scholarly journals Immunoglobulin G4-related Ophthalmic Disease with Peripheral Eosinophilia and Elevated Immunoglobulin E Levels

2021 ◽  
Vol 20 (3) ◽  
pp. 124-128
Author(s):  
Yeon Ju Lim ◽  
Soo Jung Lee
Keyword(s):  
Immunoglobulin E ◽  
Extraocular Muscles ◽  
Diffuse Fibrosis ◽  
High Dose ◽  
Peripheral Eosinophilia ◽  
Immunoglobulin G4 ◽  
Eyelid Swelling ◽  
Laboratory Examinations ◽  
Intravenous Steroids ◽  
Ophthalmic Disease

Purpose: To report a case with peripheral eosinophilia and elevated immunoglobulin (Ig) E levels, subsequently diagnosed as IgG4-related ophthalmic disease involving the extraocular muscles.Case summary: A 56-year-old male visited the allergy department presenting with systemic urticaria and bilateral eyelid swelling that began 5 months prior. Laboratory examinations showed elevated levels of serum eosinophil and IgE, 1,309 IU/uL and 1,793 IU/mL, respectively. Orbital computed tomography revealed that all extraocular muscles and the bilateral exophthalmos were enlarged, and the patient was referred to the ophthalmology department. Eye alignment was orthophoric for all gaze directions, and limited abduction (-1) was noted in both eyes. An incisional biopsy of the extraocular muscles was conducted. Histopathological findings showed lymphoid aggregates, diffuse fibrosis, and an increased IgG4+/IgG+ plasma cell ratio of 40%, which led to the diagnosis of IgG4-related ophthalmic disease. An elevated IgG4 serum level (1,710 mg/dL) was also noted. The patient received high-dose intravenous steroids and eyelid swelling improved after two months. Levels of serum eosinophil, IgE, and IgG4 all decreased after three months.Conclusions: IgG4-related ophthalmic disease may be accompanied by eosinophilia and elevated IgE. These findings may facilitate future diagnoses of this disease.

scholarly journals Upregulated Expression of Activation-Induced Cytidine Deaminase in Ocular Adnexal Marginal Zone Lymphoma with IgG4-Positive Cells

2021 ◽  
Vol 22 (8) ◽  
pp. 4083
Author(s):  
Asami Nishikori ◽  
Yoshito Nishimura ◽  
Rei Shibata ◽  
Koh-ichi Ohshima ◽  
Yuka Gion ◽  
...  
Keyword(s):  
Marginal Zone ◽  
Somatic Hypermutation ◽  
Cytidine Deaminase ◽  
Marginal Zone Lymphoma ◽  
Immunoglobulin G4 ◽  
Intensity Index ◽  
Activation Induced Cytidine Deaminase ◽  
Systemic Disorder ◽  
Lymphoplasmacytic Infiltration ◽  
Ophthalmic Disease

Immunoglobulin G4-related disease (IgG4-RD) is a systemic disorder characterized by tissue fibrosis and intense lymphoplasmacytic infiltration, causing progressive organ dysfunction. Activation-induced cytidine deaminase (AID), a deaminase normally expressed in activated B-cells in germinal centers, edits ribonucleotides to induce somatic hypermutation and class switching of immunoglobulin. While AID expression is strictly controlled under physiological conditions, chronic inflammation has been noted to induce its upregulation to propel oncogenesis. We examined AID expression in IgG4-related ophthalmic disease (IgG4-ROD; n = 16), marginal zone lymphoma with IgG4-positive cells (IgG4+ MZL; n = 11), and marginal zone lymphoma without IgG4-positive cells (IgG4- MZL; n = 12) of ocular adnexa using immunohistochemical staining. Immunohistochemistry revealed significantly higher AID-intensity index in IgG4-ROD and IgG4+ MZL than IgG4- MZL (p < 0.001 and = 0.001, respectively). The present results suggest that IgG4-RD has several specific causes of AID up-regulation in addition to inflammation, and AID may be a driver of oncogenesis in IgG4-ROD to IgG4+ MZL.

