Case presentation We report the case of an 18 years old, greek male patient with Hughes-Stovin syndrome, who initially presented with deep vein thrombosis. [ncbi.nlm.nih.gov]

Case presentation An 18 years old, Greek male patient presented with edema of his left leg. [olox.pro]

• Abnormal Pulse

  Cardiovascular and respiratory systems did not show abnormalities. Pulses were palpable and symmetrical in the lower and upper limbs; swelling of the left calf; negative pathergy. The joints did not show signs of inflammation. [scielo.br]

• Leg Weakness

  She then developed bilateral hand and leg weakness, diffuse hyper-reflexia, and a thoracic sensory level to pinprick on the right with reduced vibration sense in the left leg. She admitted to medication non-adherence. [cmsc.confex.com]

• Malar Rash

  Clinical assessment included any symptoms or signs suggestive of systemic lupus or antiphospholipid syndrome like malar rash, alopecia, livedo reticularis, Raynaud’s phenomenon, and any skin lesions suggestive of leukocytoclastic vasculitis. [link.springer.com]

• Focal Neurological Deficit

  […] features (e.g. headache, seizures, focal neurological deficits) 7. [radiopaedia.org]

A similar extensive workup was performed to investigate autoimmune causes. [cureus.com]

An extensive rheumatological, infectious and hematological workup was negative. He developed acute right heart failure requiring surgical removal of the right atrial mass, and tricuspid valve replacement (TVR). [jacc.org]

Her coagulation profile was normal and workup for systemic lupus erythematosus and antiphospholipid antibody syndrome was negative. Her chest X-ray showed homogenous radiopaque lesion in the right lower lobe [Figure 1]a. [indjvascsurg.org]

Fig. 1 Flowchart suggested initial workup/treatment of patients with HSS. [link.springer.com]

Having a standard approach to the evaluation of a cavitary lung lesion can facilitate an expedited workup. [wavenutrition.in]

• Foam Cell

  The elastic and muscular fibers become annihilated whereas tunica media is completely filled with lymphocytes, plasma cells and foam cells in the affected vessels. [g3m.me]

The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment. [orpha.net]

This increased morbidity and the failure of both surgical and medical treatments led to the patient requiring a lung transplant. [cureus.com]

Because she didin’t carry the criteria for Behcet’s disease, she was diagnosed as Hughes-Stovin Syndrome (HSS) and steroid treatment was started. The patient who had a response to the treatment, stay in remission for a long time. [turkthoracj.org]

Case 3 presented very unwell, but he was extubated 24 hrs after starting treatment and Infliximab was used as an induction treatment. [ard.bmj.com]

Prognosis of HSS Venular aneurysms have a better prognosis than arterial aneurysms. [starhealth.in]

Overall, patients with HSS have a poor prognosis and aneurysmal rupture is the leading cause of death. However, early diagnosis and timely intervention is crucial in improving the prognosis. [ncbi.nlm.nih.gov]

The prognosis was poor in patients with HSS, and aneurysmal rupture was the main cause of death. [turkthoracj.org]

Prognosis As most patients with HSS are diagnosed late in the disease course, the syndrome is associated with significant mortality due to massive hemoptysis resulting from PAA rupture or systemic bronchial artery hypertrophy secondary to ischemia related [orpha.net]

The Korean Journal of Thoracic and Cardiovascular Surgery Feb 13, 2018 Abstract Hughes-Stovin syndrome is a rare disorder of unknown etiology. [meta.org]

Etiology The etiology of HSS is unknown; however, it is assumed that HSS is a form of vasculitis following a similar mechanism of pathogenesis to that thought to be involved in BD. [orpha.net]

There is a need to clearly elucidate the genetic, etiologic and pathologic basis for HSS in the future. [ncbi.nlm.nih.gov]

Summary Epidemiology Prevalence is unknown but fewer than 30 cases have been reported in the literature since its first description in 1959 by Hughes and Stovin. [orpha.net]

Epidemiology, genetics, pathogenesis, organ system involvement, differential diagnosis, novel treatments, surgical management, and prognosis are just some of the topics addressed. [books.google.de]

Pulmonary Complications of Cystic Fibrosis Patrick A Flume MD Introduction Pneumothorax Epidemiology Pathophysiology Diagnosis Management Massive Hemoptysis Epidemiology Pathogenesis Diagnosis Management Respiratory Failure Pathogenesis Management Summary [wavenutrition.in]

A similar pathophysiology seems to underlay the two syndromes and BS may represent a more full phenotype of this pathophysiology with multisystem involvement. Conclusion: HSS may be a forme fruste of BS. [cmsc.confex.com]

Indeed, pulmonary involvement can be indistinguishable between these two conditions of unknown pathophysiology. [scinapse.io]

Thus without the intimate knowledge of this disease pathophysiology we cannot apply a scholastic template to the clinical presentation of SHS patients. [dokumenty.site]

Conventional pulmonary angiography could not be performed, because thrombus in both the inferior and superior vena cavae prevented catheter passage. [ncbi.nlm.nih.gov]

In our case, LMWH resulted useful in preventing the pulmonary embolism, despite the use of anticoagulants is still debated in HSS. [ard.bmj.com]

Conventional pulmonary M- giography could not be per- formed, because thrombus in both the inferior and superior vena cavae prevented cathe- ter passage. [documents.tips]

By that logic, long term anticoagulation may have the potential to prevent further aneurysm formation by targeting this particular mechanism [ 62 ]. [g3m.me]

Use of antiplatelet and anticoagulants does not prevent vascular thromboses and is therefore generally not recommended due to risk of fatal massive hemorrhage 7. [radiopaedia.org]