Management Options for Linear Scleroderma ("En Coup de Sabre").

BACKGROUND: En coup de sabre (ECDS) is a form of linear-scleroderma, primarily affecting the face. Despite effectiveness of pharmacological interventions in the management of lesions, sequelae of cutaneous deformities are common. OBJECTIVE: Review the existing surgical tools for the correction of "en coup de sabre" ECDS lesions and facial deformities. MATERIALS AND METHODS: The authors conducted a literature search for reports on surgical interventions for ECDS deformations, in the electronic databases of PubMed, Scopus, and Cochrane Library Databases. RESULTS: Twenty-six publications reporting 39 patients were found in the literature. The average age of patients was 25 ± 4.7 years. Thirty-one of the patients were females. Six methods were found in the literature for surgical correction of ECDS. Fat grafting was performed in 41% of patients, surgical reconstruction in 18%, injectable fillers in 15%, implants in 10%, botulinum toxin injections in 8%, and bone/cartilage grafts in 8%. Imaging studies of the skull and face were performed in 36% of patients. Bony defects were found in 64.3% of them. CONCLUSION: Different surgical and minimally invasive options exist in the armamentarium of clinicians correcting ECDS deformities. Tailoring the method of correction to patient's needs and expectation is crucial in ensuring patient satisfaction.

Social distancing and bacteraemia in the time of COVID-19.

BACKGROUND: Social distancing was the predominant strategy used to mitigate the spread of Coronavirus disease 2019 (COVID-19) at the start of the COVID-19 pandemic. AIMS: To study the impact of social distancing on the incidence of bacteraemia. The number of admitted patients with positive blood cultures in April-May 2020 in one tertiary medical centre was compared with the number during the same period in the previous 3 years (April-May 2017-2019). METHODS: Retrospective review of all positive blood cultures from January to July in the years 2017-2020. RESULTS: There were fewer cases of Streptococcus bacteraemia as well as coagulase-negative Staphylococcus bacteraemia and other possible contaminated blood cultures in April-May 2020. Compared with the previous 3 years, the incidence of Streptococcus pneumoniae bacteraemia among all bacteraemias was lower in April-May 2020 (5%) than in 2017-2019 (12.0%; 95% confidence interval 10.3-14.1%). In general, fewer cases of bacteraemia caused by oropharynx organisms were observed in April-May 2020; only 6 cases versus 31 (95% confidence interval 10-53) during the same period in 2017-2019. Only one case of S. pneumoniae bacteraemia was observed in April-May 2020 and its percentage among all bacteraemias was lower in April-May 2020 (0.4%) than during the same period in 2017-2019 (3.3%). CONCLUSION: The incidences of streptococcal bacteraemia and bacteraemia of organisms transmitted through respiratory secretions were lower when there were social distancing restrictions. Adopting measures of social distancing may decrease the morbidity from bacteraemia caused by oropharynx and respiratory bacteria.

Ophthalmic adverse events following facial injections of botulinum toxin A: A systemic literature review.

BACKGROUND: Over the years, botulinum toxin has found its place as a neuromuscular blocking agent in numerous medical fields. Since the approval of botulinum toxin by the FDA for cosmetic indications in 2002, it had become the most commonly performed esthetic procedure worldwide, with ever-growing demand. The characteristics of the toxin, along with the facial areas it is injected to, could possibly account for a wide array of complication. METHODS: The authors conducted a literature search for reported cases of ophthalmic adverse events following Botulinum toxin facial injections in the electronic databases of PubMed and Cochrane Library databases. RESULTS: The authors found 25 publications, reporting 49 cases of ophthalmic adverse events following botulinum toxin injections. Injections for cosmetic indications accounted for 51% of all injections, treatment of blepharospasms for 22% of cases, protective ptosis for 11% of cases, and treatment of hemifacial spams for 8% of cases. The average quantity of botulinum toxin injected to a single patient ranged between 1.25 and 75 units, with a median of 13.75 units.Majority of injections for cosmetic indications were performed to the lateral canthal area (56%), followed by the glabella (28%) and the forehead (20%).Adverse events following injections included diplopia (64%), ptosis (14%), and decrease in visual acuity or vision loss (8%). CONCLUSIONS: Botulinum toxin is gaining extreme popularity in the management of a wide area of diseases and for cosmetic indications. Proper knowledge of potential adverse events is crucial for the clinician in attempt to decrease complications.

Clinical implications of ANCA positivity in a hospital setting: a tertiary center experience.

