Can the mucosal attacks of Behçet's disease be predicted?

The primary objective of this study was to investigate the association between disease activity and serum and salivary procalcitonin (Pct) levels in patients with Behçet's disease (BD). The study included patients diagnosed with BD and age-matched healthy volunteers (N: 48, N: 32). Serum and salivary Pct levels were quantified using enzyme-linked immunosorbent assay (ELISA) in the laboratories of Diskapi Yildirim Beyazit Training and Research Hospital. No significant disparity was observed in serum and salivary Pct levels between the patient and control groups (p > 0.05). Furthermore, there was no noteworthy correlation between disease activity and serum and salivary Pct values (p > 0.05). However, the serum Pct level in patients with active oral ulcers was significantly elevated compared to those without active oral ulcers (p: 0.003). Serum Pct emerges as a valuable marker for monitoring oral aphthous ulcer attacks within the patient population.

Inflammatory Arthritis in a 19-month-old with Von Hippel-Lindau Disease.

Von Hippel-Lindau disease (VHL) is a rare autosomal dominant disease characterized by progressive development of cysts and tumors. Juvenile idiopathic arthritis (JIA) is a chronic inflammatory disorder and the most common arthritis in children. Although the mechanism of pathogenesis is not fully understood, JIA is thought to be a polygenic, autoimmune-mediated disease. Inherited or acquired disorders resulting in immune dysregulation can lead to neoplastic and autoimmune disease, but very few cases of patients with VHL and concomitant autoimmune disease are reported in the literature. Herein, we describe, to the best of our knowledge, the first reported case of a child with VHL and inflammatory arthritis, and we discuss three possible pathophysiologic mechanisms that could link VHL and JIA. Understanding the shared pathophysiology and genetics of both diseases may help guide future direction of targeted therapies and lead to improved clinical outcomes.

Retropharyngeal involvement in multisystem inflammatory syndrome in children: Case report and review of literature.

A diagnosis of multisystem inflammatory syndrome in children should be made in the appropriate context and after ruling out other infectious causes. At the same time, clinicians should be diligent as the initial presentation can be unusual and the clinical picture can evolve over time. We report a case that was initially diagnosed as a retropharyngeal infection that did not improve on appropriate antimicrobial coverage. However, as the clinical picture evolved, the patient was found to have multisystem inflammatory syndrome in children and appropriately responded to immunomodulatory treatment. Pediatric infectious diseases practice has been significantly affected by the COVID-19 virus and multisystem inflammatory syndrome in children; data are still emerging as the pandemic evolves. We report this case and conduct literature review to expand the body of evidence about the association between multisystem inflammatory syndrome in children and retropharyngeal involvement.

A case of ANCA-associated vasculitis in a 16-year-old female following SARS-COV-2 infection and a systematic review of the literature.

BACKGROUND: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a rare form of vasculitis in children. SARS-CoV-2, the virus that causes COVID-19 infection, seems to trigger autoimmunity and new-onset autoimmune disease in pediatric and adult patients. We present a case of new-onset AAV following COVID-19 infection in an adolescent patient, and we review the literature of AAV following COVID-19 infection. CASE PRESENTATION: An adolescent female with a history of asthma was diagnosed with mild COVID-19 infection and subsequently developed persistent cough, wheezing, hearing loss, arthralgias, and rash. Her imaging and laboratory workup showed pulmonary nodules and cavitary lesions, elevated inflammatory markers, negative infectious testing, and positive ANCA. She was treated with glucocorticoids, rituximab, and mycophenolate mofetil. At six-month follow-up, she had improvement in her symptoms, pulmonary function tests, imaging findings, and laboratory markers. CONCLUSIONS: We report the second case of new-onset anti-PR3, C-ANCA vasculitis and the fourth case of pediatric-onset AAV following COVID-19 infection. A systematic review of the literature found 6 cases of new-onset AAV in adults after COVID-19 infection. Pediatric and adult patients who develop AAV post COVID-19 infection have few, if any, comorbidities, and show marked radiographic and symptomatic improvement after treatment. There is increasing evidence for COVID-19-induced autoimmunity in children and our case highlights the importance of considering AAV in a child following a recent COVID-19 infection because timely treatment may improve clinical outcomes.