Asthma: Anti-Immunoglobulin E Therapy with Omalizumab for Asthma

2007 ◽  
Vol 41 (9) ◽  
pp. 1397-1410 ◽  
Author(s):  
Leslie Hendeles ◽  
Christine A Sorkness
Keyword(s):  
Clinical Trials ◽  
Cost Effectiveness ◽  
Randomized Clinical Trials ◽  
Inhaled Corticosteroids ◽  
Immunoglobulin E ◽  
Data Extraction ◽  
Symptom Control ◽  
High Dose ◽  
Search Term ◽  
Ige Mediated

Objective: To evaluate data on anti-immunoglobulin E (anti-IgE) therapy for asthma. Data Sources: Information was selected from PubMed from 1989 to May 2007 using the search term omalizumab and included randomized, controlled trials. These studies evaluated asthma treatment with omalizumab and focused on its efficacy, tolerability, and cost-effectiveness in this population. Study Selection and Data Extraction: All randomized clinical trials were reviewed (23 were identified and 19 were included; 3 were not relevant and 1 contained duplicative data). Other articles using the search words anti-IgE therapy and cost-effectiveness were evaluated; relevant information was extracted. Data Synthesis: IgE-dependent mechanisms play an important role in the development and maintenance of airway inflammation in asthma. Omalizumab is a subcutaneously administered monoclonal anti-IgE antibody that reduces unbound IgE concentrations and promotes down-regulation of IgE receptors. Results from clinical trials in adults, adolescents, and children with poorly controlled IgE-mediated asthma have shown that omalizumab improves symptom control and allows patients to be managed with lower doses of inhaled corticosteroids (ICS). It has been well tolerated in clinical trials lasting as long as 52 weeks, but injection-site reactions are common (45% in omalizumab group vs 43% in placebo group) and anaphylaxis has occurred in 0.2% of patients. A consensus expert panel has recommended that omalizumab should be considered for patients 12 years of age or older with allergic asthma who are inadequately controlled on guideline-based therapy and require maintenance therapy with systemic corticosteroids or high-dose ICSs, or who have poor adherence to ICS therapy. Conclusions: Anti-IgE therapy provides an effective and generally safe approach to the treatment of patients with IgE-mediated asthma who are not adequately controlled by conventional guideline-based medications. However, the potential benefit must be weighed against the cost and inconvenience of this new therapy.

Therapeutic Effect of Superficial Cryotherapy versus Intralesional Corticosteroids injection in Alopecia Areata

QJM ◽  
2021 ◽  
Vol 114 (Supplement_1) ◽  
Author(s):  
Nour El-dissouki Ibrahim ◽  
Mahira Hamdy Elsayed ◽  
Ahmed Abdel-Fattah Afify
Keyword(s):  
Side Effects ◽  
Alopecia Areata ◽  
Response To Treatment ◽  
High Dose ◽  
Efficacy And Safety ◽  
Steroid Injections ◽  
Intralesional Steroid ◽  
Males And Females ◽  
Intravenous Steroids ◽  
Effects Assessment

Abstract Alopecia areata, one or more round bald patches appear suddenly, most often on the scalp. It can affect males and females at any age. It starts in childhood in about 50%, and before the age of 40 years in 80%. The exact mechanism is not yet understood. There is not yet any reliable cure for alopecia areata. Several topical treatments used for alopecia areata are reported to result in temporary improvement in some people. Their role and efficacy are unknown. Injections of triamcinolone acetonide 2.5–10 mg/ml into patchy scalp, its efficacy is temporary. Oral and pulse intravenous steroids in high dose can lead to temporary regrowth of hair. The sensitisers diphenylcyclopropenone provoke hair growth in treated areas Now, superficial cryotheray is introduced as a possible treatment for Alopecia Areata. With minimial side effects and being less painful. So, this study clarifies the therapeutic efficacy and safety of superficial cryotherapy for treatment of patchy Alopecia areata. Aim of the study The aim of the study is to evaluate and compare the efficacy and safety of Superficial Cryotherapy and Intralesional corticosteroids in the treatment of patchy Alopecia Areata. Patients and methods study included 20 patients complaining of Alopecia Areata. 3 patches of scalp alopecia areata will be randomly subjected to either superficial cryotherapy twice monthly for 3 months (3 cycles, 2-3 seconds) or intralesional steroid injections once monthly for 3 months (triaminoclone acetonide 1:7, 1ml) or intralesional saline (1 ml). Assessment for the response to treatment will be done at one month and three month following treatment by digital photography and by phototrichogram by comparing number of Terminal, and vellus and hair thickness, Also assessment for side effects of therapy will be done. Results Patients treated with TCA showed statistically significant high response compared to patients treated with cryotherapy. Also, side effects assessment showed minimal side effects with treatment with TCA compared to cryotherapy. Conclusion TCA treatment of alopecia areata is much tolerable than treatment with cryotherapy with also better response rates.