ANCA testing plays an established critical role in the diagnosis of ANCA Associated vasculitis (AAV). The spectrum of diseases associated with positive ANCA has recently broadened, thus calling into question the diagnostic implications of ANCA positivity in a hospital setting. We retrospectively studied all adult patients who had a positive ANCA test (by Indirect Immunofluorescence (IIF), ELISA or both) performed over the span of 19 years. Subjects were then divided into discordant (positive on one assay) and concordant ANCA (positive on both assays) groups based on their ANCA positivity status. The two groups were then compared with regards to their demographic, clinical and laboratory characteristics, the indication for ANCA testing in both groups and their final diagnoses. Of the 9189 ANCA tests ordered during the 19-year span of the study, 389 (4.2%) were positive. Two hundred and forty subjects met the exclusion criteria (patients aged less than 18 years or the lack of clinical and laboratory data in the medical file) thus resulting in a final cohort of 149 subjects. Of them, 122 subjects had discrepant ANCA results and 27 had matching ANCA results. Most cases in the discrepancy group were IIF positive and ELISA negative (86.8%). The diagnosis of AAV was highly unlikely in cases with discrepant IIF and ELISA serologies compared to cases with matching IIF and ELISA serologies (4.1% versus 44.4%, p value < 0.001). The diagnosis of AAV in unlikely in subjects with discrepancies between IIF and ELISA, particularly with only positive IIF.

Ruptured hemorrhagic corpus luteum as a presenting symptom of systemic lupus erythematous.

A young female presented to the emergency room with ruptured hemorrhagic corpus luteum (RHCL). Her workup revealed a new diagnosis of SLE with nephritis and positive lupus anticoagulant (LAC) test without thrombocytopenia. We reviewed the literature and found one similar case of a 23-year-old subject who presented with a RHCL that was found to be the presenting symptom of SLE; unlike the current case, the patient presented with severe anemia (Hg 6.7 g/dl) and thrombocytopenia (10,000/ml). Possible mechanisms are discussed.

Survival of patients with giant cell arteritis: a controversial issue.

OBJECTIVES: Epidemiologic studies differ regarding overall survival in giant cell arteritis (GCA). In this review we evaluated longevity and the impact of several disease parameters on survival of GCA patients. METHODS: Review of the medical literature during the period 1975-2018, using PubMed database. RESULTS: Epidemiologic studies addressing the issue of survival in GCA patients used variable methods of calculating mortality rates in relation to background population or in relation to selected controls. Several epidemiologic studies found that survival of GCA patients was similar to that of the general population. Others reported increased mortality in patients with GCA, or in subgroups of GCA patients. 5-Year and 10-year survival rates differed considerably among studies: 5-year survival rates ranged between 60-90% (except for 2 extremes of 35% and 97%), and 10-year survival rates ranged between 48-81%. Reasons for these discrepancies are unclear, and may be related to differences in populations, in the period of the study, and in study methods. Several studies found that mortality was increased in female GCA patients, and some reported increased mortality early in the course of the disease (mostly within the first 2 years after diagnosis). The deleterious effect of vision loss on survival was noted in a few studies, although most studies did not address the issue of mortality in this particular subgroup of GCA patients. CONCLUSIONS: Epidemiologic studies varied considerably in the reported outcomes of GCA patients: some found that the overall survival was similar to that of the general population while others reported increased mortality in GCA or in subgroups of GCA patients.

Uric Acid Level as a Predictor of Long-Term Mortality in Advanced Age Population.

BACKGROUND: Hyperuricemia is associated with the development, progression and outcome of several diseases. The purpose of this study is to evaluate the serum uric acid (UA) levels as a predictor of long-term mortality in an older population (age 60 years and above). MATERIALS AND METHODS: Patients older than 60 years who were hospitalized in the departments of geriatrics and internal medicine in Shaare Zedek Medical Center in Jerusalem during a period of 4 months (March-June 2014) were included in this observational study. Association between hyperuricemia and long-term mortality were analyzed using multiple logistic regression, Kaplan-Meier and Cox proportional hazards regressions analysis. RESULTS: A total of 624 patients were included in our study with mean age of 77.2 ± 14.6 years. Overall, 381 patients died during the follow-up period (61.1%). Mortality rate in the hyperuricemic group (> 7 mg/dL) was higher (69.1%) than in the normouricemic group 58.4%. (P = 0.004). The median survival for hyperuricemic patients was significantly shorter compared to normouricemic patients (606 and 1018 days, respectively, P < 0.0001). High levels of UA were significantly associated with higher long-term mortality in patients with cardiovascular disease at their admission (P < 0.000). CONCLUSIONS: Elevated levels of UA in older patients in acute settings is a predictor of long-term mortality.

