Evaluation of Intravenous Immunoglobulin G treatment in Outpatients Rheumatology Practice

Objective: Intravenous immunoglobulin is an alternative therapeutic agent that can be used off-label in many autoimmune rheumatological diseases. The aim of this study is to evaluate the autoimmune rheumatological diseases characteristics in which intravenous immunoglobulin therapy is used and the efficacy and safety of this therapy. Methods and Methods: We performed a retrospective review of 133 patients with autoimmune rheumatological disease who received at least 1 course of intravenous immunoglobulin treatment at Hacettepe University Rheumatology Outpatient Clinic between January 2013 and December 2020. The autoimmune rheumatological disease demographic and clinical features, organ involvements, treatment phases (primary-secondary or infection), treatment responses and adverse effects were evaluated. Results: A total of 79% (n=105) patients were female and the mean±SD age was 45.5±16.9 years. The most common underlying rheumatic diseases were systemic lupus erythematosus (35%, n=47) and dermatomyositis/polymyositis (35%, n=47). Intravenous immunoglobulin therapy was most commonly used for resistant/relapsed myositis and haematological involvement. The median (IQR) intravenous immunoglobulin treatment course was 6.5 (13) and the duration of intravenous immunoglobulin treatment was 10.8 (24) months. Although it is used as second-line therapy in 77% of patients, complete clinical response was observed in 32% and partial response in 47%. There was a significant reduction in the median (IQR) steroid doses (methylprednisolone or equivalent dose) patients received from baseline after intravenous immunoglobulin treatment [30 (33) vs 8 (12), p<0.0001]. It was observed that the use of conventional disease-modifying antirheumatic drugs decreased after intravenous immunoglobulin treatment and the use of rituximab increased. Adverse effects associated with intravenous immunoglobulin treatment (10%) and discontinuation (4%) were found to be very low. Conclusion: Intravenous immunoglobulin treatment was commonly given in systemic lupus erythematosus and dermatomyositis/polymyositis patients because of hematological involvement and resistant/relapsed myositis in our study, respectively. Although it is mainly the second-line treatment, two-thirds of the patients achieved a complete/partial response. Side effects and related discontinuation due to intravenous immunoglobulin treatment are very few.

Factors influencing surgical success in concomitant horizontal strabismus

Objective: To identify the factors affecting surgical success in patients who underwent horizontal concomitant strabismus surgery. Materials and Methods: Medical records of 143 patients who underwent concomitant horizontal strabismus surgery and who were followed up at least 6 months at Ordu University, Faculty of Medicine, Department of Ophthalmology between January 2015 and December 2020 were reviewed retrospectively. Surgical success was defined as postoperative orthophoria or deviation angle of 10 prism diopters (PD) or less at 6 months after surgery. Results: 101 (70.63%) patients with esotropia and 42 (29.37%) patients with exotropia were operated. The mean follow-up period was 18.54 ± 17.51 months, the mean distance and near preoperative deviation were 46.94 ± 11.62 PD and 47.29 ± 11.81 PD, respectively. The surgical success was 83.92% in all patients, 87.13% in esotropic patients, and 76.19% in exotropic patients. Additional surgery was performed in 8 (5.59%) patients with >20 PD residual strabismus in the following year. Preoperative distance and near deviations were found to be significantly related with surgical success (p=0.020 and p=0.026). As the preoperative deviation decreases, the surgical success increases significantly. The deviation type, age at onset, age at the time of surgery, the interval between diagnosis and surgery, gender, or preoperative amblyopia were not significantly related to surgical success (p>0.05). Conclusion: The surgical success was found to be high following concomitant horizontal strabismus surgery. Since the preoperative deviation angle is the significant factor determining surgical success, preoperative evaluations should be done carefully. Patients and their parents should also be warned about the probability of additional surgery.

Is There an Effect of an Hour Education for Decreasing the Severity of Low Back Pain and Increasing Functionality in Office Workers?

