Pattern of pulmonary vasculitis and major vascular involvement in Hughes-Stovin syndrome (HSS): brief report of eight cases

AbstractTo describe the pattern of pulmonary artery vasculitis and the characteristic computed tomographic pulmonary angiography (CTPA) signs in patients with Hughes-Stovin syndrome (HSS). In a retrospective study, the medical records of eight HSS patients (six men), seen between February 2008 and January 2018, were reviewed regarding history, disease characteristics, laboratory investigations, imaging, and treatments. The mean (SD) age was 37.375 ± 8.65 years (range 30–55) and mean (SD) follow-up 30 ± 41.60 months (range 9–132). In all patients, routine laboratory investigations and complete coagulation profile were done. In all, CTPA studies were performed as well as and Doppler ultrasound for suspected deep vein thrombosis (DVT). Four patients had a history of thrombophlebitis, and DVT was observed in all, in two cases bilateral. Arterial thromboses involving popliteal, tibial, common iliac, and femoral arteries were observed in one patient. All patients had mild to moderate hemoptysis, and one had massive hemoptysis. None of the patients had a history of recurrent mouth and/or genital ulcers, uveitis, or arthritis. In all patients, CTPA identified bilateral pulmonary artery aneurysms (PAAs) with adherent in situ thrombosis and mural enhancement in all patients. Lobar PA branches were involved in all patients, segmental in six and main PA in five patients. Proper immunomodulators were initiated early, with favorable outcome; none was treated with TNF-α antagonists. HSS is a systemic vasculitis that may affect virtually all major veins and arteries in patients with normal coagulation profile. PAAs, adherent in situ thrombosis, and mural wall enhancement are characteristic CTPA signs. Early treatment with immunomodulators is essential.Key Points• Hughes Stevin syndrome (HSS) is a systemic vasculitis that may affect virtually all major veins and arteries in patients. It has a normal coagulation profile.• Computed tomography (CT) pulmonary angiography is considered to be the most important diagnostic tool to assess the degree and the extent of the characteristic pulmonary artery aneurysms, and in situ thrombosis, and mural wall enhancement.• It is likely that HSS syndrome is often not recognized and misdiagnosed as deep venous thrombosis (DVT) with pulmonary thromboembolism.• Early treatment with combined immunomodulators is essential to ensure favorable outcome.

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Pulmonary vasculitis in Hughes-Stovin syndrome (HSS): a reference atlas and computed tomography pulmonary angiography guide—a report by the HSS International Study Group

Clinical Rheumatology ◽

10.1007/s10067-021-05912-3 ◽

2021 ◽

Author(s):

Yasser Emad ◽

Yasser Ragab ◽

Cal Robinson ◽

Sonia Pankl ◽

Pablo Young ◽

...

Keyword(s):

Computed Tomography ◽

Pulmonary Artery ◽

Systemic Vasculitis ◽

International Study ◽

Pulmonary Angiography ◽

Study Group ◽

Computed Tomography Pulmonary Angiography ◽

International Study Group ◽

Pulmonary Vasculitis ◽

Pulmonary Artery Aneurysms

Abstract Introduction Hughes-Stovin syndrome (HSS) is a systemic vasculitis characterized by widespread venous/arterial thrombosis and pulmonary artery aneurysms (PAAs), which is associated with serious morbidity and mortality. All fatalities reported in HSS resulted from unpredictable fatal suffocating hemoptysis. Therefore, it is necessary to recognize pulmonary complications at an early stage of the disease. Objectives The aims of this study are to develop a reference atlas of images depicting the characteristic features of HSS by computed tomography pulmonary angiography (CTPA). To make a guide for physicians by developing a classification of PAAs according to the severity and risk of complications associated with each distinct lesion type. Methods The Members of the HSS International Study Group (HSSISG) collected 42 cases, with high-quality CTPA images in one radiology station and made reconstructions from the source images. These detailed CTPA studies were reviewed for final image selection and approved by HSSISG board members. We classified these findings according to the clinical course of the patients. Results This atlas describes the CTPA images that best define the wide spectrum of pulmonary vasculitis observed in HSS. Pulmonary aneurysms were classified into six radiographic patterns: from true stable PAA with adherent in-situ thrombosis to unstable leaking PAA, BAA and/or PAP with loss of aneurysmal wall definition (most prone to rupture), also CTPA images demonstrating right ventricular strain and intracardiac thrombosis. Conclusion The HSSISG reference atlas is a guide for physicians regarding the CTPA radiological findings, essential for early diagnosis and management of HSS-related pulmonary vasculitis. Key Points• The Hughes-Stovin syndrome (HSS) is a systemic vasculitis characterized by extensive vascular thrombosis and pulmonary artery aneurysms (PAAs) that can lead to significant morbidity and mortality.• All fatalities reported in HSS were related to unpredictable massive hemoptysis; therefore, it is critical to recognize pulmonary complications at an early stage of the disease.• The HSS International Study Group reference atlas classifies pulmonary vasculitis in HSS at 6 different stages of the disease process and defines the different radiological patterns of pulmonary vasculitis notably pulmonary artery aneurysms, as detected by computed tomography pulmonary angiography (CTPA).• The main aim of the classification is to make a guide for physicians about this rare syndrome. Such a scheme has never been reached before since the first description of the syndrome by Hughes and Stovin since 1959. This classification will form the basis for future recommendations regarding diagnosis and treatment of this syndrome.

