255th ENMC workshop: Muscle imaging in idiopathic inflammatory myopathies. 15th January, 16th January and 22nd January 2021 - virtual meeting and hybrid meeting on 9th and 19th September 2022 in Hoofddorp, The Netherlands.

The 255th ENMC workshop on Muscle Imaging in Idiopathic Inflammatory myopathies (IIM) aimed at defining recommendations concerning the applicability of muscle imaging in IIM. The workshop comprised of clinicians, researchers and people living with myositis. We aimed to achieve consensus on the following topics: a standardized protocol for the evaluation of muscle images in various types of IIMs; the exact parameters, anatomical localizations and magnetic resonance imaging (MRI) techniques; ultrasound as assessment tool in IIM; assessment methods; the pattern of muscle involvement in IIM subtypes; the application of MRI as biomarker in follow-up studies and clinical trials, and the place of MRI in the evaluation of swallowing difficulty and cardiac manifestations.

The effect of a 24-week training focused on activities of daily living, muscle strengthening, and stability in idiopathic inflammatory myopathies: a monocentric controlled study with follow-up.

Background: The structural and functional changes of the skeletal muscles in idiopathic inflammatory myopathies (IIM) caused by inflammation and immune changes can be severely disabling. The objective of this study was to assess the effect of a 24-week program combining a supervised training of activities of daily living (ADL), resistance, and stability with home exercise for improving muscle function, compared to a daily home-based exercise representing the regular outpatient care.

Methods: Fifty-seven patients with IIM were consecutively and non-selectively enrolled in an intervention (IG, n = 30) or control (CG, n = 27) group.

Clusterin is upregulated in serum and muscle tissue in idiopathic inflammatory myopathies and associates with clinical disease activity and cytokine profile.

Objectives: The aim of this cross-sectional study was to explore the circulating and skeletal muscle expression of clusterin (CLU) in inflammatory myopathies (IIM) and its potential implication in pathogenetic mechanisms of the disease.

Methods: A total of 85 IIM patients and 86 healthy controls (HC) were recruited. In addition, 20 IIM patients and 21 HC underwent a muscle biopsy.

Altered dynamics of lipid metabolism in muscle cells from patients with idiopathic inflammatory myopathy is ameliorated by 6 months of training.

Key Points: Regular exercise improves muscle functional capacity and clinical state of patients with idiopathic inflammatory myopathy (IIM). In our study, we used an in vitro model of human primary muscle cell cultures, derived from IIM patients before and after a 6-month intensive supervised training intervention to assess the impact of disease and exercise on lipid metabolism dynamics. We provide evidence that muscle cells from IIM patients display altered dynamics of lipid metabolism and impaired adaptive response to saturated fatty acid load compared to healthy controls.

How Imaging Can Assist with Diagnosis and Monitoring of Disease in Myositis.

Purpose Of Review: To summarize recent findings and progression in the field of imaging of inflammatory myopathies. The most commonly used method is magnetic resonance imaging, and the article focuses on this technique, but covers also several other less frequently used or emerging methods.

Recent Findings: A relatively good agreement exists regarding some technical parameters, area of investigation, and assessment of inflammatory activity and chronic damage using magnetic resonance imaging.

Alterations in activin A-myostatin-follistatin system associate with disease activity in inflammatory myopathies.

Objectives: The aim of this study was to investigate the systemic and skeletal muscle levels of atrophy-associated myokines in patients with idiopathic inflammatory myopathies (IIM) and their association with clinical characteristics of myositis.

Methods: A total of 94 IIM patients and 162 healthy controls were recruited. Of those, 20 IIM patients and 28 healthy controls underwent a muscle biopsy.

The role of imaging in evaluating patients with idiopathic inflammatory myopathies.

Objectives: To describe imaging modalities for diagnosing and monitoring of patients with idiopathic inflammatory myopathies.

Methods: A detailed literature search summarising recent data documenting the contribution of different imaging techniques to current management of idiopathic inflammatory myopathies was performed.

Results: An overview of methods most frequently used for evaluation of inflammatory myopathies and the description of their role in the diagnostic and monitoring process is presented.

MRI scoring methods used in evaluation of muscle involvement in patients with idiopathic inflammatory myopathies.

Purpose Of Review: MRI is a promising imaging method commonly used to assess muscle involvement in patients with idiopathic inflammatory myopathies (IIM). MRI enables evaluation of both activity and damage and is therefore an ideal noninvasive diagnostic and monitoring tool. Despite its widespread use, there is no universally accepted method for scoring and reporting of MRI findings.

Application of the DETECT algorithm for detection of risk of pulmonary arterial hypertension in systemic sclerosis: data from a Czech tertiary centre.

Objective: The early, simple and reliable detection of pulmonary arterial hypertension (PAH) in SSc (DETECT) study described a new algorithm for early detection of PAH in patients with SSc. The aim of this retrospective, single-centre, cross-sectional study was to apply a modified DETECT calculator in patients with SSc in the East Bohemian region, Czech Republic, to assess the risk of PAH and to compare these results with PAH screening based on the European Society of Cardiology/European Respiratory Society (ESC/ERS) 2009 guidelines.

Methods: Sixty patients were recruited with a diagnosis of SSc (according to ACR criteria), aged 27-78 years.

Embolization of uterine fibroids from the point of view of the gynecologist: pros and cons.

Uterine artery embolization (UAE) is a minimally invasive procedure with large symptomatic potential in treatment of women with uterine leiomyomas. Due to specificities of this method and possible complications the appropriate indication is crucial. Patient' symptoms, age, plans for pregnancy, and surgical and reproductive history play a major role in decision-making regarding appropriate subjects for UAE.

Hysteroscopic management of displaced levonorgestrel-releasing intrauterine system.

