Israel Medical Association Journal

Background: In 2006, the Israeli Ministry of Health distributed guidelines for improving cardiopulmonary resuscitation (CPR) knowledge among hospital staff. The impact of these guidelines on survival after in-hospital cardiac arrest (IHCA) is unclear.

Objectives: To compare rates of incidence and survival to discharge after IHCA, preceding and subsequent to issuance of the guidelines: 1995–2005 and 2006–2015.

Methods: Data were retrieved from the computerized records of patients who had an IHCA and underwent CPR. In addition, we retrieved data available from the hospital's resuscitation committee that included number, type, methods of training in CPR refresher courses, type and number of audits carried out during the past 10 years, and type of CPR quality assessments.

Results: From 1995 to 2015, IHCA incidence increased from 0.7 to 1.7 per 1000 admissions (P < 0.001), while survival rate did not increase (P = 0.37). Survival for shockable rhythms increased from 15.4 to 30.2% (P = 0.05) between the two time periods. The ratio of non-shockable to shockable rhythms increased from 2.4 to 4.6 (P = 0.01) between the two time periods.

Conclusions: Overall IHCA survival did not improve following the issuance of guidelines requiring CPR refresher courses, although survival improved for patients with initial shockable dysrhythmia. A decrease of events with initial shockable dysrhythmia, an increase with acute renal failure, and a decrease occurring in intensive care units contributed to understanding the findings. We found that CPR refresher courses were helpful, although an objective measure of their effectiveness is lacking.

Medical practice is a form of art, with each complex detail essential to the welfare of the individuals in the care of the physician. Art and medicine have shared a close relationship in a variety of ways for centuries, as demonstrated by anatomical drawings and textbooks from the 16th century. Leonardo da Vinci, driven by his fascination with the details of the human body and how it functioned, succeeded in creating an anatomical model of the cerebral ventricles and the aorta using molten wax and a glass structure, respectively (Heart and Its Blood Vessels). By using water that contained grass seeds, this experiment enabled him to study blood flow. da Vinci’s engrossment with the complexity of the human body is reflected in many of his drawings, including the famous depiction of the human physique in his drawing of the Vitruvian Man. This drawing, which defines the ideal proportions of the human body and their correlation with geometry, is an example of how artistic and scientific objectives integrate with each other.

A 47 year old man presented with a combination of dry mouth and lightheadedness while standing. His medical background was unremarkable except for cigarette smoking and hyperlipidemia. Sjögren’s syndrome was ruled out, and he was referred for evaluation of orthostatic hypotension, which by then included syncopal episodes and injuries. Additional symptoms included dry eyes, constipation, reduced sweating, and erectile dysfunction. After excluding medications and structural cardiac abnormalities as causes of orthostatic hypotension, a clinical autonomic evaluation was performed. The pattern of beat-to-beat blood pressure associated with performance of the Valsalva maneuver, and a low plasma norepinephrine level that did not increase in response to standing, established that the orthostatic hypotension was neurogenic. Treatment with an alpha-adrenoceptor agonist and fludrocortisone yielded partial improvement. After systemic diseases involving autonomic failure were excluded, cardiac sympathetic neuroimaging was performed by ¹²³I-metaliodobenzylguanidine (MIBG) scanning. The normal uptake seen in the heart indicated intact post ganglionic sympathetic innervation. There were no signs of central neurodegeneration or peripheral neuropathy. Because of symptoms and signs of both parasympathetic and sympathetic failure without denervation, an autonomic ganglionopathy was considered. A high titer of antibody to the neuronal nicotinic receptor, which mediates ganglionic neurotransmission, was obtained. The diagnosis of autoimmune autonomic ganglionopathy (AAG) was made, and the management strategy shifted to first lowering the antibody burden by plasma exchanges and then instituting chronic anti-autoimmune treatment with rituximab and a low dose of cortiosteroid. The patient showed remarkable improvement.

Background: Magnetic resonance imaging (MRI) is the most sensitive imaging modality for the detection of sacroiliitis. Diagnosing sacroiliitis on MRI is not always straightforward and can be challenging in some cases.

Objectives: To evaluate the prevalence of alternative diagnoses suggested by MRI and characterize the MR appearance of the most common ones.

