[Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)]. / Eosinophile Granulomatose mit Polyangiitis (Churg-Strauss-Syndrom).

This article presents the case of a patient with dyspnea, a history of poorly controlled asthma, sinonasal polyposis, blood eosinophilia and transient pulmonary infiltrates. The autoantibodies antinuclear antibodies, cytoplasmic anti-neutrophil cytoplasmic antibodies (c-ANCA) and perinuclear anti-neutrophil cytoplasmic antibodies (p-ANCA) were negative. These symptoms are typical for eosinophilic granulomatosis with polyangiitis (EGPA, alternatively known as Churg-Strauss syndrome). The delay between onset and diagnosis in this case was several years. Although EGPA belongs to the spectrum of ANCA-associated vasculitis, less than 50% of EGPA patients are ANCA positive. Cardiac involvement (such as endomyocardial infiltration, arrhythmia and pericarditis) is the major cause of early death and a poor prognosis. In therapeutic regimens glucocorticoids and/or another immunosuppressant (e. g. cyclophosphamide, methotrexate or azathioprine) are used. The so-called five-factor score is a useful tool for assessment of prognosis.

Measurements After Image Post-processing Are More Precise in the Morphometric Assessment of Thoracic Aortic Aneurysms: An Intermodal and Intra-observer Evaluation.

OBJECTIVES: Precise pre-procedural anatomical analysis of aneurysmal anatomy is essential for successful thoracic endovascular aortic repair (TEVAR). Since surgeons and radiologists have to perform multiple measurements in the same patient, high intra-observer reliability of any imaging method is mandatory. Commercially available three dimensional (3D) post-processing techniques are expected to be superior to conventional two dimensional multiplanar reconstructions (MPRs) derived from computed tomography angiograms (CTAs). However, few data exist to support this view. This study aims to evaluate the intermodal and intra-observer differences using 3D software (3surgery) in descending thoracic aortic aneurysms (dTAAs). METHODS: Pre-operative CTAs (performed between 2004 and 2010) of 30 dTAAs (mean maximum diameter 61.4 ± 13 mm) were assessed by three independent investigators with different experience in the measurement of aortic pathologies. Intra-observer reliability and intermodal differences (3D vs. 2D) were investigated using pre-specified measurement points (distances of total length, maximum diameter, proximal and distal landing zones). Statistical analyses were performed using the Bland-Altman method and a mixed regression model. RESULTS: Intermodal comparison showed that 2D measurements significantly underestimate the measured distances (maximum diameter 3.7 mm [95% CI -5.3 to -2.1] and landing zone maximum 1.4 mm [95% CI -2.0 to -0.2] shorter with 2D, p < .05). In almost all 3D measurements, all investigators showed lower variability comparing the intra-observer differences, most notably in the measuring point total length (reduction of the SD up to 7.9 mm). CONCLUSIONS: These data show that both techniques led to significant measurement disparity. This occurs especially at the point of indication (maximum diameter) and the total length of the aneurysm (important for correct stent graft selection). But overall the variability is reduced with the 3D technique, which also tends to measure greater distances. The use of post-processing software therefore leads to more precise device selection for TEVAR in TAA.

[Clinically unclear painful swelling of the upper arm]. / Klinisch unklare schmerzhafte Schwellung des Oberarms.

In contrast to the intramedullary (80%) and intracortical (14%) subtypes, the subperiostal subtype of aneurysmatic bone cysts (sABC) is relatively rare (6%). Females are affected more frequently than males, whereby the diaphysis is predominantly affected and less frequently the metaphysis of long bones as well as the vertebral column. Especially in conventional radiography sABCs can mimic aggressive lesions. Cross-sectional imaging can potentially reduce the wide range of differential diagnoses. Due to typical imaging features magnetic resonance imaging (MRI) is the most valuable modality to reduce the range of possible differential diagnoses. MRI usually presents a multicystic appearance with a hypointense rim of the lesion, contrast-enhancing cyst walls, fluid levels and edema-like changes in the adjacent tissue. In sABCs with solid components the diagnosis cannot be made with confidence and the suspicion must be confirmed by biopsy. The therapy of primary lesions consists of curettage or the complete excision of the sABC and the defects are subsequently filled with spongiosa or bone cement depending on the size of the lesion.