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.
abstract:Objectives: Asthma control is often difficult to measure. The aim of this study was to compare physicians' personal clinical assessments of asthma control with the Global Initiative for Asthma (GINA) scoring. Methods: Physicians in the adult pulmonary clinics of a tertiary hospital in Oman first documented their subjective judgment of asthma control on 157 consecutive patients. Immediately after that and in the same proforma, they selected the individual components from the GINA asthma control table as applicable to each patient. Results: The same classification of asthma control was achieved by physicians' clinical judgment and GINA classification in 106 cases (67.5%). In the other 32.5% (n = 51), the degree of control by clinical judgment was one level higher than the GINA classification. The agreement was higher for the pulmonologists (72%) as compared to non-pulmonologists (47%; P = 0.009). Physicians classified 76 patients (48.4%) as well-controlled by clinical judgment compared to 48 (30.6%) using GINA criteria (P <0.001). Conversely, they classified 34 patients (21.7%) as uncontrolled as compared to 57 (36.3%) by GINA criteria (P <0.001). In the 28 patients who were clinically judged as well-controlled but, by GINA criteria, were only partially controlled, low peak expiratory flow rate (PEFR) (46.7%) and limitation of activity (21.4%) were the most frequent parameters for downgrading the level of control. Conclusion: Using clinical judgment, physicians overestimated the level of asthma control and underestimated the uncontrolled disease. Since management decisions are based on the perceived level of control, this could potentially lead to under-treatment and therefore sub-optimal asthma control.
Hughes-Stovin syndrome is a very rare clinical entity characterized by pulmonary artery aneurysms and deep vein thrombosis (DVT). Here we report the case of a 53-year-old man, admitted to Sultan Qaboos University Hospital, Muscat, Oman, with bilateral pulmonary artery aneurysms and lower-limb DVT who developed massive hemoptysis. He was managed successfully with high-dose steroids in combination with cyclophosphamide.
Pulmonary complications in leptospirosis, though common, are often unrecognized in a non-endemic area. We report here a patient with leptospirosis and severe pulmonary involvement who was treated with meropenem (1 g every 8 hours), moxifloxacin (400 mg once daily), and high doses of corticosteroids. Systemic steroids were continued for 3 months because of persistent pulmonary lesions.
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