The infant and toddler with wheezing

2019 ◽  
Vol 40 (6) ◽  
pp. 393-395
Author(s):  
Nurcicek Padem ◽  
Rachel Glick Robison
Keyword(s):  
Young Children ◽  
Inhaled Corticosteroids ◽  
Immunoglobulin E ◽  
Bacterial Colonization ◽  
High Dose ◽  
Predictive Index ◽  
Double Blind ◽  
Recurrent Wheezing ◽  
Young Infants ◽  
Tract Infections

Recurrent wheezing is common in young infants and toddlers, with 50% of all children having at least one wheezing episode in the first 6 years of life. Initial wheezing episodes in young children often are linked to respiratory infections due to viral pathogens, such as respiratory syncytial virus, human rhinovirus, human metapneumovirus, and influenza virus. Bacterial colonization of the neonatal airway also may be significant in the late development of recurrent wheeze and asthma. Wheezing in young children can be classified into specific phenotypes based on the onset and persistence of wheezing. Although some children will only wheeze transiently in early childhood, persistent wheezing is often classified as immunoglobulin E (IgE) associated and/or atopic or nonatopic. By using a modified asthma predictive index, future development of asthma can be interpreted, especially in high-risk populations. It is recommended to follow National Asthma Education and Prevention Program (NAEPP) guidelines for initiation of treatment; however, asthma management of young children often requires tailored regimens. Inhaled corticosteroids used as a daily controller medication have been shown to aid symptoms and exacerbation control; however, these do not change the natural course of the disease or progression to asthma. Although randomized double-blind studies in preschoolers investigated a role of macrolide antibiotics in early infection as well as high-dose inhaled corticosteroids during severe lower respiratory tract infections, more research is needed in this field to understand mechanisms of asthma development and optimal treatment in this young age group.

Diagnosis and Management of Severe Asthma

2018 ◽  
Vol 39 (01) ◽  
pp. 091-099 ◽  
Author(s):  
Kian Fan Chung
Keyword(s):  
Severe Asthma ◽  
Inhaled Corticosteroids ◽  
Immunoglobulin E ◽  
High Dose ◽  
Blood Eosinophil ◽  
Exhaled Breath ◽  
Eosinophilic Asthma ◽  
Severe Eosinophilic Asthma ◽  
Asthma Patients ◽  
Long Acting

AbstractSevere therapy-resistant asthma has been defined as “asthma which requires treatment with high dose inhaled corticosteroids (ICSs) plus a second controller (and/or systemic corticosteroids) to prevent it from becoming ‘uncontrolled’ or which remains ‘uncontrolled’ despite this therapy”. Patients who usually present with ‘difficult-to-treat asthma’ should first be assessed to determine whether he/she has asthma with the exclusion of other diagnoses and if so, whether the asthma can be classified as severe therapy-resistant. This necessitates an assessment of adherence to medications, confounding factors, and comorbidities. Increasingly, management of severe therapy-resistant asthma will be helped by the determination of phenotypes to optimize responses to existing and new therapies. Severe asthma patients are usually on a combination of high dose ICS and long-acting β-agonist (LABA) and, in addition, are often on a maintenance dose of oral corticosteroids. Phenotyping can be informed by measuring blood eosinophil counts and the level of nitric oxide in exhaled breath, and the use of sputum granulocytic counts. Severe allergic asthma and severe eosinophilic asthma are two defined phenotypes for which there are efficacious targeted biologic therapies currently available, namely anti-immunoglobulin E (IgE) and anti-interleukin (IL)-5 antibodies, respectively. Further progress will be realized with the definition of noneosinophilic or non-T2 phenotypes. It will be important for patients with severe asthma to be ultimately investigated and managed in specialized severe asthma centers.

scholarly journals Influence of female sex and fertile age on neuromyelitis optica spectrum disorders

2016 ◽  
Vol 23 (8) ◽  
pp. 1092-1103 ◽  
Author(s):  
Nadja Borisow ◽  
Ingo Kleiter ◽  
Anna Gahlen ◽  
Katrin Fischer ◽  
Klaus-Dieter Wernecke ◽  
...  
Keyword(s):  
Neuromyelitis Optica ◽  
Age At Onset ◽  
High Dose ◽  
Female Sex ◽  
Gender And Age ◽  
Female Predominance ◽  
Spectrum Disorders ◽  
Intravenous Steroids ◽  
The Impact ◽  
Aquaporin 4 Antibody

