C and Poletti V: Endobronchial ultrasound in Dieulafoy's disease of the bronchus: An additional application of EBUS. Monaldi Arch Chest Dis. 73:166–168. 2010.PubMed/NCBI View Article : Google Scholar


Kolb T, Gilbert C, Fishman EK, Terry P, Pearse D, Feller-Kopman D and Yarmus L: Dieulafoy's disease of the bronchus. Am J Respir Crit Care Med. 186(1191)2012.PubMed/NCBI View Article : Google Scholar


Fang Y, Wu Q and Wang B: Dieulafoy's disease of the bronchus: Report of a case and review of the literature. J Cardiothorac Surg. 9(191)2014.PubMed/NCBI View Article : Google Scholar


Dalar L, Sökücü SN, Özdemir C, Büyükkale S and Altın S: Endobronchial argon plasma coagulation for treatment of Dieulafoy disease. Respir Care. 60:e11–e13. 2015.PubMed/NCBI View Article : Google Scholar


Xie BS, Chen YS, Lin MF, Huang QH and Lin ZS: Dieulafoy's disease of the bronchus: A case report and review of the literature. Zhonghua Jie He He Hu Xi Za Zhi. 29:801–803. 2006.PubMed/NCBI(In Chinese).


Yang RH, Li JF, Liu J, Sun KK, Cao ZL and Gao ZC: Dieulafoy disease of the bronchus: 3 cases report with literature review. Zhonghua Jie He He Hu Xi Za Zhi. 36:577–580. 2013.PubMed/NCBI(In Chinese).


Liu FL, Chen EG, Zhou P, Jin M, Yang L and Ying KJ: Tracheal lobular capillary hemangioma: Two case report and review of the literature. Zhonghua Jie He He Hu Xi Za Zhi. 33:849–852. 2010.PubMed/NCBI(In Chinese).


Hadjiphilippou S, Shah PL, Rice A, Padley S and Hind M: Bronchial dieulafoy lesion. A 20-year history of unexplained hemoptysis. Am J Respir Crit Care Med. 195(397)2017.PubMed/NCBI View Article : Google Scholar


Madan K, Dhungana A, Hadda V, Mohan A and Guleria R: Flexible bronchoscopic argon plasma coagulation for management of massive hemoptysis in bronchial Dieulafoy's disease. Lung India. 34:99–101. 2017.PubMed/NCBI View Article : Google Scholar


Niu HL, Yi P, Wang H, Wang FH, Liu W, Gao Q, Chen ZR, Xia JQ and Zeng RX: Infantile Dieulafoy's disease of bronchus: Report of a case. Zhonghua Bing Li Xue Za Zhi. 46:731–732. 2017.PubMed/NCBI View Article : Google Scholar : (In Chinese).


Wadji MB and Farahzadi A: Dieulafoy's disease of the bronchial tree: A case report. Sao Paulo Med J. 135:396–400. 2017.PubMed/NCBI View Article : Google Scholar


Yang D, Rong C, Gu J, Xu L, Zhang J, Zhang G and Shen C: Dieulafoy disease of the trachea with recurrent episodes of massive hemoptysis: A case report. Medicine (Baltimore). 96(e5855)2017.PubMed/NCBI View Article : Google Scholar


Bonnefoy V, Garnier M, Tavolaro S, Antoine M, Assouad J, Fartoukh M and Gibelin A: Bronchial Dieulafoy's disease: Visualization of embolization particles in bronchial aspirate. Am J Respir Crit Care Med. 198:954–955. 2018.PubMed/NCBI View Article : Google Scholar


Mincholé E, Penin RM and Rosell A: The utility of linear endobronchial ultrasound for the incidental finding of Dieulafoy disease of the bronchus. J Bronchology Interv Pulmonol. 25:e48–e50. 2018.PubMed/NCBI View Article : Google Scholar


Wang F, Kuang TG, Wang JF and Yang YH: A rare cause of recurrent fatal hemoptysis: Dieulafoy's disease of the bronchus. Chin Med J (Engl). 131:2758–2759. 2018.PubMed/NCBI View Article : Google Scholar


Chen W, Chen P, Li X, Gao X and Li J: Clinical characteristics and treatments for bronchial Dieulafoy's disease. Respir Med Case Rep. 26:229–235. 2019.PubMed/NCBI View Article : Google Scholar


