Pulmonary artery pseudoaneurysms (PAPs) in Hughes-Stovin syndrome (HSS) as an emerging concept for a potentially fatal course

Introduction: Hughes-Stovin syndrome (HSS) is a systemic vasculitis characterized by pulmonary artery aneurysms (PAA) and widespread venous and arterial thrombosis. Fatalities in HSS resulted from unforeseeable fatal suffocating hemoptysis. It is critical to early detect pulmonary involvement to take timely measures against inevitable serious life-threatening complications. Case presentation: A 27 year old Filipino male patient presented to the emergency department with an acute attack of massive hemoptysis. The markers of inflammation were elevated with anemia and a normal coagulation profile. The patient had bilateral lower limb edema with tender calf muscles. Color Doppler ultrasound revealed bilateral deep vein thrombosis. Importantly, an urgent computerized tomography pulmonary angiography (CTPA) revealed bilateral large PAAs which matched the pattern of pseudoaneurysms described by the HSS international study group (HSSISG). There was no history of iridocyclitis, recurrent oral or genital ulcers. Accordingly, the patient was diagnosed with HSS. The patient received intravenous pulse methylprednisolone (1 g/3 days), then oral prednisone (1 mg/kg/d/3 months), and monthly pulse cyclophosphamide (1 g) for three months. The patient remained symptom-free, yet on the third pulse of cyclophosphamide;he patient contracted corona virus disease-2019 (COVID-19) infection and died three weeks later from a fatal episode of massive hemoptysis. The case was discussed and the recent literature was reviewed relative to fatal hemoptysis associated with pulmonary artery pseudoaneurysms (PAPs) pattern in HSS Conclusion(s): HSS presenting with massive hemoptysis could be lifethreatening and the PAPs are considered a double edged sword. Detailed description of such rare cases is warranted for optimum future management.Copyright © 2023

Frail Silk: Is the Hughes-Stovin Syndrome a Behçet Syndrome Subtype with Aneurysm-Involved Gene Variants?

Hughes-Stovin syndrome is a rare disease characterized by thrombophlebitis and multiple pulmonary and/or bronchial aneurysms. The etiology and pathogenesis of HSS are incompletely known. The current consensus is that vasculitis underlies the pathogenic process, and pulmonary thrombosis follows arterial wall inflammation. As such, Hughes-Stovin syndrome may belong to the vascular cluster with lung involvement of Behçet syndrome, although oral aphtae, arthritis, and uveitis are rarely found. Behçet syndrome is a multifactorial polygenic disease with genetic, epigenetic, environmental, and mostly immunological contributors. The different Behçet syndrome phenotypes are presumably based upon different genetic determinants involving more than one pathogenic pathway. Hughes-Stovin syndrome may have common pathways with fibromuscular dysplasias and other diseases evolving with vascular aneurysms. We describe a Hughes-Stovin syndrome case fulfilling the Behçet syndrome criteria. A MYLK variant of unknown significance was detected, along with other heterozygous mutations in genes that may impact angiogenesis pathways. We discuss the possible involvement of these genetic findings, as well as other potential common determinants of Behçet/Hughes-Stovin syndrome and aneurysms in vascular Behçet syndrome. Recent advances in diagnostic techniques, including genetic testing, could help diagnose a specific Behçet syndrome subtype and other associated conditions to personalize the disease management.