Background: Gender and age at onset are important epidemiological factors influencing prevalence, clinical presentation, and treatment response in autoimmune diseases. Objective: To evaluate the impact of female sex and fertile age on aquaporin-4-antibody (AQP4-ab) status, attack localization, and response to attack treatment in patients with neuromyelitis optica (NMO) and its spectrum disorders (neuromyelitis optica spectrum disorder (NMOSD)). Methods: Female-to-male ratios, diagnosis at last visit (NMO vs NMOSD), attack localization, attack treatment, and outcome were compared according to sex and age at disease or attack onset. Results: A total of 186 NMO/SD patients (82% female) were included. In AQP4-ab-positive patients, female predominance was most pronounced during fertile age (female-to-male ratio 23:1). Female patients were more likely to be positive for AQP4-abs (92% vs 55%; p < 0.001). Interval between onset and diagnosis of NMO/SD was longer in women than in men (mean 54 vs 27 months; p = 0.023). In women, attacks occurring ⩽40 years of age were more likely to show complete remission ( p = 0.003) and better response to high-dose intravenous steroids ( p = 0.005) compared to woman at >40 years. Conclusion: Our data suggest an influence of sex and age on susceptibility to AQP4-ab-positive NMO/SD. Genetic and hormonal factors might contribute to pathophysiology of NMO/SD.

scholarly journals A150 INFLIXIMAB INDUCED EOSINOPHILIC PNEUMONITIS IN AN ADOLESCENT

2021 ◽  
Vol 4 (Supplement_1) ◽  
pp. 152-154
Author(s):  
E Carrión-Jaramillo ◽  
M Chilvers ◽  
C Barker
Keyword(s):  
Pulmonary Function ◽  
Respiratory Symptoms ◽  
Nasal Congestion ◽  
Pulmonary Function Tests ◽  
Skin Testing ◽  
Fungal Growth ◽  
High Dose ◽  
Delayed Presentation ◽  
Peripheral Eosinophilia ◽  
Bronchodilator Effect

Abstract Background Infliximab is used to induce and maintain remission of inflammatory bowel disease (IBD). Pulmonary abnormalities in IBD are rare, but comprise airway, parenchymal and interstitial lung complications. Pulmonary adverse effects reported after Infliximab include tuberculosis reactivation, invasive aspergillosis, interstitial pneumonitis, pulmonary oedema and alveolar hemorrhage. Aims Four cases previously reported associate Infliximab to eosinophilic pneumonitis. However, this is the first in pediatrics. Methods Case report of an adolescent female with IBD and a rare adverse reaction to Infliximab Results Our patient was diagnosed with ulcerative pancolitis, Mayo 2, initially treated with 5-ASA. Five months into therapy she was switched to azathioprine and Infliximab (10mg/kg every 8 weeks) due to worsening disease. She attained and maintained clinical and biochemical remission for the next 1.5 years. Azathioprine was stopped at the patient’s request. One month later, she presented with cough and nasal congestion but was afebrile. Peripheral eosinophilia was noted. Salbutamol and steroid inhalers were started, without improvement. She was admitted to hospital due to progressive cough, shortness of breath, and wheezing. Physical examination was otherwise unremarkable. Pulmonary function test showed FVC of 69%, FEV1 of 45% with bronchodilator effect of 12% and a FEV1/FVC of 59. Her blood work showed eosinophilia (4.42 x109/L) and an elevated IgE (4596 ug/L). Serum-specific IgE for Aspergillus was positive; but skin testing was negative. An X-ray showed marked bilateral interstitial thickening in the mid and lower zones. Chest CT showed extensive bronchiectasis with bronchial mucous plugging. A bronchoscopy with bronchoalveolar lavage (BAL) reported marked eosinophilia, negative for mycobacterium, bacterial and fungal growth. Other infectious and autoimmune etiologies were ruled out. She responded successfully to high-dose prednisone (50 mg/day). At discharge, symptoms had improved, and her pulmonary function tests were normal (FEV1 of 95% predicted and FEV1/FVC 84). Upon steroid taper, she relapsed in pulmonary symptoms, eosinophilia, as well as a flare of her IBD. Reinduction with steroids resolved her symptoms and eosinophilia. Due to her loss of response to Infliximab and pulmonary, she was switched to Vedolizumab with good response. After stopping Infliximab her lung function improved significantly (FVC of 96% predicted and FEV1 of 98% predicted) and has remained so. Peripheral eosinophilia dropped to 0.2 x109/L. Her IBD is currently in remission without any respiratory symptoms. Conclusions Eosinophilic pneumonitis can be a complication of Infliximab. Our patient had a delayed presentation of respiratory symptoms, 16 months after a satisfactory response to Infliximab. Although responsive to steroids, she ultimately needed to be switched to another immunosuppressive agent. Funding Agencies None

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