The evaluation of pancreas β-cell autoantibodies in non-diabetic COVID-19 patients

ABSTRACT Objective: This study aims to evaluate potential pancreas endocrine damage due to SARS-CoV-2 by measuring β-cell autoantibodies in COVID-19 patients. Subjects and methods: Between June and July 2020, 95 inpatients with a positive COVID-19 test result after polymerase-chain-reaction (PCR) and who met the inclusion criteria were enrolled in our study. Laboratory parameters that belong to glucose metabolism and β-cell autoantibodies, including anti-islet, anti-glutamic acid decarboxylase, and anti-insulin autoantibodies, were measured. β-cell autoantibodies levels of the patients were measured during COVID-19 diagnosis. Positive results were reevaluated in the 3rd month of control. Results: In the initial evaluation, 4 (4.2%) patients were positive for anti-islet autoantibody. Only one (1.1%) patient was positive for anti-glutamic acid decarboxylase autoantibody. No patient had positive results for anti-insulin autoantibody. FPG, HbA1c, and C-peptide levels were similar in patients who were split into groups regarding the initial positive or negative status of anti-islet and anti-GAD autoantibodies (p>0.05). In the 3rd month after the initial measurements, anti-islet autoantibody positivity of 2 (50%) of 4 patients and anti-glutamic acid decarboxylase positivity of 1 (100%) patient were persistent. Finally, 3 (3.1%) patients in the whole group were positive for anti-islet autoantibody in the 3rd month of control. No difference was determined between the initial and the 3rd month of parameters of glucose metabolism. Conclusion: Following an ongoing autoantibody positivity in the present study brings the mind that SARS-CoV-2 may be responsible for the diabetogenic effect. Clinicians should be aware of autoantibody-positive DM as a potential autoimmune complication in patients with SARS-CoV-2.

The evaluation of pancreas ß-cell autoantibodies in non-diabetic COVID-19 patients.

Objective: This study aims to evaluate potential pancreas endocrine damage due to SARS-CoV-2 by measuring ß-cell autoantibodies in COVID-19 patients. Subjects and methods: Between June and July 2020, 95 inpatients with a positive COVID-19 test result after polymerase-chain-reaction (PCR) and who met the inclusion criteria were enrolled in our study. Laboratory parameters that belong to glucose metabolism and ß-cell autoantibodies, including anti-islet, anti-glutamic acid decarboxylase, and anti-insulin autoantibodies, were measured. ß-cell autoantibodies levels of the patients were measured during COVID-19 diagnosis. Positive results were reevaluated in the 3rd month of control. Results: In the initial evaluation, 4 (4.2%) patients were positive for anti-islet autoantibody. Only one (1.1%) patient was positive for anti-glutamic acid decarboxylase autoantibody. No patient had positive results for anti-insulin autoantibody. FPG, HbA1c, and C-peptide levels were similar in patients who were split into groups regarding the initial positive or negative status of anti-islet and anti-GAD autoantibodies (p>0.05). In the 3rd month after the initial measurements, anti-islet autoantibody positivity of 2 (50%) of 4 patients and anti-glutamic acid decarboxylase positivity of 1 (100%) patient were persistent. Finally, 3 (3.1%) patients in the whole group were positive for anti-islet autoantibody in the 3rd month of control. No difference was determined between the initial and the 3rd month of parameters of glucose metabolism. Conclusion: Following an ongoing autoantibody positivity in the present study brings the mind that SARS-CoV-2 may be responsible for the diabetogenic effect. Clinicians should be aware of autoantibody-positive DM as a potential autoimmune complication in patients with SARS-CoV-2.