Objectives: In this study, we gave information about appropriate working position to the office workers who have low back pain for last 3 months and we aimed to determine the effect of this intervention on frequency and level of low back pain and functionality of the workers. Materials and Methods: This study was an interventional study. Data were collected in September 2017 and December 2017 using face to face interview method. Information was given by a one-hour conference and distributing 4 informative brochures between October 2017 and November 2017. Results: 409 persons participated in the study and 141 of them who had low back pain for last 3 months formed the intervention group. 59.7% of the participants noted that they usually perform desk work. Logistic regression analysis showed that low back pain was 3,25 times more frequent among women than men, and 2,41 times more frequent among workers who had non-communicable disease. Among workers who had low back pain and attended the conference and read at least one brochure, mean Visual Analog Scale score was 5,97±1,2 before intervention and 5,03±1,1 after intervention (p<0,001) and mean Roland Morris Low Back Pain and Disability Questionnaire score were and 9,98±1,7 before intervention, and 8,91±1,3 after intervention, respectively (p<0,001). No difference was determined among workers who didn’t attend any of these two interventions (p>0,05). Conclusions: Considering the effect of intervention, this result shows that implementation of intervention with health promotion approach at workplace is an appropriate method for reducing severity of low back pain and increasing functionality.

Ophthalmic Manifestations of ANCA-Associated Vasculitis

Ocular manifestations in antineutrophil cytoplasmic antibody-associated vasculitis can be associated with the general or limited form of disease and can even occur in the absence of systemic disease. Ocular manifestations of associated vasculitis can be the first symptom of previously not manifested or undiagnosed systemic disease, allowing ophthalmologists to contribute to the diagnosis. Although its ocular findings are variable and nonspecific, the presence of necrotizing changes and peripheral corneal involvement accompanying scleral inflammation are important clues suggesting systemic vasculitis, especially associated vasculitis. The disease may affect all layers of the eye; scleritis and orbital involvement being the most common. Conjunctivitis, episcleritis, peripheral ulcerative keratitis, uveitis and retinal vasculitis are other ocular findings that may be observed during the disease course. Ocular involvement is most commonly seen in granulomatosis with polyangiitis followed by eosinophilic granulomatosis with polyangiitis and microscopic polyangiitis. Due to the high morbidity and mortality of associated vasculitis, it is very important to recognize the ocular manifestations of vasculitis as a sign of the underlying systemic disease and an indicator for the disease activity. Treatment varies depending on the location and severity of the ocular involvement. Although localized medical and surgical treatments can help to manage associated ocular disease, systemic immunosuppressive medications are often required to control the underlying disease. With the increasing availability and use of biological agents, prognosis has improved in patients with severe ocular complications. Rituximab appears to be useful in inducing remission and controlling relapses in patients with ocular involvement of associated vasculitis, particularly in cyclophosphamide resistant cases. A multidisciplinary approach in the diagnosis, treatment and follow-up of patients with associated vasculitis is required in order to achieve successful results.

Mechanical Versus Manual Chest Compression: A Retrospective-Cohort in Out-of-Hospital Cardiac Arrest

Objective: In cardiac arrest cases, high quality cardiopulmonary resuscitation and effective chest compression are vital issues in improving survival with good neurological outcomes. In this study, we investigated the effect of mechanical chest compression devices on 30- day survival in out-of-hospital cardiac arrest. Materials and Methods: This retrospective case-control study was performed on patients who were over 18 years of age and admitted to the emergency department for cardiac arrest between January 1, 2016 and January 15, 2018. Manual chest compression was performed to the patients before January 15, 2017, and mechanical chest compression was performed after this date. Return of spontaneous circulation, hospital discharge, and 30-day survival rates were compared between the groups of patients in terms of chest compression type. In this study, the LUCAS-2 model piston-based mechanical chest compression device was used for mechanical chest compressions. Results: The rate of return of spontaneous circulation was significantly lower in the mechanical chest compression group (11.1% vs 33.1%; p < 0.001). The 30-day survival rate was higher in the manual chest compression group (6.8% vs 3.7%); however, this difference was not statistically significant (p = 0.542). Furthermore, 30-day survival was 0% in the trauma group and 0.6% in the patient group who underwent cardiopulmonary resuscitation for over 20 minutes. Conclusion: It can be seen that the effect of mechanical chest compression on survival is controversial; studies on this issue should continue and, furthermore, studies on the contribution of mechanical chest compression on labor loss should be conducted.