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Aneurysm of the Pulmonary Artery in Fallot’s Tetralogy

Case Reports in Cardiology ◽

10.1155/2017/1384905 ◽

2017 ◽

Vol 2017 ◽

pp. 1-4

Author(s):

Kawtar Afrikh ◽

Loua Hattach ◽

Nadia Fellat ◽

Mustapha El Bakkali ◽

Halima Benjelloun

Keyword(s):

Pulmonary Artery ◽

Ductus Arteriosus ◽

Left Pulmonary Artery ◽

Case Presentation ◽

Fallot's Tetralogy ◽

Fallot’S Tetralogy ◽

History Of ◽

Pulmonary Artery Aneurysms ◽

Magnetic Resonance Imaging Mri ◽

Arterial Aneurysms

Introduction. Pulmonary artery aneurysms are a rare entity. Etiologies of these findings are multiple, but they are exceptionally associated with Fallot’s Tetralogy. In this study, we present an unusual case of an important aneurysm of the left pulmonary artery associated with Fallot’s Tetralogy disease. Case Presentation. A 30-year-old woman has been admitted for dyspnea and cyanosis. The data which had been obtained from echocardiography, cardiac catheterization, and angio-magnetic resonance imaging (MRI) suggested the existence of an important aneurysm of the left pulmonary artery associated with a regular Fallot’s disease with a pulmonic stenosis. We have noticed the presence of a small restrictive patent ductus arteriosus (PDA). Therefore, the patient was referred to surgical correction. Conclusion. Pulmonary artery aneurysms associated with Fallot’s Tetralogy are rarely reported. The natural history of these rare arterial aneurysms has to be clarified.

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Spontaneous pulmonary artery thrombus in a neonate

The Egyptian Heart Journal ◽

10.1186/s43044-021-00167-4 ◽

2021 ◽

Vol 73 (1) ◽

Author(s):

Y. S. Shrimanth ◽

Krishna Prasad ◽

Adari Appala Karhtik ◽

Parag Barwad ◽

C. R. Pruthvi ◽

...

Keyword(s):

Pulmonary Artery ◽

Respiratory Distress ◽

Disease Risk ◽

Maternal Diabetes ◽

Left Pulmonary Artery ◽

Pulmonary Angiography ◽

Haemodynamic Instability ◽

Artery Thrombosis ◽

Heart Disease Risk Factors ◽

Heart Disease Risk

Abstract Background Pulmonary artery thrombosis is rare in neonates and mimics as persistent pulmonary hypertension or congenital heart disease. Risk factors include septicemia, dehydration, polycythemia, maternal diabetes, asphyxia, and inherited thrombophilias. They present with cyanosis and respiratory distress. Careful echocardiogram assessment helps in identifying the thrombus in the pulmonary artery and its branches. Computed tomography pulmonary angiography confirms the diagnosis. Case presentation We present a case of term neonate who presented with respiratory distress and cyanosis and a detailed echocardiogram revealed thrombus in the origin of left pulmonary artery. The neonate was managed initially with unfractionated heparin and later with low molecular weight heparin with which there was significant resolution of the thrombus Conclusion Spontaneous pulmonary artery thrombosis though rare should be suspected in any cyanotic neonate with respiratory distress. Management in these cases depends on the haemodynamic instability and lung ischemia.