Tang P, Wu T, Li C, Lv C, Huang J, Deng Z and Ding Q: Dieulafoy disease of the bronchus involving bilateral arteries: A case report and literature review. Medicine (Baltimore). 98(e17798)2019.PubMed/NCBI View Article : Google Scholar


White C, Ottaviano P, Munn N, Shweihat Y and Zeid F: Massive hemoptysis due to recurrence of bronchial to pulmonary vascular malformation: A case report. Respir Med Case Rep. 26:248–250. 2019.PubMed/NCBI View Article : Google Scholar


Liu Y, Li Y, Xing X, Xiao Y, Li Z, Yang Y and Wu X: Diagnosis and treatment of Dieulafoy's disease of the bronchus. China J Endosc. 20:795–799. 2014.


Xia XD, Ye LP, Zhang WX, Wu CY, Yan SS, Weng HX, Lin J, Xu H, Zhang YF, Dai YR, et al: Massive cryptogenic hemoptysis undergoing pulmonary resection: Clinical and pathological characteristics and management. Int J Clin Exp Med. 8:18130–18136. 2015.PubMed/NCBI


Padilla-Serrano A, Estrella-Palomares V, Martinez-Palacios B and Gonzalez-Spinola J: A case of massive hemoptysis related to a smoking-history: An acquired form of the Dieulafoy's disease? Rev Port Pneumol. 21:276–279. 2015.PubMed/NCBI View Article : Google Scholar


Antoune M, Maniacal G, Bazelly B, et al: Dieulafoy's vascular malformation of the bronchus report of 3 cases. In: 1998 Annual Meeting of the United States and Canada Academy of Pathology. 11(171)1998.


Rennert D, Gharagozloo F, Schwartz AM, Margolis M, Tempesta B and Wu J: Dieulafoy's lesion of the bronchus: Report of a case and review of the literature. Pathol Case Rev. 12:93–95. 2007.


Fields EL and De Keratry DR: Dieulafoy disease of the bronchus: Case report and presentation of a novel therapeutic modality. J Bronchology Interv Pulmonol. 15:107–109. 2008.


Wang T, Zhang J, Wang Y, Pei Y, Xu M and Zhang C: Diagnosis of bronchial artery - pulmonary artery fistula by the combination of narrow band imaging with bronchial arteriography: A case report. Zhonghua Jie He He Hu Xi Za Zhi. 38:148–149. 2015.


Sharifi M, Messersmith R, Newman B, Chung Y and Lakier JB: Bronchial arteriovenous malformation in a child with hemoptysis. A case report. Angiology. 47:203–209. 1996.PubMed/NCBI View Article : Google Scholar


Cerezo Lajas A, Rodríguez Guzmán MDC and de Miguel Díez J: Left Bronchial Artery Aneurysm. Arch Bronconeumol. 55(215)2019.PubMed/NCBI View Article : Google Scholar : (In English, Spanish).


Dermawan JK, Ko JS and Billings SD: Intravascular Lobular Capillary Hemangioma (Intravascular Pyogenic Granuloma): A Clinicopathologic Study of 40 Cases. Am J Surg Pathol: Jun 2, 2020 (Epub ahead of Print). doi: 10.1097/PAS.0000000000001509.


Özgül MA, Tanrıverdi E, Gül Ş, Asuk ZY, Acat M, Abbaslı K, Fener NA and Çetinkaya E: A Rare Cause of Hemoptysis in Childhood: Tracheal Capillary Hemangioma. Turk Thorac J. 18:131–133. 2017.PubMed/NCBI View Article : Google Scholar

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Xing X, Liu J, Xu S, Deng Y and Yang J: Research advances in Dieulafoy's disease of the bronchus (Review). Exp Ther Med 23: 100, 2022
Xing, X., Liu, J., Xu, S., Deng, Y., & Yang, J. (2022). Research advances in Dieulafoy's disease of the bronchus (Review). Experimental and Therapeutic Medicine, 23, 100.
Xing, X., Liu, J., Xu, S., Deng, Y., Yang, J."Research advances in Dieulafoy's disease of the bronchus (Review)". Experimental and Therapeutic Medicine 23.1 (2022): 100.
Xing, X., Liu, J., Xu, S., Deng, Y., Yang, J."Research advances in Dieulafoy's disease of the bronchus (Review)". Experimental and Therapeutic Medicine 23, no. 1 (2022): 100.