Pulmonary Artery Aneurysm in a Teenager with Diabetes and COVID-19 Infection

Introduction: Pulmonary artery aneurysms (PAAs) are exceedingly rare. Etiology includes congenital, idiopathic, and acquired. Bacterial and fungal infections are the most common acquired causes. Herein described is a patient with new-onset diabetes mellitus I (DM1) with COVID-19 infection complicated by PAA and mucormycosis. Case Description: A 17-year-old female with new-onset DM1 was admitted to the PICU with diabetic ketoacidosis, and COVID-19 infection complicated by multifocal necrotizing pneumonia. She was treated with remdesivir, antibiotics, systemic glucocorticoids, and discharged on inhaled glucocorticoids. Two weeks later she presented with hemoptysis. Chest computed tomography angiography (CTA) showed a resolving necrotizing pneumonia with a 16 mm aneurysmal dilatation of the proximal portion of the right inferior pulmonary artery (RIPA). Hemoptysis resolved, with no intervention required. One month later she presented again with hemoptysis. Repeat chest CTA demonstrated increasing aneurysmal dilatation, measuring 20 mm in diameter. Echocardiography showed no evidence of endocarditis, congenital heart defects, or elevated right ventricular pressures. A comprehensive infectious workup was negative (Table 1). Due to recurrent symptoms, progressive aneurysmal enlargement, and concerns for rupture, patient underwent RIPA occlusion by cardiac catheterization. Two months later hemoptysis recurred. Chest CTA revealed erosion of the occlusion device into the right inferior segmental bronchus. She underwent emergent right middle and lower lobectomy, and arterial bronchial fistula repair. Lung histology revealed non-septate hyphae with peribronchial and perivascular necrotizing granulomas concerning for mucormycosis (Figure 1). She was treated with amphotericin B and discharged on oral posaconazole. Discussion: The incidence of PAA in adults is estimated to be 1 in 14,000 patients. In adults, the upper limit of normal of an interlobar PA by CTA is 17mm. Our patient's RIPA was dilated up to 20 mm, for which she underwent occlusion of the RIPA. The proinflammatory state generated by COVID-19 can result in vascular inflammation and ultimately aneurysmal dilatation. Desnos et al. reported four cases of hemothorax secondary to PAA rupture in COVID-19 patients on ECMO for severe ARDS. The etiology for PAA formation in our patient had a complex interplay of factors including new-onset diabetes, COVID-19 vasculitis, exposure to systemic glucocorticoids, and an opportunistic infection with Mucor spp. Mucormycosis in diabetic patients with COVID-19 has a mortality of 31% in adults. We believe that the lobectomy performed for the management of PAA in our patient led to better outcomes since surgical debridement is a mainstay of mucormycosis treatment, along with antifungal therapy. Conclusion: PAA in children is uncommon. We describe a diabetic patient with COVID-19 pneumonia, complicated by PAA and mucormycosis. In patients with COVID-19 presenting with hemoptysis, it is important to have a high index of suspicion for PAA. Furthermore, diabetic patients with COVID-19 treated with systemic steroids can be at increased risk for mucormycosis. (Table Presented).

A Rare Case of Peripheral Pulmonary Artery Aneurysm and Cavitating Pneumonia in a Patient with COVID-19 Managed with an Endovascular Method.

Peripheral pulmonary artery aneurysm (PAA), being a rare condition, is considered extremely rare following coronavirus disease 2019 (COVID-19). We present a 58-year-old male who presented with fever, malaise, and dry cough. SARS-CoV-RNA transcription-mediated amplification test was positive for the patient. After 2 days, he developed hemoptysis and back pain, and a CT scan revealed a pulmonary aneurysm, evidence of alveolar hemorrhage, and Necrotizing pneumonia. He was scheduled for pulmonary artery angiography. The angiography confirmed a fusiform aneurysm and partial coiling of the aneurysmal sac, and indoor and backdoor embolization was performed. In the follow-up, a CT scan showed complete thrombosis of the aneurysmal sac, and the patient was free of symptoms. Peripheral PAAs can show a variety of symptoms. They can even be asymptomatic. The infectious pathologies of this condition are less common than the other. COVID-19 is an extremely rare pathology. To the best of our knowledge, this is the first case of necrotizing pneumonia and peripheral PPA in an adult. Moreover, it was followed by COVID-19. A vital takeaway note for physicians is to consider PAAs as a complication when treating COVID-19 patients who don't show signs of improvement or even show signs of exacerbation.