Multisystem Inflammatory Syndrome in an Adolescent Following SARS-CoV-2 Exposure Despite Three Doses of a COVID-19 Vaccine.

The multisystem inflammatory syndrome in children (MIS-C) is a known complication of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection in the pediatric population. Recent studies have demonstrated high efficacy of a two-dose vaccine series in preventing MIS-C among adolescents. To date, such studies have only included children exposed to SARS-CoV-2 prior to the emergence of the Omicron variant (B.1.1.529). We report a case of an adolescent who received three doses of a vaccine yet developed MIS-C following known exposure to SARS-CoV-2. Given the uncertainty in whether current vaccines offer as much protection against MIS-C due to the Omicron variant or any potential new variants as they have for older variants, pediatric providers should maintain a high index of suspicion for MIS-C regardless of vaccination status.

Could Irisin Levels be Affected by Physical Activity in Patients with Schizophrenia?

OBJECTIVE: The aim of this study was to explore the effect of physical activity and metabolic parameters on irisin levels in patients with schizophrenia and healthy controls. METHODS: Ninety-six patients with schizophrenia and 63 healthy controls comprised the study population. The participants were separated into three groups: inactive, low activity, and sufficiently active according to International Physical Activity Questionnaire short form (IPAQ-SF). We measured irisin levels using Enzyme linked immunosorbent assay. We also calculated exercise levels by using the IPAQ-SF for each individual. The independent samples ttest was used in the data analysis to compare irisin levels according to the activity levels of the patients with schizophrenia and controls. RESULTS: The levels of irisin were higher in the healthy controls (p < 0.001) compared to schizophrenia groups. When the activity levels of the schizophrenia and healthy control groups were compared, the irisin levels of the low activity and sufficiently active groups with schizophrenia were found to be lower than those of the low activity and sufficiently active groups in the healthy controls (respectively p = 0.014; p < 0.001). CONCLUSION: Irisin levels could be affected by physical activity and these results must be supported with new studies.

Evaluation of serum anti-Müllerian hormone levels in women with Hashimoto thyroiditis in the reproductive age

Background/aim: Autoimmune thyroid disease in women is associated with subfertility and early pregnancy loss, and patients with primary ovarian insufficiency have a high prevalence of thyroid autoimmune disorders. The aim of this study was to investigate the association between Hashimoto thyroiditis (HT) and ovarian reserve. Materials and methods: Levels of serum thyroid stimulating hormones, thyroid autoantibodies, and anti-Müllerian hormone (AMH) were measured in women with HT and a healthy control group between 2018 and 2019. Results: Evaluation was made of 108 premenopausal women with HT, and a control group of 172 healthy females with normal antithyroid antibody levels and thyroid function. Serum AMH levels were determined to be significantly lower in the HT group compared to the control group. Conclusion: Ovarian reserve evaluated by serum AMH concentration is affected by thyroid autoimmunity independently of antithyroid antibodies type or titers.

Cryptococcus albidus Fungemia in an Immunosuppressed Child: Case Report and Systematic Literature Review.

Cryptococcus albidus, synonymous with Naganishia albida, rarely causes opportunistic infection in immunocompromised individuals. Its clinical features, particularly in children, are not well defined. Here, we report a case of C albidus fungemia in an immunosuppressed child; we also present results of a systematic review, for which we searched PubMed, Embase, and Web of Science using the keywords "cryptococcus" and "albidus." Our goal was to describe the spectrum of disease, diagnostic approaches, therapies, and outcomes. We identified 20 cases of invasive infection, only 2 of which involved children, and 7 cases of noninvasive infection. The reports originated in the Americas, Europe, and Asia. Of those with invasive infection, 16 (80%) patients had an underlying chronic disorder or had received immunosuppressive therapy, 8 (40%) had fungemia, and 6 (30%) had a central nervous system infection. The attributable case fatality rate was 40%. C albidus is an opportunistic yeast that can rarely cause life-threatening fungemia and central nervous system infection in individuals of any age, especially those who are immunocompromised.

