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

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.

Mesenchymal Stem Cell Conditioned Medium as Good as Methyl Prednisolone in Decreasing Levels of Interleukin 10 and The Degree of Pulmonary Vasculitis in Lupus Mice

Background: Systemic lupus erythematosus is a chronic autoimmune disease that affects target organs. mesenchymal stem cell conditioned medium has immunosuppressive, anti-inflammatory, and immunoregulatory properties in lupus. Methyl prednisolone is a standard drug for lupus with immunosuppressive and anti-inflammatory properties. This study aims to compare the therapeutic effect of mesenchymal stem cell conditioned medium administration compared to methyl prednisolone on interleukin 10 levels and the degree of pulmonary vasculitis of lupus mice. Methods: The subjects were 24 female mice of Mus musculus Balb/C strain, which were categorized into 4 groups of 8 mice, i.e. the control group receiving 0.5 cc of 0.9% NaCl injection and placebo, the lupus group receiving 0.5 cc of pristane injection and placebo, and the treatment mesenchymal stem cell conditioned medium group receiving 0.5 cc pristane injection and mesenchymal stem cell conditioned medium 0,5 cc, and methylprednisolone group receiving 0,5 cc pristiane injection and methylprednisolone p.o 1,5 mg/kgbodyweight. After 24 days the mice were terminated and kidney and blood samples were taken. Statistical analysis was performed using ANOVA test followed by independent T-test. The p value was considered significant when the p < 0.05. Results: The study showed that there was no difference on the levels of interleukin level10 among mesenchymal stem cell conditioned medium goup and methyl prednisolone group (CM = 5,94 ± 2,49 pg/mL, mp = 5,86+1,73 pg/mL; p = 1) and the degree of pulmonary vasculitis (CM= 1,94 ± 0,25, MP=1,89+ 0,11 pg/ml; p = 0.667). Mesenchymal stem cell conditioned medium as good as methyl prednisolone in decreasing levels of interleukin 10 and the degree of pulmonary vasculitis in lupus mice. Conclusion: Mesenchymal stem cell conditioned medium as good as methyl prednisolone in decreasing levels of interleukin 10 and the degree of pulmonary vasculitis in lupus mice Bangladesh Journal of Medical Science Vol.20(2) 2021 p.426-430