Incomplete Partition type I: Radiological Evaluation of the Temporal Bone

Objective: Incomplete partition type I is an uncommon congenital anomaly of the inner ear, characterized by typical cystic cochleovestibular appearance. Incomplete partition type I was firstly defined as cystic cochlea and vestibule without large vestibular aqueduct; however, large vestibular aqueduct and/or enlarged endolymphatic duct could rarely be seen in incomplete partition type I anomaly. Correct diagnosis of the type of cochlear malformation and differentiation of incomplete partition type I is necessary for patient management and surgical approach. Our aim was to document the temporal bone imaging findings in a series of patients with incomplete partition type I. Materials and Methods: CT (n=85) and/or MRI (n=80) examinations of 99 ears in 59 incomplete partition type I patients were retrospectively evaluated. All structures of the otic capsule were retrospectively assessed. The appearances of cochlea and vestibule, vestibular aqueduct/endolymphatic duct, semicircular canals were qualitatively evaluated by an experienced neuroradiologist. The vertical dimension of vestibular aqueduct and/or endolymphatic duct (from the point where the duct arises from the vestibule) was measured on CT/MRI. Anterior-posterior diameter of the internal acoustic canal and the diameter of cochlear aperture were measured on CT. The cochleovestibular nerves were evaluated on sagittal-oblique high T2-weighted imaging. Results: All 99 ears had defective partition with unpartitioned cochlear basal turn and absent interscalar septae, separated but cystic cochlea. The vestibule was enlarged in all ears except one. Semicircular canals were usually dysplastic (92.9%). A total of 35 incomplete partition type I ears (35.3%) had large vestibular aqueduct and/or enlarged endolymphatic duct. Internal acoustic canal was wide in 21% of ears. Cochlear aperture was wide in 5.9% of ears. Cochlear nerve was either hypoplastic or aplastic in about a quarter of incomplete partition type I ears. Conclusion: In up to one-third of incomplete partition type I patients, an associated large vestibular aqueduct /endolymphatic duct could be seen accompanying typical inner ear findings. Although the cochlear nerves are normal in the majority of cases, auditory brainstem implantation may be necessary in certain cases of incomplete partition type I anomaly.

Liposomal Amphotericin B Induced Acute Reactions

Three formulations of amphotericin B are available: liposomal, lipid complex and conventional. The liposomal amphotericin B is more preferred agent than other formulations because of its tolerability, safety and potent antifungal activity. However, the liposomal amphotericin B can cause infusion-related reactions. In this case report, we aimed to report a patient who developed infusion-related reactions during the treatment with the liposomal amphotericin B but eventually tolerated the prolonged infusion. In this case report, we present a patient who developed an infusion-related reaction during The liposomal amphotericin B treatment. A 26-year-old male patient with acute promyelocytic leukemia was hospitalized for the third course of chemotherapy. Due to the invasive fungal infection history in previous hospitalizations, the liposomal amphotericin B 400 mg (IV, 5 mg/kg) once daily was initiated as secondary antifungal prophylaxis. Swelling in infusion site and chest pain were reported within 10 minutes of the liposomal amphotericin B administration, and the infusion rate was slowed down to 400 mg/6 hours from 400 mg/2 hours. All these reactions disappeared with prolonged infusion time. The patient received a total of 7 liposomal amphotericin B doses subsequently without any reaction during the chemotherapy cycle. In our experience, the liposomal amphotericin B-induced infusion-related reactions can be resolved by prolonging the infusion time.

Could We Use Vital Signs and Lactate Levels Together to Predict the Prognosis in Abdominal Pain?

Objectives: Abdominal pain occupies most of the emergency department admissions. This entity leads to research various markers for the early detection of causes in patients presenting with abdominal pain. There is limited data about collaborations between lactate levels and vital signs at admission in abdominal pain. Materials and Methods: Patients aged 18 years and older, who had presented with abdominal pain to the Emergency Department, in total 102 patients, were included to the study. The patients’ demographics, vital signs, abdominal physical examination findings, diagnosis and outcomes of patients were recorded. The 1 ml venous blood samples were collected by blood gase injectors from the patients and lactate levels were analyzed. Results: Female patients’ percentile was 68,6% and mean age was 39 years old. The systolic blood pressure levels were normal in 45 patients, low in 26% patients and high in 29% patients. The seventy-six of the patients had normal pulse rate and 26 of them were tachycardic. Most common diagnosis was non-specific abdominal pain (37,25%) and the least common was ovarian torsion/intracystic hemorrhage (0,98%) and obstruction due to hernia (0,98%). There was no statistically significant relation between lactate level and pulse rate (p=0,637), systolic blood pressure (p=0,052), diastolic blood pressure (p=0,095), respiratory rate (p=0,527), body temperature (p=0,040) and oxygen saturation (p=0,905). Similarly, no significant association occurred between lactate levels and diagnosis and outcomes. Conclusion: Further studies including more patients groups have to be done in order to attain more reliable data about this topic.