The relationship between semiquantitative parameters derived from technetium-99m metoxyisobutylisonitrile dual-phase parathyroid single-photon emission computed tomography images and disease severity in primary hyperparathyroidism.

OBJECTIVE: The relationship between multiple semiquantitative indices on technetium-99m metoxyisobutylisonitrile (Tc-99m MIBI) parathyroid single-photon emission computed tomography (SPECT) and clinical, laboratory, and radiological data was investigated in primary hyperparathyroidism. PATIENTS AND METHODS: Ninety-three patients who had a histopathologically confirmed single parathyroid adenoma (PT) were enrolled. Regions of interests were drawn around the PT, neighboring thyroid (T), and background in early and late planar images and isocontour regions of interests around the adenoma in SPECT images (PT SPECT). The relationships between early and late PT, parathyroid adenoma counts/neighboring thyroid counts, parathyroid counts-thyroid counts (PT-T), PT washout, retention index, PT SPECT and serum parathormone (PTH), Ca, P, urinary Ca levels, weight of the adenoma, neck ultrasonography, renal ultrasonography, and bone mineral density findings were investigated. RESULTS: There was a positive correlation between the weight of the adenoma and serum PTH and calcium (Ca) levels (P<0.001), between serum PTH and Ca levels (P<0.001), early PT-T and serum Ca levels (P=0.027), late PT-T and weight of the adenoma (P=0.04), and PT SPECT and serum Ca levels (P=0.046) and a reverse correlation between PT SPECT and serum phosphorus (P) levels (P=0.04). Serum Ca levels were significantly higher and P levels were lower in the group with PT SPECT values above 116. PT SPECT and late parathyroid adenoma counts/neighboring thyroid counts values were significantly higher in the group with serum Ca levels of more than 11 mg/dl. Femoral T and Z scores were significantly lower in patients with lower PT washout. Early PT was significantly lower in patients with coexisting thyroiditis compared with patients with both thyroiditis and thyroid nodules (P=0.034). CONCLUSION: Semiquantitative evaluation of a Tc-99m MIBI parathyroid SPECT study may help predict disease severity in primary hyperparathyroidism.

SIADH in Systemic JIA Resolving After Treatment With an IL-6 Inhibitor.

Interleukin-6 (IL-6) is implicated in the pathogenesis of both systemic juvenile idiopathic arthritis (SJIA) and syndrome of inappropriate secretion of antidiuretic hormone (SIADH), but the 2 have not been previously described as occurring together. We report a case of a 6-year-old girl with symptoms of arthralgia, daily fevers, evanescent rash, lymphadenopathy, and laboratory evaluation showing elevated inflammatory markers, consistent with SJIA. At presentation, the patient had hyponatremia with a sodium level of 128 mEq/L. She had low serum osmolality with elevated urine osmolality, consistent with SIADH. Hyponatremia improved temporarily during times of fluid restriction as expected in SIADH, but did not resolve until SJIA was treated successfully with tocilizumab, an IL-6 receptor antibody that inhibits IL-6 activity. The positive response to treatment with tocilizumab supports the role of IL-6 in the pathogenesis of both SJIA and SIADH. Patients with SJIA should be monitored for SIADH to avoid complications of untreated hyponatremia.

A Sweet Case of Mycoplasma.

Sweet syndrome, also known as acute febrile neutrophilic dermatosis, is an uncommon inflammatory disorder marked by fever and swelling of the skin that can be very painful. It is especially rare in the pediatric population. Infection is a well-known trigger for Sweet syndrome, but this entity has, to our knowledge, never been described after Mycoplasma infection. Herein, we describe the first pediatric case of febrile neutrophilic dermatosis associated with Mycoplasma infection.

