Behçet\'s syndrome (BS) is a rare chronic multisystemic inflammatory disorder of unknown etiopathogenesis. BS is classified as a vasculitis of variable vessel size, which can manifest in both arterial and venous blood vessels. BS commonly presents with mucocutaneous and ocular manifestations. Superficial and deep vein thrombosis is present in 50% of patients, with atypical venous thrombosis affecting the inferior vena cava, superior vena cava, hepatic veins with Budd-Chiari syndrome, portal vein, cerebral sinuses, and right atrium and ventricle. Arterial manifestations include in situ thrombosis, pulmonary artery aneurysms, aneurysms of the abdominal aorta, and aneurysms of visceral and peripheral arteries. This article reports a new case of BS in a 28-year-old female patient who presented with severe dyspnea and hemoptysis. Echocardiography and cardiovascular magnetic resonance imaging led to the diagnosis of endomyocardial fibrosis and a large right ventricular thrombus with pulmonary embolism. Computed tomography angiography revealed multiple pulmonary aneurysms and emboli. Rare findings such as endomyocardial fibrosis and Budd-Chiari syndrome were noted. This case highlights the role of medical imaging modalities in diagnosing rare syndromes such as BS, as demonstrated in the current case.

Hughes-Stovin syndrome (HSS) is a rare vasculitis of unknown etiology. The disease is characterized by pronounced inflammation and damage to the vessel walls, with subsequent widespread vascular thrombosis and the formation of pulmonary artery aneurysms that can lead to fatal hemoptysis. This disorder can be mistaken for other conditions, such as chronic thromboembolic pulmonary disease (CTEPD) without or with pulmonary hypertension at rest (CTEPH).We report the case of a 20-year-old female with HSS, which was misdiagnosed as CTEPH and subsequently treated with anticoagulants, which led to severe hemoptysis and eventually death of the patient. This case highlights the challenges of diagnosing HSS at early stages of the disease.HSS should be considered in young patients with signs of large vessel vasculitis in combination with thrombotic occlusions of pulmonary arteries, with or without aneurysms of the pulmonary arteries, and particularly, if there are no risk factors for thromboembolic disease.

Three young males with Hugh-Stovin\'s syndrome presented with cough, haemoptysis, fever, raised inflammatory markers, and pulmonary artery aneurysm. Only one had recurrent oral ulcers suggestive of Behcet\'s disease, and none were HLA B51 positive. All responded well to immunosuppression but eventually needed either an endovascular procedure or surgery.

Hughes-Stovin Syndrome (HSS) is a rare clinical condition characterized by thrombophlebitis as well as multiple pulmonary and bronchial aneurysms. It commonly presents with coughing, dyspnea, fever, chest pain, and hemoptysis, and its management usually consists of surgical and medical approaches. In this report, we discuss a case of a patient with HSS. A 30-year-old male patient was admitted to the pulmonary medicine ward for hemoptysis. After evaluation with chest CT, bilateral pulmonary embolism and pulmonary aneurysms were observed. Due to a history of aphthous lesions, Behçet\'s disease (BD) was considered the initial diagnosis; however, the patient did not fit the criteria and was later diagnosed with HSS. Intravenous methylprednisolone was initiated, along with a maintenance treatment with cyclophosphamide. Treatment response was observed in the fourth month; however, due to the persistence of hemoptysis, additional cycles of cyclophosphamide were later required, under which the patient\'s condition has been stable. HSS currently lacks clear diagnostic criteria, and further studies are needed to investigate genetic backgrounds, familial transmissions, and treatment alternatives.

We present an adolescent male with a single intracardiac mass and pulmonary emboli, complicated by peripheral venous thrombosis and subsequent development of pulmonary pseudoaneurysms, leading to diagnosis of Hughes-Stovin syndrome. Remission was achieved with cyclophosphamide, corticosteroids, and pseudoaneurysm resection and maintained with infliximab and methotrexate.

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.

Hughes-Stovin syndrome is a rare disease characterized by thrombophlebitis associated with arterial or bronchial aneurysms. Even though it was described first in 1911, it is scarcely reported in the literature. Hughes-Stovin syndrome diagnosis is based on clinical manifestations as well as radiological findings. There are no validated criteria or specific laboratory findings to confirm the diagnosis. Computed tomography pulmonary angiography remains the gold standard for the diagnosis and follow-up of radiological findings, as they were recently described in a critical analysis of the largest cohort in the literature. The aim of this review is to draw attention to this rare but potentially fatal disease and to discuss its therapeutic options.

Hughes-Stovin syndrome is a very rare condition with no defined diagnostic criteria. We present the case of a 26-year-old man who had haemoptysis revealing Hughes-Stovin syndrome. We will consider the aetiology, therapeutic and evolutionary aspects of this disease.
UNASSIGNED: Hughes-Stovin syndrome is a very rare disorder of unknown aetiology which can be fatal.It is considered a variant of Behcet\'s disease.Early diagnosis and treatment improve prognosis.

Mycobacterium chelonae usually causes localized cutaneous infections and abscesses but has the potential to cause disseminated infections, especially in immunocompromised hosts. We report a 27-year-old man with Hughes-Stovin syndrome and catastrophic antiphospholipid syndrome who was on chronic immunosuppressant therapy and developed disseminated M. chelonae infection. To the best of our knowledge, this is the first case report of M. chelonae infection in a patient with Hughes-Stovin syndrome.

BACKGROUND: First described in 1959, Hughes-Stovin syndrome is a very rare disorder combining vascular aneurysms, especially from pulmonary arteries, and thrombosis. The disease affects mostly the young male and is sometime associated with Behçet\' disease.
METHODS: Here, we report the case of a 19-year-old man with hemoptysis and dyspnea revealing recurrent pulmonary embolisms despite efficient anticoagulant therapy. The patient subsequently developed fever and an inflammatory syndrome. Physical examination showed ulcers of the tongue. Angio-CT revealed recent pulmonary embolism, femoral vein thrombosis, and a unique threatening aneurysm of a left pulmonary artery segment. The aneurysm was embolized and simultaneously a vena cava filter was inserted.
CONCLUSIONS: Hughes-Stovin syndrome requires immediate therapeutic decision, with an important risk of the anticoagulation. High dose steroids and in most cases, intensive immunosuppressive therapies are required such as cyclophosphamide.