NK/T cell Lymphoma as a Rare Cause of an Oronasal Fistula

NK/T cell lymphoma is one of the most unique and rare forms of extranodal non-Hodgkin’s lymphoma, mostly derived from natural killer cell lineages and occasionally cytotoxic T cell lines. Due to the non-specific presentation of NK/T cell lymphoma such as nasal obstruction, nasal discharge, and epistaxis, diagnosis is often an issue and can be misleading. Oronasal fistula following a non-healing ulcer on the soft palate, can be one of the clinical presentations of NK/T cell lymphoma. Here, we are reporting a rare case of NK/T cell lymphoma in a 32-year-old gentleman who presented with an oronasal fistula post tissue biopsy for a non-healing ulcer over the soft palate. The tissue biopsy of the soft palate mass was revealed as NK/T cell lymphoma and was staged as Stage1b after computed tomographic imaging revealed a local tumour invasion without sign of nodal involvement and no metastasis. The patient eventually started with the SMILE Protocol and responded well up to the date. The ulcer healed but the fistula persisted. Primary closure was planned by the oromaxillofacial surgery team after completion of chemotherapy.

Conventional Synthetic Dmards in Psoriatic Arthritis: Changing Practice in Biologic Era; Real-Life Results from HURBIO-PsA Registry

Objectives: Psoriatic arthritis is a chronic musculoskeletal disorder which may affect skin, joints, bone and enthesis. Conventional synthetic disease modifying anti-rheumatic drugs are first-line treatment options and biologic disease modifying anti-rheumatic drugs are recommended in psoriatic arthritis patients who are intolerant/not controlled well with conventional synthetic disease modifying anti-rheumatic drugs. Although survival data of the conventional synthetic disease modifying anti-rheumatic drugs without concomitant biologic disease modifying anti-rheumatic drugs are available, the effect of biologic disease modifying anti-rheumatic drugs on the retention of conventional synthetic disease modifying anti-rheumatic drugs is still a question of interest. Materials and Methods: Psoriatic arthritis patients who received at least 1 dose of biologic disease modifying anti-rheumatic drugs, using at least 1 conventional synthetic disease modifying anti-rheumatic drugs (methotrexate, leflunomide, hydroxychloroquine and sulfasalazine) at the time of biologic disease modifying anti-rheumatic drugs starting visit and registered in the Hacettepe University BIOlogical Database-Psoriatic Arthritis were included in this retrospective longitudinal analysis. Demographic and disease-specific data at first and last follow-up visit were collected. Unadjusted retention rate of each conventional synthetic disease modifying anti-rheumatic drugs was assessed. Overall prescription of conventional synthetic disease modifying anti-rheumatic drugs at first and last follow-up visit were compared. Results: A total of 266 (191(71.8%) female) patients was included. Median follow-up duration under biologic treatment was 43.4 (19.4-80.1) months. Median retention duration of each conventional synthetic disease modifying anti-rheumatic drugs were similar. Between the first and last visit; there was a 29.3% decrease in methotrexate use (61.7% vs. 43.6%; p<0.001), 8.4% decrease in leflunomide use (31.2% vs. 28.6%; p=0.30), 30.0% decrease in sulfasalazine use (11.3% vs. 7.9%; p=0.05), 31.1% decrease in hydroxychloroquine use (16.9% vs. 11.7%; p=0.001), 12.5 % decrease in glucocorticoids use (51.1% vs. 44.7%; p=0.015). At last visit, 59 (22.2%) patients were conventional synthetic disease modifying anti-rheumatic drugs -free: 20 (7.5%) patients were using only glucocorticoids, 39 (14.7%) patients were conventional synthetic disease modifying anti-rheumatic drugs + glucocorticoid-free. Conclusion: Although conventional synthetic disease modifying anti-rheumatic drugs were significantly discontinued in an important percent of patients after the initiation of biologic disease modifying anti-rheumatic drugs, percentage of patients using glucocorticoids at last visit was still high. Studies aiming to demonstrate when, in whom and how to discontinue conventional synthetic disease modifying anti-rheumatic drugs are needed.