Segmental arterial mediolysis: a vasculitis mimicker. A single centre experience.

OBJECTIVES: Segmental arterial mediolysis (SAM) is a rare vasculopathy of unknown aetiology. It is non-atherosclerotic, non-inflammatory, non-hereditary, non-infectious, large to medium-sized arteriopathy. SAM is a condition which in some circumstances behaves as a vasculitis mimicker and should be recognised in order to provide appropriate treatment and avoid unnecessary immune-suppressive therapy. METHODS: We report a single-centre experience of 6 consecutive SAM cases (3 males and 3 females). A literature search of cases reported with SAM was performed and data summarised. RESULTS: Abdominal or flank pain was the presenting symptom in 5 of the 6 patients. CT angiography (CTA) was the method of diagnosis in all 6 patients. 3 patients underwent therapeutic angiography; 2 with angiographic embolisation because of bleeding, and one patient needed a stent insertion because of left renal infarction. 2 patients underwent FDG-PET to rule out vasculitis. Serological tests were negative in all case, but C-reactive protein was elevated in 4 of them. 2 patients were treated with angiographic embolisation due to bleeding, 2 treated with anti-platelet therapy, one with stent insertion, and one with antihypertensive treatment. A medical literature review of 160 additional cases shows that abdominal or flank pain was the chief complaint in the vast majority of the cases. Renal and abdominal medium-sized arteries were the most commonly involved. CTA was the preferred method of diagnosis. CONCLUSIONS: SAM should be suspected in cases presenting with abdominal or flank pain. Angiographic features should be carefully studied by experienced radiologists to rule out vasculitis.

Trends in antimicrobial resistance among bacteria causing septic arthritis in adults in a single center: A 15-years retrospective analysis.

Septic arthritis (SA) is commonly associated with Staphylococcal or Streptococcal infections. Overtime, there has been a global increase in the distribution of antimicrobial resistance within both Gram-positive bacteria such as methicillin-resistant Staphylococcus aureus (MRSA) and Gram-negative bacteria such as extended-spectrum beta-lactamase (ESBL) positive Enterobacteriacea. The aim of this study was to determine whether this change in epidemiology similarly affected the distribution of resistant pathogens causing SA. The study was conducted at the Shaare Zedek Medical Center in Jerusalem, Israel. All adult patients diagnosed with SA during 2002-2016 were included in the cohort. Antimicrobial resistance trends were examined over three periods: 2002-2009, 2010-2013, and 2014-2016. Of 85 patients with SA, mean age of patients was 66.8 (± 20.3) years, with male predominance (n = 62, 66%). Most SA cases involved native knee joints and more than 85% (n = 80) were acquired in the community. The most common isolates were S. aureus (n = 38, 45%) and beta-hemolytic streptococci (n = 13, 15%). MRSA SA was diagnosed in 8% of all SA cases (n = 7). An increasing, although non-significant trend in MRSA SA was observed during the study period (p = 0.3). Gram-negative infections were uncommon (n = 14). No ESBL-positive or carbapenem-resistant Enterobacteriacea were detected. Over a 15-year study period, no significant increase in resistant pathogens causing SA was observed. In the era of antibiotic stewardship, these results strengthen our practice of administering narrow-spectrum antimicrobials empirically for SA. However, our findings cannot be generalized to regions with higher rates of MRSA in the community.

Giant Cell Arteritis and Polymyalgia Rheumatica: 2016 Update.