Methods: Consecutive MRI examinations of the sacroiliac joints (SIJ) performed between 2005 and 2012 were retrospectively evaluated for the presence of structural and active sacroiliitis findings according to the Assessment of SpondyloArthritis International Society guidelines. Alternative diagnoses, including degenerative changes, diffuse idiopathic skeletal hyperostosis (DISH), Osteitis condensans ilii (OCI), septic sacroiliitis/discitis, stress reaction as well as anatomic variants, were registered

Results: We evaluated 281 MRI examinations, 116 males, 165 females, average age 44 ± 15 years. Sacroiliitis was found in 71 examinations (25%) and alternative diagnoses were suggested in 87 (31%) (OCI 8.9%, anatomic variants 5.3%, septic sacroiliitis 5.3%, degenerative findings 4.3%, diffuse idiopathic skeletal hyperostosis [DISH] 1.5%, stress reaction 0.7%, tumor 0.3%). A normal examination was found in the remaining 123 examinations. Patients with alternative diagnoses were older than those with sacroiliitis (62 vs. 47 years of age, respectively, P > 0.05). Alternative pathologies in the SIJ were significantly more common in females (66) than males (21), P < 0.05.

Conclusions: A substantial proportion of patients with suspected sacroiliitis had normal SIJ while the rest were more commonly diagnosed with other pathologies. A referral by an experienced rheumatologist may improve the sensitivity and specificity of this important examination.

Enthesitis is a term that refers to inflammation at tendon, ligament, or joint capsule insertions. The entheses are increasinlgly considered to be the primary site of joint inflammation in the spondyloarthropathies including psoriatic arthritis (PsA). Great advances have occurred in the understanding of enthesopathy, which has resulted in a better understanding of the etiopathogenesis of PsA. Enthesitis is difficult to assess on both clinical examination and on imaging because of the overlap in features between mechanical, degenerative, and inflammatory pathologies. Ultrasonography frequently detects entheseal abnormalities in patients with psoriasis, despite the absence of clinical symptoms of arthropathy and the longitudinal value of such lesions for PsA prediction remains unknown. The role of magnetic resonance imaging (MRI) in the assessment and monitoring of enthesitis is not fully agreed on but it is clearly superior for the assessment of spinal polyenthesitis and for diffuse peri-enthseal osteitis that can occur anywhere in the skeleton. Nuclear medicine, including conventional positron-emission tomography (PET) and high-resolution PET scan (hrPET), is more of a research tool for enthesitis and can, for example, help distinguish between PsA and osteoarthritis. Entheseal abnormalities are common in osteoarthritis, which creates diagnostic difficulty from PsA. Entheseal changes, especially on imaging, may also occur in rheumatoid arthritis (RA) and likely reflect the extension of the inflammatory process from the adjacent synovium. The nail is anatomically anchored to the skeleton via a mini-enthesis network. An association between ultrasonography determined distal interphalageal joint (DIP) extensor tendon enthesopathy and clinical nail disease was found, which highlights the pivotal role of the enthesis in this PsA risk factor. This review summarizes the relevant insights and implication of imaging for enthesitis, primarily in PsA but also in other arthropathies.

Axial spondyloarthritis (axSpA) covers the stage of non-radiographic axial spondyloarthritis (nr-axSpA) and classic ankylosing spondylitis. The pathognomonic findings of axSpA are mainly inflammatory and osteoproliferative changes in the sacroiliac joints (SIJ) and the spine. Various imaging techniques are being used in daily practice for assessment of disease-specific changes, such as periarticular bone marrow edema, erosions, sclerosis, fat metaplasia and ankylosis in the SIJ or spondylitis, spondylodiscitis, facet joint involvement, or syndesmophytes in the spine of patients with axSpA. Conventional radiographs are still considered the gold standard for assessment of structural changes, while the method of for detection of inflammatory changes is magnetic resonance imaging (MRI).

A result for an MRI in the SIJ is considered positive for axSpA when more than one lesion is present on one MRI slice, If there is one lesion only, this should be present on at least two consecutive slices. For the spine, inflammatory lesions should preferably be located in the corner of the vertebral bodies, while occurrence of spondylitis in three or more vertebral corners is considered highly suggestive of axSpA.

This review gives a detailed overview about the benefits and limitations of all available imaging techniques in patients with axSpA, explains the usage of imaging techniques in the context of diagnosis and differential diagnosis of the disease, and reports on the potential future trends in the area of imaging of the axial skeleton in patients who are suspicious for this diagnosis.

Over the last 15 years ultrasound has gained importance for the clinical management of patients with inflammatory rheumatic diseases, especially rheumatoid arthritis. This review summarizes the recent developments and achievements in the use of ultrasound in RA, as well as the unmet needs.

Background: Strategies aimed at expanding the organ donor pool have been sought, which has resulted in renewed interest in donation after cardio-circulatory death (DCCD), also known as non-heart beating donors (NHBDs).

Objectives: To describe the derivation and implementation of a protocol for DCCD in Israel and report on the results with the first six cases. 