Rare and interesting case of solitary peripheral pulmonary artery aneurysm

Background: We report a rare case of solitary peripheral pulmonary artery aneurysm in a patient who was evaluated for haemoptysis. Incidentally, his total antibodies were positive for Coronavirus 2019 infection. Patient underwent right lower lobectomy uneventfully. Peripheral pulmonary artery aneurysms arising from segmental or intrapulmonary branches are extremely rare. Untreated, the majority end fatally due to sudden rupture and exsanguination. The purpose of this article is to report our rare case and review the pertinent literature. Case Study: A 40-year-old man presented with an episode of haemoptysis. He had a history of intermittent mild grade fever, cough and dyspnea lasting for a month. He had no history of haemoptysis in the past. He had no pre-existing medical conditions or Coronavirus 2019 (COVID-19) infection. His clinical examination was unremarkable. Blood investigations were within normal limits. Reverse transcription polymerase chain reaction test was negative for COVID-19 infection, but his total antibodies test was elevated -117 (biologicalreference range <1.0). 2D Echocardiography was normal. Chest radiography showed a solitary pulmonary lesion in the right lower lung zone [Figure 1a].A computed tomography of the chest plain and contrast confirmed the presence of a 3.7 cm-3.6 cm, well-defined, circumscribed and densely enhancing lesion in apicoposterior segment of right lower lobe. It is seen along the course of descending branch of the right pulmonary artery. Areas of consolidation are also seen in apicoposterior segment. Postcontrast study shows heterogenous enhancement of this lesion suggestive of an aneurysm. The rest of lung parenchyma was normal [Figure 1b and c].The diagnosis of a solitary peripheral pulmonary artery aneurysm (PAA) was considered and right lower lobectomy was performed through posterolateral thoracotomy. Discussion: The estimated incidence of PAA is 1 in 14 000 autopsies, and these lesions can be central aneurysms and peripheral aneurysm. An aneurysm can be true or pseudo aneurysm. In this patient, an aneurysm is a true aneurysm and origin may be idiopathic or post-inflammatory with superadded fungal infection in thrombus, post-COVID-19 infection. Long-term follow up is required to observe the future course Conclusion: True solitary peripheral PAA is an extremely rare entity. A high degree of suspicion is needed for diagnosing PAAs on imaging. Intervention is mandatory as soon as the diagnosis is made, to prevent rupture and death. PAA has been managed most often by lobectomy but occasionally by pulmonary artery repair or endovascular approach.

Pulmonary mucormycosis and vascular complications in the setting of new-onset diabetes mellitus and COVID-19

Introduction: We present the case of a 17-year old female who had an unusual course of pulmonary vascular complications in the setting of new onset type 1 diabetes mellitus and Covid-19 infection. Objectives: Study the course of an unusual case of new onset type 1 diabetes mellitus (T1DM) presenting with diabetic ketoacidosis (DKA) in the setting of COVID-19. Understand that poorly controlled DM is a pro-inflammatory, procoagulant, and immunosuppressive condition and can synergistically act with SARS-Cov-2 in potentiating endothelial damage. Methods: A 17-year old previously healthy female was admitted to our institution for management of DKA in the setting of new onset T1DM. COVID test was positive on admission, however, she was afebrile and without respiratory distress at that time. After 2 days, DKA resolved, however, she presented fever. A chest X-ray showed necrotizing multifocal pneumonia with effusion, which was treated with remdesivir and antibiotics. No oxygen supplementation was needed, but course was complicated by right lower pulmonary artery aneurysm, requiring endovascular occlusion. The device eroded into pulmonary artery and a broncho-arterial fistula developed, needing emergent repair and lobectomy. Results: Histopathology of lung parenchyma showed necrotizing granulomas with hyphae, Mucor was isolated. It was treated with systemic antifungals. Immunologic and rheumatologic workup was negative. Conclusions: While poorly controlled DM is known to be a proinflammatory, pro-coagulant, and immunosuppressive condition, SARS-Cov-2 potentiates endothelial damage, suggesting a possible synergic effect in our patient's unique presentation. While sino-orbital mucormycosis is commonly seen in adults with DM, in children, the infection affects only about 15% with only scarce case reports of pulmonary involvement. Additional studies are needed to understand the synergy of a severe DM presentation with COVID-19 and its potential respiratory and systemic complications. (Figure Presented).

Rare and interesting case of solitary peripheral pulmonary artery aneurysm.

We report a rare case of solitary peripheral pulmonary artery aneurysm in a patient who was evaluated for haemoptysis. Incidentally, his total antibodies were positive for Coronavirus 2019 infection. Patient underwent right lower lobectomy uneventfully. Peripheral pulmonary artery aneurysms arising from segmental or intrapulmonary branches are extremely rare. Untreated, the majority end fatally due to sudden rupture and exsanguination. The purpose of this article is to report our rare case and review the pertinent literature.