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North polar trough formation due to in-situ erosion as a source of young ice in mid-latitudinal mantles on Mars

Scientific Reports ◽

10.1038/s41598-021-83329-3 ◽

2021 ◽

Vol 11 (1) ◽

Author(s):

J. Alexis P. Rodriguez ◽

Kenneth L. Tanaka ◽

Ali M. Bramson ◽

Gregory J. Leonard ◽

Victor R. Baker ◽

...

Keyword(s):

Katabatic Wind ◽

Spiral Pattern ◽

Near Surface ◽

Valuable Source ◽

History Of ◽

North Polar ◽

Human Exploration ◽

Erosional Event

AbstractThe clockwise spiral of troughs marking the Martian north polar plateau forms one of the planet’s youngest megastructures. One popular hypothesis posits that the spiral pattern resulted as troughs underwent poleward migration. Here, we show that the troughs are extensively segmented into enclosed depressions (or cells). Many cell interiors display concentric layers that connect pole- and equator-facing slopes, demonstrating in-situ trough erosion. The segmentation patterns indicate a history of gradual trough growth transversely to katabatic wind directions, whereby increases in trough intersections generated their spiral arrangement. The erosional event recorded in the truncated strata and trough segmentation may have supplied up to ~25% of the volume of the mid-latitude icy mantles. Topographically subtle undulations transition into troughs and have distributions that mimic and extend the troughs’ spiraling pattern, indicating that they probably represent buried trough sections. The retention of the spiral pattern in surface and subsurface troughs is consistent with the megastructure’s stabilization before its partial burial. A previously suggested warm paleoclimatic spike indicates that the erosion could have occurred as recently as ~50 Ka. Hence, if the removed ice was redeposited to form the mid-latitude mantles, they could provide a valuable source of near-surface, clean ice for future human exploration.

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Development of a Field Deployable Firebrand Flux and Condition Measurement System

Fire Technology ◽

10.1007/s10694-020-01074-x ◽

2020 ◽

Author(s):

Simone Zen ◽

Jan C. Thomas ◽

Eric V. Mueller ◽

Bhisham Dhurandher ◽

Michael Gallagher ◽

...

Keyword(s):

Imaging Techniques ◽

Time History ◽

Infrared Camera ◽

Thermal Conditions ◽

Particle Characterization ◽

Fire Dynamics ◽

Rate Of Spread ◽

New Instrument ◽

History Of

AbstractA new instrument to quantify firebrand dynamics during fires with particular focus on those associated with the Wildland-Urban Interface (WUI) has been developed. During WUI fires, firebrands can ignite spot fires, which can rapidly increase the rate of spread (ROS) of the fire, provide a mechanism by which the fire can pass over firebreaks and are the leading cause of structure ignitions. Despite this key role in driving wildfire dynamics and hazards, difficulties in collecting firebrands in the field and preserving their physical condition (e.g. dimensions and temperature) have limited the development of knowledge of firebrand dynamics. In this work we present a new, field-deployable diagnostic tool, an emberometer, designed to provide measurement of firebrand fluxes and information on both the geometry and the thermal conditions of firebrands immediately before deposition by combining a visual and infrared camera. A series of laboratory experiments were conducted to calibrate and validate the developed imaging techniques. The emberometer was then deployed in the field to explore firebrand fluxes and particle conditions for a range of fire intensities in natural pine forest environments. In addition to firebrand particle characterization, field observations with the emberometer enabled detailed time history of deposition (i.e. firebrand flux) relative to concurrent in situ fire behaviour observations. We highlight that deposition was characterised by intense, short duration “showers” that can be reasonably associated to spikes in the average fire line intensity. The results presented illustrate the potential use of an emberometer in studying firebrand and spot fire dynamics.

