Hughes-Stovin syndrome: an unusual cause of pulmonary artery aneurysms

Ribeiro, Bruno Niemeyer de Freitas; Ribeiro, Renato Niemeyer de Freitas; Zanetti, Gláucia; Marchiori, Edson

doi:10.1590/0100-3984.2015.0048

Cited by 13 publications

(6 citation statements)

References 12 publications

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“…Roberts et al ( 38 ) demonstrated near perfect interobserver agreement for the severity and extent of bronchiectasis (κ = 0.87 and κ = 0.82, respectively), and moderate interobserver agreement for bronchial wall thickening. Diederich et al ( 26 ) found poorer interobserver agreement for those same three parameters (κ = 0.78, κ = 0.76, and κ = 0.64, respectively).…”

Section: Discussionmentioning

confidence: 95%

Radiologist agreement on the quantification of bronchiectasis by high-resolution computed tomography

et al. 2017

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ObjectiveTo evaluate radiologist agreement on the quantification of bronchiectasis by high-resolution computed tomography (HRCT).Materials and MethodsThe HRCT scans of 43 patients with bronchiectasis were analyzed by two radiologists, who used a scoring system to grade the findings. Kappa (κ) values and overall agreement were calculated.ResultsFor the measurement and appearance of bronchiectasis, the interobserver agreement was moderate (κ = 0.45 and κ = 0.43, respectively), as was the intraobserver agreement (κ = 0.54 and κ = 0.47, respectively). Agreement on the presence of mucous plugging was fair, for central distribution (overall interobserver agreement of 68.3% and κ = 0.39 for intraobserver agreement) and for peripheral distribution (κ = 0.34 and κ = 0.35 for interobserver and intraobserver agreement, respectively). The agreement was also fair for peribronchial thickening (κ = 0.21 and κ = 0.30 for interobserver and intraobserver agreement, respectively). There was fair interobserver and intraobserver agreement on the detection of opacities (κ = 0.39 and 71.9%, respectively), ground-glass attenuation (64.3% and κ = 0.24, respectively), and cysts/bullae (κ = 0.47 and κ = 0.44, respectively). Qualitative analysis of the HRCT findings of bronchiectasis and the resulting individual patient scores showed that there was an excellent correlation between the observers (intraclass correlation coefficient of 0.85 and 0.81 for interobserver and intraobserver agreement, respectively).ConclusionIn the interpretation of HRCT findings of bronchiectasis, radiologist agreement appears to be fair. In our final analysis of the findings using the proposed score, we observed excellent interobserver and intraobserver agreement.

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Section: Discussionmentioning

confidence: 95%

Radiologist agreement on the quantification of bronchiectasis by high-resolution computed tomography

et al. 2017

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“…Even though oro-genital ulcers and uveitis are uncommon in HSS, some consider it an incomplete form of BD due to the lack of other features of complete BD. 5 These individual PAAs may be recognized as complete PAAs or pulmonary artery pseudoaneurysms (PAP). 3 Pseudoaneurysms are histologically characterized by the involvement of only the external layers of the arterial wall (the media and adventitia) and are at greater risk of ruptures and bleeding.…”

Section: Discussionmentioning

confidence: 99%

Hughes-Stovin Syndrome: An Experience of Management of 3 Cases From a Tertiary Health Care Centre

Sachdev

Yadav

Balakrishnan

et al. 2023

Clin Med�Insights�Arthritis�Musculoskelet Disord

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“…Depending on the extent of pulmonary artery aneurysm and potential bleeding, surgical resection or angiographic embolization might be considered. [11,12] In cases of inadequate cyclophosphamide response TNF-α-Antagonists (alone or in combination with cyclophosphamide) may offer a solution in managing life-threatening complications. [13] CONSENT Signed informed consent was obtained from the patient for publication of this case report.…”

Section: Discussionmentioning

confidence: 99%

Severe cardiovascular involvement in Hughes-Stovin syndrome

Groga‐Bada

Klumpp

Fougère

et al. 2017

CRIM

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Hughes-Stovin syndrome (HSS) is a rare autoimmune vasculitis and is characterized by the simultaneous presence of deep venous thrombosis and pulmonary artery aneurysms. The exact etiology and pathogenesis of this life-threatening syndrome is currently unknown. The disease is thought to be a variant of Behcet's disease with major vascular involvement. Here we report a case of a 19-year-old man from West Africa with a severe cardiovascular manifestation of HSS. The patient was referred to our hospital with dyspnoea, recurrent fever and swelling of the left leg. Echocardiography revealed extensive biventricular thrombi. He responded very well to immunosuppressive therapy in combination with anticoagulation using low-molecular-weight heparins (LMWHs). Thrombolysis was consciously avoided.

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Hughes-Stovin syndrome: an unusual cause of pulmonary artery aneurysms

Cited by 13 publications

References 12 publications

Radiologist agreement on the quantification of bronchiectasis by high-resolution computed tomography

Radiologist agreement on the quantification of bronchiectasis by high-resolution computed tomography

Hughes-Stovin Syndrome: An Experience of Management of 3 Cases From a Tertiary Health Care Centre

Severe cardiovascular involvement in Hughes-Stovin syndrome

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