The relationship between serum levels of angiogenin, bFGF, VEGF, and ocular involvement in patients with Behçet's disease.

OBJECTIVES: The aim of this study was to investigate the possible role of angiogenin, vascular endothelial growth factor, (VEGF) and basic fibroblast growth factor (bFGF) in the pathogenesis of BD. DESIGN AND METHODS: Sixty-five patients with BD and 21 healthy control subjects were included in the study, and serum angiogenin, bFGF, and VEGF concentrations were measured by using in-vitro enzyme immunoassay (ELISA) kits according to the manufacturer's instructions. RESULTS: The median serum angiogenin level was significantly higher in patients with BD (391.8; range:151.6-594.8 pg/ml) than controls (298.8; range:241.9-449.6 pg/ml) (p = 0.001). The levels were similar in both ocular and non-ocular BD patients (p = 0.537). The mean serum bFGF level was higher in patients with BD (38.8 ± 12.3 pg/ml) than controls (33.2 ± 11.3 pg/ml); the median serum VEGF level was also higher in BD patients (239.7; range:53-991.3 pg/ml) than controls (189.4; range:53.6-357.9 pg/ml). But these differences were not statistically significant. Serum bFGF and VEGF levels were also not different statistically in ocular and non-ocular Behçet's patients. There was no statistically significant relationship between serum angiogenin, bFGF, and VEGF levels and the presence of active eye disease or anatomic location of uveitis. While there was a correlation of borderline significance in angiogenin levels between the patients with anterior uveitis and panuveitis (p = 0.053), we did not obtain any correlation between serum angiogenin, bFGF, and VEGF levels and the duration of BD. CONCLUSIONS: This study suggests that angiogenin may be associated with pathophysiology of BD, and highlights the need of further investigation of the role of angiogenin, bFGF, and VEGF serum levels in BD susceptibility and its clinical manifestations.

Serum vitronectin levels in patients with Behçet's disease.

BACKGROUND/AIMS: To determine serum vitronectin levels in Behçet patients with and without ocular involvement, and to evaluate the relationship between vitronectin concentrations and clinical manifestations of Behçet's disease (BD). METHODS: Sixty-five patients with BD and 21 control subjects were included. All patients were queried for the clinical manifestations of BD. Serum vitronectin concentrations were determined by using in vitro enzyme immunoassay kits. RESULTS: Serum vitronectin levels between the patients and the control subjects were not different. There was no statistically significant difference between vitronectin levels in Behçet patients with and without ocular involvement. No correlation was found between vitronectin concentrations and clinical manifestations. CONCLUSION: This is the first study evaluating vitronectin levels in Behçet patients. Further studies involving larger numbers of subjects would be useful to improve our understanding of the functions of vitronectin in BD.

Consensus treatment plans for induction therapy of newly diagnosed proliferative lupus nephritis in juvenile systemic lupus erythematosus.

OBJECTIVE: To formulate consensus treatment plans (CTPs) for induction therapy of newly diagnosed proliferative lupus nephritis (LN) in juvenile systemic lupus erythematosus (SLE). METHODS: A structured consensus formation process was employed by the members of the Childhood Arthritis and Rheumatology Research Alliance after considering the existing medical evidence and current treatment approaches. RESULTS: After an initial Delphi survey (response rate = 70%), a 2-day consensus conference, and 2 followup Delphi surveys (response rates = 63-79%), consensus was achieved for a limited set of CTPs addressing the induction therapy of proliferative LN. These CTPs were developed for prototypical patients defined by eligibility characteristics, and included immunosuppressive therapy with either mycophenolic acid orally twice per day, or intravenous cyclophosphamide once per month at standardized dosages for 6 months. Additionally, the CTPs describe 3 options for standardized use of glucocorticoids, including a primarily oral, a mixed oral/intravenous, and a primarily intravenous regimen. There was consensus on measures of effectiveness and safety of the CTPs. The CTPs were well accepted by the pediatric rheumatology providers treating children with LN, and up to 300 children per year in North America are expected to be candidates for the treatment with the CTPs. CONCLUSION: CTPs for induction therapy of proliferative LN in juvenile SLE based on the available scientific evidence and pediatric rheumatology group experience have been developed. Consistent use of the CTPs may improve the prognosis of proliferative LN, and support the conduct of comparative effectiveness studies aimed at optimizing therapeutic strategies for proliferative LN in juvenile SLE.