Methods: After receiving approval from an extraordinary ethics committee, Ministry of Health, the steering committee of the National Transplant Center defined and reached consensus on the unique challenges presented by a DCCD program. These protocol included medical aspects (construction of a clinical pathway), social and ethical aspects (presentation of the protocol at a public gathering(, legal/ethical aspects (consent for organ preservation procedures being either implied if the donor had signed an organ donor card or received directly from a surrogate decision maker), and logistical aspects (pilot study confined to kidney retrieval and to four medical centers). Data regarding organ donors and recipients were recorded.

Results: The protocol was implemented at four medical centers. Consent for organ donation was received from four of the six potential donors meeting criteria for inclusion, in all cases, from a surrogate decision maker. Of the eight kidneys retrieved, only four were suitable for transplantation, which was carried out successfully for four recipients. Graft function remained normal in all cases in 6–12 months follow-up. 

Conclusions: The DCCD program was successfully implemented and initial results are encouraging, suggesting that expansion of the program might further aid in decreasing the gap between needs and availability of organs.

 

Primary Sjögren’s syndrome (pSS) is a chronic autoimmune disease mainly affecting exocrine glands. However, a subgroup of patients experiences extraglandular manifestations which worsens disease prognosis. To date evidence based guidelines for the management of pSS are lacking, hence the therapeutic approach is mainly based on expert opinion and data from other connective tissue diseases. In recent years, several studies have explored the efficacy and safety of biologic agents in pSS and after the failure of tumor necrosis factor inhibitors, the attention has been focused on compounds directly targeting B or T lymphocytes. The aim of this review article is to provide an overview of available data about B and T cell targeting in pSS and of future directions based on ongoing trials. 

Background: The goals of treatment for rheumatoid arthritis (RA) are remission and low disease activity (LDA). However, many patients do not reach or maintain these targets with regard to disease control. 

Objective: To identify predictive factors of remission/LDA in a cohort of RA patients who started treatment with first line tumor necrosis factor-inhibitors (TNF-i). 

Methods: We included 308 RA patients treated with first line TNF-i for 2 years to evaluate remission/LDA based on the 28-joint disease activity score (DAS28). Predictive factors considered for achievement of remission/LDA were: gender, age at the time of TNF-i treatment, early arthritis, baseline C-reactive protein (CRP) and erythrocyte sedimentation rate levels, RF/anti-citrullinated protein antibody positivity, good/moderate European League Against Rheumatism response at 6 months, co-morbidities, and concomitant disease modifying antirheumatic drugs (DMARDs). Intention to treat, receiver operating characteristic curve, and univariate and multivariate analyses by logistic regression were performed. 

Results: Positive predictors of remission/LDA in both the univariate and the multivariate analyses were: male gender, age at the time of TNF-i treatment ≤ 54 years, negative baseline CRP, and concomitant DMARDs. The presence of any co-morbidity resulted to be a negative predictor of remission/LDA in both the univariate and the multivariate analyses. 

Conclusions: Demographic and clinical features were identified as reliable predictors of both the achievement and the maintenance of treatment targets in a cohort of RA patients treated for 2 years with first line TNF-i. 

 

Background: Pulmonary arterial hypertension (PAH) is a significant consequence of congenital heart disease (CHD). Its presence and severity is associated with increased morbidity and mortality. 

Objectives: To evaluate the clinical and demographic characteristics of adults with congenital heart diseases (ADCHD) and PAH at a single center. 

Methods: A prospective registry of all patients with PAH was conducted between 2009 and 2015. 

Results: Thirty-two patients were identified. The mean age at the last visit was 44 years (range 19–77 years). The prevalence of PAH among all ADCHD patients was 6% (95% confidence interval 4.3%–8.4%). A much higher prevalence (53%) was found in patients with Down syndrome. Most patients with PAH had moderate or severe disease. Fifteen patients (47%) were treated with pulmonary vasodilators and 6 (19%) with combination therapy. The average World Health Organization functional class was 2.6. Morbidity included cerebral vascular accident or transient ischemic attack in 22% (mostly in patients with right-to-left shunt) and arrhythmia in 37% of the patients. During a median follow-up of 3.5 years, 5 patients (15.6%) died. Of 13 women with no mental retardation, 11 were or had been married and all had children (between 1 and 13, mean 3.3). 

Conclusions: Patients with congenital heart disease and PAH have significant morbidity and mortality. PAH is more prevalent in patients with Down syndrome. While pulmonary pressure during the reproductive years was not always known, 27% of women with PAH at the time of the study were multiparous.