Giant cell arteritis (GCA) and polymyalgia rheumatica (PMR) are both more common among people of North European decent than among Mediterranean people. Women are 2-3 times more commonly affected. Giant cell arteritis and PMR are extremely rare before age 50 years. Polymyalgia rheumatica may be "isolated" or associated with GCA. There is increased expression of inflammatory cytokines in temporal arteries of PMR patients, without overt histological evidence of arteritis. One-third of "isolated" PMR patients have vascular uptake in positron emission tomography (PET) scans, suggesting clinically unrecognized, "hidden" GCA. Typical manifestations of GCA are headache, tenderness over temporal arteries, jaw claudication, PMR, acute vision loss, and low-grade fever. Bilateral aching of the shoulders with morning stiffness is typical for PMR. In both conditions sedimentation rate and C-reactive protein are elevated, and anemia and thrombocytosis may occur. Color duplex ultrasonography of the temporal arteries may aid in GCA diagnosis. Temporal artery biopsy showing vasculitis, often with giant cells, confirms GCA diagnosis. In cases with negative biopsy one must rely on the clinical presentation and laboratory abnormalities. The diagnosis of PMR is made primarily on clinical grounds. Other conditions that may mimic GCA or PMR must be excluded. Glucocorticoids are the treatment of choice for both conditions. Prompt treatment is crucial in GCA, to prevent irreversible complications of acute vision loss and stroke. Addition of low-dose aspirin may further prevent these complications. The average duration of treatment is 2-3 years, but some patients require a prolonged course of treatment, and some may develop disease-related or treatment-related complications. No steroid-sparing agent has been proven to be widely effective thus far, but some promising therapeutic agents are currently being studied.

Dual-energy computed tomography as a diagnostic tool for gout during intercritical periods.

AIM: The aim of this study is to evaluate the diagnostic yield of dual-energy computed tomography (DECT) in detection of uric acid accumulation in joints or periarticular structures in patients suspected of having gout, in their intercritical period. METHODS: Patients with a history of recurrent, short-lived mono- or oligo-arthralgia or arthritis, referred to the rheumatology clinic for diagnosis of their condition, were included in this retrospective evaluation. RESULTS: DECT confirmed the diagnosis of gout in 30 of 50 patients (60%). A positive DECT was present in 12 of 16 cases (75%) with serum uric acid > 8.5 mg/dL, compared to seven of 13 cases (54%) and two of five cases (40%) with levels of 6.1-8.5 mg/dL and ≤ 6 mg/dL, respectively. The diagnostic impact of screening hands and feet were highest (78% and 56%, respectively). Follow-up data were available for 24 of the 30 patients with urate deposits identified by DECT. Twenty-one were treated with urate-lowering agents, all responded with lowering of serum uric acid and cessation of flares. Follow-up data were available for 16 of the 20 patients with no urate deposits identified by DECT. Gout was diagnosed in two of them by synovial fluid examination during subsequent flares. Both positive and negative predictive values of DECT for diagnosing gout in this patient population were 87%. Following DECT, treatment regimen was modified to gout-specific therapy in 52% of the patients. DISCUSSION: The ability to make a definite diagnosis of gout by DECT imaging in a substantial number of asymptomatic patients in the intercritical period should help in treatment decision-making and improve patient adherence to long-term urate-lowering therapy.

The Incidence of Primary Systemic Vasculitis in Jerusalem: A 20-year Hospital-based Retrospective Study.

OBJECTIVE: The incidence of primary systemic vasculitides varies among different geographic regions and ethnic origins. The aim of this study was to examine the incidence rates of vasculitides in the Jerusalem Jewish population, and to examine possible trends in incidence rates over a 20-year period. METHODS: The clinical databases of inpatients at the 2 medical centers in Jerusalem were searched for patients with vasculitis diagnosed between 1990-2009. Individual records were then reviewed by one of the authors. The significance of trends in incidence rates throughout the study period was evaluated by Pearson correlation coefficient. RESULTS: The average annual incidence rate of polyarteritis nodosa was 3.6/million adults (95% CI 1.6-4.7). Incidence rates did not change significantly during this period (r = 0.39, p = 0.088). The incidence of granulomatosis with polyangiitis (GPA) was 4.1 (2.2-5.9) for the whole period, during which it increased significantly (r = 0.53, p < 0.05). The incidence of microscopic polyangiitis (MPA) was lower: 2.3 (1.2-3.5)/million. It also increased significantly (r = 0.55, p < 0.05). The incidence of eosinophilic granulomatosis with polyangiitis was 1.2 (0.4-1.9), which remained stable throughout the study period. The incidence of Takayasu arteritis was 2.1/million (95% CI 1.2-2.9), and it also remained stable. Giant cell arteritis (GCA) incidence was 8.1 (5.7-10.6)/100,000 population aged 50 years or older. In sharp contrast with other vasculitides, its incidence decreased significantly throughout the study period (r = -0.61, p < 0.01). CONCLUSION: The incidence rates of vasculitides in the Jewish population of Jerusalem are in the lower range of global incidence rates. While GPA and MPA incidence are increasing, GCA incidence is decreasing.