Aim: This study was designed to evaluate feasibility and effectiveness of hysteroscopic intervention in the management of symptoms related to the displaced levonorgestrel-releasing intrauterine system (LNG-IUS).

Material And Methods: One hundred and thirteen patients with displaced LNG-IUS presenting with irregular uterine bleeding, pelvic pain or asymptomatic displacement were recruited for hysteroscopic examination. Displaced LNG-IUS was relocated by hysteroscopic intervention and the effect on symptoms and LNG-IUS position was followed.

[Laparoscopic uterus sparing treatment of uterine fibroids].

Objective: To report up-to date knowledge on laparoscopic, uterine sparing treatment of uterine fibroids.

Study Design: Review.

Setting: Uterine Fibroids Center, Department of Obstetrics and Gynecology, Charles University, Prague.

[Laparoscopic lymph-node dissection in gynecological surgery].

Objective: To analyze our experience with transperitoneal laparoscopic dissection of lymphatic nodes in women with gynecologic malignancies.

Study Design: Retrospective clinical study.

Setting: Center of gynecologic endoscopy and minimally invasive surgery; Department of Obstetrics and Gynecology, General Faculty Hospital and 1st Medical Faculty in Prague.

[Office hysteroscopy - management and results].

Objective: Sum up the knowledge about office hysteroscopy.

Type Of Study: Review of literature and our own experience.

Setting: Department of Gynaecology and Obstetrics, First Faculty of Medicine, Charles University Prague and General Teaching Hospital Prague.

Effect of a selective progesterone receptor modulator on induction of apoptosis in uterine fibroids in vivo.

Aim. To determine if hormonal treatment induces apoptosis in uterine fibroids. Methods.

Fumarate hydratase gene mutation in two young patients with sporadic uterine fibroids.

Fumarate hydratase (FH) is a key enzyme of the Krebs cycle. Germline mutations in the FH gene encoding fumarate hydratase cause autosomal dominant syndromes multiple cutaneous and uterine leiomyomata and hereditary leiomyomatosis and renal cell cancer (HLRCC). Few data have been published on the role of FH gene mutation in development of uterine fibroids outside the context of multiple cutaneous and uterine leiomyomata /HLRCC.

[Genetic factors in etiology of uterine fibroids].

Uterine fibroids are the most common pelvic tumors in women of reproductive age. The cause of development of uterine fibroids is still unknown, however recent cytogenetic and genetic studies led to advancement in understanding of etiology of these tumors. In accordance with the latest findings up to 40% of uterine fibroids bear some chromosomal abnormalities.

Uterine artery embolization versus laparoscopic uterine artery occlusion: the outcomes of a prospective, nonrandomized clinical trial.

Purpose: To compare outcomes of two different types of occlusive therapy of uterine fibroids.

Methods: Women with fibroid(s) unsuitable for laparoscopic myomectomy (LM) were treated with uterine artery embolization (UAE) or laparoscopic uterine artery occlusion (LUAO).

Results: Before the procedure, patients treated with UAE (n = 100) had a dominant fibroid greater in size (68 vs.

Hysteroscopy after uterine fibroid embolization: evaluation of intrauterine findings in 127 patients.

Aim: Several atypical hysteroscopy findings have been described in association with uterine artery embolization (UAE). The purpose of this study was to evaluate the types and frequency of these findings in the largest published series of patients.

Material And Methods: Premenopausal patients after bilateral UAE for symptomatic intramural fibroid underwent subsequent hysteroscopic examination 3-9 months following UAE.

Reproduction after myomectomy: comparison of patients with and without second-look laparoscopy.

Myomectomy is associated with a high risk of de-novo adhesion formation that may decrease fertility. The purpose of this study was to compare the reproductive outcome of patients after laparoscopic or open myomectomy who underwent second-look (SL) hysteroscopy and laparoscopy including adhesiolysis with patients with no SL intervention. A total of 170 patients underwent open or laparoscopic myomectomy at one centre.

Comparative outcomes of hysteroscopic examinations performed after uterine artery embolization or laparoscopic uterine artery occlusion to treat leiomyomas.

Hysteroscopic examination of the uterine cavity revealed that patients previously treated for intramural myoma(s) by uterine artery embolization had a significantly higher incidence of abnormal findings compared with patients treated by laparoscopic occlusion of uterine arteries (59.5% vs. 2.

"See and treat" hysteroscopy after missed abortion.

A prospective study was conducted on the incidence of intrauterine pathology after missed abortion diagnosed and treated by hysteroscopy. A hysteroscopy was performed in 100 women four to 12 weeks after a dilatation and curettage for missed abortions. Uterine malformations were found in 12 patients, intrauterine adhesions in seven and submucous myoma in two cases.

[Methods using ischemia in uterine fibroids treatment].

Objective: To compare principle, advantages, disadvantages, and risks of conservative (i.e. uterus saving) methods of treatment of uterine fibroids, which are focused on elimination or reduction of their perfusion.

[Sexual functions after laparoscopically assisted vaginal hysterectomy (LAVH) and total laparoscopic hysterectomy (TLH) in preoperatively asymptomatic women].

Objective: To find the consequences of laparoscopically assisted vaginal hysterectomy (LAVH) and total laparoscopic hysterectomy (TLH) for sexual functions in preoperatively asymptomatic women.

Design: Prospective study.

Setting: Department of Obstetrics and Gynecology, 1st Faculty of Medicine, Charles University and General Teaching Hospital in Prague.

[Spontaneous symphysis pubis rupture followed by intraabdominal bleeding during the vaginal delivery].

Symphysis pubis rupture is rare but life-threatening complication of vaginal delivery. Prompt diagnosis and therapy of this complication is crucial. We present a case report of a patient with a symphysis pubis rupture followed by severe intraabdominal bleeding.