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.

Objective. To compare findings as observed on enhanced magnetic resonance imaging (MRI) of the knee joints, in oligoarticular-undifferentiated arthritis (UA) in those with established rheumatoid arthritis (RA) and spondyloarthropathy (SpA).

Hughes–Stovin syndrome (HSS), characterized by the combination of multiple pulmonary artery aneurysms and deep vein thrombosis, is a rare and an under-recognized clinical entity with less than 40 published cases in English medical literature. Vascular venous thrombotic events, as occurring in the course of Behçet’s disease (BD), are also described in HSS, e. g., vena cava, intra-cardiac, jugular vein, iliac vein, femoral vein, and dural sinus thrombosis. We describe a 35-year-old man with HSS showing classical features of the syndrome in the form of recurrent thrombophlebitis of the lower limb veins, pulmonary arterial aneurysms, and left lower limb ischemia with extensive arterial tree involvement. The patient presented with critical arterial ischemia in the left lower limb together with aortic and left common iliac artery thrombosis, occlusion of the left superficial femoral artery, and occlusion of both lower limb arteries. Urgent vascular surgeries were carried out for limb salvage. Shortly after, the patient started on pulse corticosteroid/cyclophosphamide therapy, followed by monthly cyclophosphamide for 1 year, with much improvement. We discuss arterial involvement in HSS and similarities of HSS and BD regarding thrombotic events. We summarize the current management options of HSS. Das Hughes-Stovin-Syndrom (HSS) ist durch die Kombination von multiplen Pulmonalarterienaneurysmen und tiefer Venenthrombose charakterisiert; es stellt eine seltene sowie unterdiagnostizierte klinische Entität mit weniger als 40 publizierten Fällen in der englischsprachigen medizinischen Literatur dar. Vaskuläre Ereignisse im Sinne von Venenthrombosen, wie sie im Verlauf des M. Behçet auftreten, wurden auch bei HSS beschrieben, z. B. Vena-cava-, intrakardiale, Jugularvenen‑, Iliakalvenen‑, Femoralvenen- und Duralsinusthrombosen. Hier wird der Fall eines 35-jährigen Mannes mit HSS beschrieben, der klassische Zeichen des Syndroms in Form rezidivierender Thrombophlebitiden der Beinvenen, Pulmonalarterienaneurysmen und eine Ischämie des linken Beins mit extensiver Beteiligung der arteriellen Äste aufwies. Bei dem Patienten zeigten sich eine kritische arterielle Ischämie im linken Bein in Kombination mit einer Thrombose der Aorta sowie der linken A. iliaca communis, Okklusion der linken A. femoralis superficialis und Okklusion beider Beinarterien. Zur Rettung der linken unteren Extremität wurden notfallmäßig Gefäßoperationen durchgeführt. Kurz darauf begann für den Patienten eine Kortikosteroid-Cyclophosphamid-Stoßtherapie mit erheblicher Besserung unter anschließender monatlicher Gabe von Cyclophosphamid über ein Jahr. In der vorliegenden Arbeit werden die arterielle Beteiligung bei HSS sowie Ähnlichkeiten von HSS und M. Behçet in Hinblick auf thrombotische Ereignisse erörtert. Zusammenfassend werden die aktuellen Optionen zur Therapie des HSS dargestellt.

The aim was to explore possible correlations of antibodies to extractable nuclear antigens (ENA) with clinical manifestations and disease activity indices in systemic lupus erythematosus (SLE) patients. A total of 70 consecutive SLE patients (64 females) were included. Disease activity was assessed by SLE activity index (SLEDAI), and British Isles Lupus Assessment Group (BILAG). Anti-Ro/SSA correlated positively with, headache (r=0.24, p=0.04), blurring of vision (r=0.25, p=0.03) and SLEDAI (r=0.25, p=0.04) and negatively with C3 (r=–0.35, p=0.003). Anti-Ro/SSA correlated with anti La/SSB antibodies (r=0.69, p<0.001), but not with anti-DNA, anti-RNP and anti-Sm antibodies. Anti-La/SSB antibodies correlated with headache (r=0.26, p=0.03), SLEDAI (r=0.25, p=0.03) and negatively with C3 (r=–0.34, p=0.004). Anti-La/SSB did not correlate with anti-RNP or anti-Sm antibodies. Anti-Sm antibodies correlated with disease duration (r=0.34, p=0.003), 24 hours urinary proteins (r=0.31, p=0.008), SLEDAI (r=0.31, p=0.009), BILAG renal score (r=0.29, p=0.02) and negatively with age at onset (r=–0.27, p=0.02), WBCs (r=–0.29, p=0.014) and C4 (r=–0.25, p=0.049). In multivariate analyses, anti-Ro/SSA antibodies remained associated with headache, blurring of vision and C3 and anti-La/SSB antibodies remained associated with C3 and with headache. Anti-Sm antibodies were independently associated with disease duration and total SLEDAI scores, while anti-RNP antibodies remained significantly associated with BILAG mucocutaneous scores only. Antibodies to ENAs are associated with clinical aspects of SLE and may play a role in the assessment of disease activity. Insight into these ENAs may lead to new approaches to diagnostic testing, accurate evaluation of disease activity and lead to target approach for SLE.