Urokinase plasminogen activator receptor levels in Behcet's disease.

PURPOSE: To determine urokinase plasminogen activator receptor (uPAR) concentrations in Behcet patients with and without ocular involvement; and to investigate the associations between uPAR levels and clinical manifestations of Behcet's disease. METHODS: Sixty-four patients with Behcet's disease (31 patients with and 33 patients without ocular involvement) and 23 healthy control subjects were included in this study. A complete ophthalmologic examination was performed. Venous blood was collected from all patients and control subjects. Serum uPAR levels were determined by using human uPAR immunoassay (Quantikine) kits. RESULTS: There was no statistically significant difference in serum uPAR levels between the patients and the control subjects (p>0.05). There were no statistically significant correlations between uPAR levels and age, gender, duration of the disease, clinical manifestations (genital ulcer, arthritis, skin lesions, ocular and vascular involvements) and activity of the disease. CONCLUSION: This finding is important since this is the first study regarding uPAR levels in Behcet's disease.

Evaluation of serum resistin levels in patients with ocular and non-ocular Behçet's disease.

BACKGROUND: Resistin, a recently identified adipocytokine, has been found to play an important role in inflammation and the processes of inflammation-related diseases. Serum resistin levels in patients with Behçet's disease (BD) have not yet been investigated. We aimed to evaluate the relation between resistin and interleukin-6 (IL-6) in Behçet patients with or without ocular involvement and in normal controls. METHODS: Twenty-two patients with BD and 19 healthy control subjects were included in this study. While 14 patients had posterior segment involvement of the eye, the other 8 did not have ocular disease. Serum resistin and interleukin-6 (IL-6), levels were measured in all samples. Data from all groups were tested for statistical significance. RESULTS: The mean resistin and IL-6 concentrations were significantly higher in patients with BD than the control subjects (p = 0.011 and p = 0.0001, respectively). There was a significant difference in resistin and IL-6 levels between the patients with non-ocular BD and controls (p = 0.013 and p = 0.0001, respectively), as well as resistin and IL-6 levels between the ocular BD group and the control group (p = 0.05 and p = 0.0001, respectively). However, there was no significant difference between patients with ocular versus non-ocular BD. INTERPRETATION: Resistin levels were found to be raised in Behçet patients with or without ocular involvement compared with the control subjects.

Serum leptin levels in patients with ocular and nonocular Behçet's disease.

AIMS: To investigate serum leptin levels in Behçet's patients with or without ocular involvement compared with healthy subjects and the relationship between serum leptin and uveitis activity in patients with ocular involvement. METHODS: Fifty-seven patients with Behçet's disease and 20 healthy control subjects were included in this study. While 27 patients had ocular involvement (18 had acute uveitis, 9 had inactive ocular involvement), 30 did not have ocular disease. C-reactive protein, alpha 1-antitrypsin, and serum leptin levels were measured in all samples. RESULTS: There was a significant difference between the patients with Behçet's disease and control group for both logarithm of leptin (P = .000) and logarithm of CRP (P = .031). Logarithm of leptin in non-ocular Behçet's patients was significantly higher compared to its level in ocular Behçet's disease and controls (P = .009). There was a significant difference between the patients with active ocular disease and control group (P = .03). CONCLUSIONS: Leptin might have a possible role in the pathogenesis of Behçet's disease.