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THU0597 CORNEAL MELT - DON’T ALWAYS BLAME RHEUMATOID ARTHRITIS

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2020-eular.2643 ◽

2020 ◽

Vol 79 (Suppl 1) ◽

pp. 540.3-540

Author(s):

A. Munir ◽

C. Sheehy

Keyword(s):

Rheumatoid Arthritis ◽

Renal Biopsy ◽

Systemic Vasculitis ◽

Lower Lobe ◽

Visual Outcome ◽

Eye Drops ◽

Poor Visual Outcome ◽

Methyl Prednisolone ◽

History Of ◽

And Function

Background:Corneal melt is a rare inflammatory disease of the peripheral cornea; it may lead to perforation of the globe and visual failure. Corneal melt can be a manifestation of systemic vasculitis in patients with RA and other conditions, such as cancer. Without early and aggressive treatment it may be associated with a poor visual outcome and a high mortality. It has been reported in patients with stable RA.Objectives:A case report in a patient with long standing but well controlled Rheumatoid Arthritis (RA) and metastatic disease.Methods:A 75 year old male with a background of sero positive Rheumatoid Arthritis for more than 10 years presented to the Eye Casualty with a two week history of a painful left red eye. His other medical history was significant for Stage IIB poorly differentiated cancer of the left lower lobe. Left lower lobectomy with a patch of diaphragm resected. Intratumoural lymphovascular invasion noted. He completed Adjuvant Carboplatin/Vinorelbine chemotherapy September, 2017. He had DVT proximal left leg 22ndof September, 2017. Follow up CT in 2018 demonstrated a right renal upper pole lesion for which he was awaiting biopsy with?metastatic lung disease vs primary renal carcinoma. His RA was well controlled on Methotrexate 10mg weekly. He had been treated by the ophthalmology team for left marginal Keratitis for the prior 2 months with steroid eye drops without significant improvement. On presentation to ED, he described sharp eye pain, waking him from the sleep, associated with watery discharge and photophobia. Examination showed corneal melt in left eye involving 25% of inferior portion of the cornea and spastic entropion with injecting eye lashes. He had no active joints and there were no other signs of vasculitis. CRP was 4.1. He had a negative ANA and ANCA; viral swabs were negative. He was admitted under the medical team. Intravenous Methyl Prednisolone was started. The patient felt better after 5 days of Methyl Prednisolone. Left temporary tarsorrhaphy was done by Ophthalmology. Cyclophosphamide was initiated after discussion with Oncologist pending the result of the renal biopsy. Patient was discharged after 5 days of admission in the hospitalResults:The renal biopsy was positive for metastatic Squamous cell carcinoma of lung. Cyclophosphamide was withdrawn and he was started on Carboplatin/Gemcitabine. The corneal melt improved with complete resolution of his visual symptoms.Conclusion:In this case, although the history of RA was felt by the ophthalmology team to be the most likely association with the corneal melt, we would argue the oncological diagnoses were likely the driving force behind the presentation.References:[1]Sule A, Balakrishnan C, Gaitonde S, Mittal G, Pathan E, Gokhale NS, et al. Rheumatoid corneal melt. Rheumatology (Oxford)2002;41:705–6.[2]S. Yano, K. Kondo, M. Yamaguchi et al., “Distribution and function of EGFR in human tissue and the effect of EGFR tyrosine kinase inhibition,” Anticancer Research, vol. 23, no. 5, pp. 3639–3650, 2003.Disclosure of Interests:None declared

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In Situ exploration of the giant planets

Experimental Astronomy ◽

10.1007/s10686-021-09775-z ◽

2021 ◽

Author(s):

O. Mousis ◽

D. H. Atkinson ◽

R. Ambrosi ◽

S. Atreya ◽

D. Banfield ◽

...

Keyword(s):

Solar System ◽

Giant Planets ◽

Atmospheric Composition ◽

Formation History ◽

History Of ◽

Composition Structure ◽

Galileo Probe ◽

Probe Measurements

AbstractRemote sensing observations suffer significant limitations when used to study the bulk atmospheric composition of the giant planets of our Solar System. This impacts our knowledge of the formation of these planets and the physics of their atmospheres. A remarkable example of the superiority of in situ probe measurements was illustrated by the exploration of Jupiter, where key measurements such as the determination of the noble gases’ abundances and the precise measurement of the helium mixing ratio were only made available through in situ measurements by the Galileo probe. Here we describe the main scientific goals to be addressed by the future in situ exploration of Saturn, Uranus, and Neptune, placing the Galileo probe exploration of Jupiter in a broader context. An atmospheric entry probe targeting the 10-bar level would yield insight into two broad themes: i) the formation history of the giant planets and that of the Solar System, and ii) the processes at play in planetary atmospheres. The probe would descend under parachute to measure composition, structure, and dynamics, with data returned to Earth using a Carrier Relay Spacecraft as a relay station. An atmospheric probe could represent a significant ESA contribution to a future NASA New Frontiers or flagship mission to be launched toward Saturn, Uranus, and/or Neptune.

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Gaia DR1 completeness within 250 pc & star formation history of the Solar neighbourhood

Proceedings of the International Astronomical Union ◽

10.1017/s1743921317006159 ◽

2017 ◽

Vol 12 (S330) ◽

pp. 148-151 ◽

Cited By ~ 1

Author(s):

Edouard J. Bernard

Keyword(s):

Star Formation ◽

Star Formation History ◽

Solar Neighbourhood ◽

The Sun ◽

Thick Disc ◽

Formation History ◽

Magnitude Diagram ◽

History Of

AbstractWe took advantage of the Gaia DR1 to combine TGAS parallaxes with Tycho-2 and APASS photometry to calculate the star formation history (SFH) of the solar neighbourhood within 250 pc using the colour-magnitude diagram fitting technique. We present the determination of the completeness within this volume, and compare the resulting SFH with that calculated from the Hipparcos catalogue within 80 pc of the Sun. We also show how this technique will be applied out to ~5 kpc thanks to the next Gaia data releases, which will allow us to quantify the SFH of the thin disc, thick disc and halo in situ, rather than extrapolating based on the stars from these components that are today in the solar neighbourhood.

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The merger-driven evolution of massive early-type galaxies

Proceedings of the International Astronomical Union ◽

10.1017/s1743921320002434 ◽

2020 ◽

Vol 15 (S359) ◽

pp. 62-66

Author(s):

Carlo Cannarozzo ◽

Carlo Nipoti ◽

Alessandro Sonnenfeld ◽

Alexie Leauthaud ◽

Song Huang ◽

...

Keyword(s):

Mass Velocity ◽

Selection Criteria ◽

Velocity Dispersion ◽

Ex Situ ◽

Scaling Relations ◽

Early Type ◽

History Of ◽

Kinematic Properties ◽

Shed Light

AbstractThe evolution of the structural and kinematic properties of early-type galaxies (ETGs), their scaling relations, as well as their stellar metallicity and age contain precious information on the assembly history of these systems. We present results on the evolution of the stellar mass-velocity dispersion relation of ETGs, focusing in particular on the effects of some selection criteria used to define ETGs. We also try to shed light on the role that in-situ and ex-situ stellar populations have in massive ETGs, providing a possible explanation of the observed metallicity distributions.

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Uretero-Iliac artery fistula: a rare cause of haematuria

BMJ Case Reports ◽

10.1136/bcr-2019-232189 ◽

2020 ◽

Vol 13 (9) ◽

pp. e232189

Author(s):

Natalia Hernandez ◽

Bethany Desroches ◽

Eric Peden ◽

Raj Satkunasivam

Keyword(s):

Iliac Artery ◽

Percutaneous Nephrostomy ◽

Arterial System ◽

Endoscopic Evaluation ◽

Stent Removal ◽

Retrograde Pyelogram ◽

History Of ◽

Gross Haematuria ◽

Multiple Episodes

A woman in her mid-forties with a history of cervical cancer requiring chemoradiation presented with bilateral ureteral strictures secondary to radiation therapy. The ureteral obstruction was initially relieved with bilateral percutaneous nephrostomy tubes, and subsequently, bilateral ureteral stents. Over the course of 8 months, she presented with multiple episodes of severe gross haematuria. This persisted even after stent removal and conversion back to percutaneous nephrostomy tubes. The initial evaluation, done with concern for an uretero-iliac artery fistula, which included bilateral retrograde pyelograms and CT angiography was non-diagnostic. Given continued haematuria, repeat endoscopic evaluation was undertaken; on retrograde pyelogram, brisk contrast was seen to pass into the arterial system, consistent with a left ureteroarterial fistula. The patient underwent endovascular iliac artery stent placement. Subsequently, the patient underwent resection of the iliac artery with endovascular graft in situ, left distal ureterectomy with proximal ureteral ligation following femoral-to-femoral bypass. This allowed for complete resolution of the patient’s gross haematuria episodes.

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