Schnitzler syndrome - an under diagnosed clinical entity

1. Tania Jain1, 
2. Chetan P. Offord2, 
3. Robert Kyle2 and 
4. David Dingli2,*

+Author Affiliations

1. ¹ Wayne State University, USA;
2. ² Mayo Clinic, USA

1. ↵* Corresponding author; email: dingli.david@mayo.edu

Abstract

Schnitzler syndrome is considered to be a rare disorder characterized by a monoclonal IgM protein and chronic urticaria that is associated with considerable morbidity. We hypothesized that the syndrome may be under recognized and patients may be deprived of highly effective therapy in the form of anakinra. We performed a retrospective search of the dysproteinemia database at Mayo Clinic as well as the medical records of all patients with chronic urticaria to determine the true incidence of the disease. We compared patients with the diagnosis of Schnitzler syndrome and those who met the criteria but in whom the syndrome was not recognized. Comparisons between groups were performed and survival curves determined. We identified 16 patients with diagnosed Schnitzler syndrome and an additional 46 patients who met diagnostic criteria. The monoclonal protein was IgMκ in 94% of patients. Therapy with anakinra in 4 patients led to rapid and complete resolution of symptoms. The median overall survival for this syndrome is greater than 12.8 years. Progression to lymphoma was only observed in 8% of patients which is lower than what has previously been reported. Schnitzler syndrome may be present in up to 1.5% of patients with a monoclonal IgM in their serum and likely under-recognized as a clinical syndrome.

• Monoclonal Gammopathy of Undetermined Significance
 
• Quality of Life
• Clinical and Molecular Epidemiology

• This Article

  1. Published online before print June 28, 2013, doi:10.3324/haematol.2013.084830haematol June 28, 2013haematol.2013.084830
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• Received January 31, 2013.
• Accepted June 10, 2013.

• Copyright © 2013, Ferrata Storti Foundation

Correlation of Increased Blood Levels of GITR and GITRL with Disease Severity in Patients with Primary Sjögren’s Syndrome

Clinical and Developmental Immunology
Volume 2013 (2013), Article ID 340751, 9 pages
http://dx.doi.org/10.1155/2013/340751

Research Article

Correlation of Increased Blood Levels of GITR and GITRL with Disease Severity in Patients with Primary Sjögren’s Syndrome

Xiaoxia Gan, Xiaoke Feng, Lei Gu, Wenfeng Tan, Xiaoxuan Sun, Chengyin Lv, and Miaojia Zhang

Department of Rheumatology, The First Affiliated Hospital of Nanjing Medical University, 300 Guangzhou Road, Nanjing 210029, China

Received 3 May 2013; Accepted 20 June 2013

Academic Editor: Guixiu Shi

Copyright © 2013 Xiaoxia Gan et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Glucocorticoid-induced tumor necrosis factor receptor family-related protein (GITR) is a type I transmembrane protein belonging to the TNFR superfamily. After activated by its ligand GITRL, GITR could influence the activity of effector and regulatory T cells, participating in the development of several autoimmune and inflammatory diseases included rheumatoid arthritis and autoimmune thyroid disease. We previously reported that serum GITRL levels are increased in systemic lupus erythematosus (SLE) patients compared with healthy controls (HC). Here, we tested serum soluble GITR (sGITR) and GITRL levels in 41 primary Sjögren’s syndrome (pSS) patients and 29 HC by ELISA and correlated sGITR and GITRL levels with clinical and laboratory variables. GITR and GITRL expression in labial salivary glands was detected by immunohistochemistry. pSS patients had significantly increased serum levels of sGITR and GITRL compared with controls (GITR: 5.66 ± 3.56 ng/mL versus 0.50 ± 0.31 ng/mL; ; GITRL: 6.17 ± 7.10 ng/mL versus 0.36 ± 0.28 ng/mL; ). Serum sGITR and GITRL levels were positively correlated with IgG (GITRL: , ; sGITR: , ) and ESR (GITRL:  ; sGITR: , ). Moreover, GITR and GITRL are readily detected in the lymphocytic foci and periductal areas of the LSGs. In contrast, the LSGs of HC subjects did not express GITR or GITRL. Our findings indicate the possible involvement of GITR-GITRL pathway in the pathogenesis of pSS. Further studies may facilitate the development of targeting this molecule pathway for the treatment of pSS.

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MicroRNAs Implicated in the Immunopathogenesis of Lupus Nephritis

Clinical and Developmental Immunology
Volume 2013 (2013), Article ID 430239, 13 pages
http://dx.doi.org/10.1155/2013/430239

Review Article

MicroRNAs Implicated in the Immunopathogenesis of Lupus Nephritis

Cristen B. Chafin¹ and Christopher M. Reilly^1,2

¹Department of Biomedical Sciences & Pathobiology, Virginia-Maryland Regional College of Veterinary Medicine, Virginia Polytechnic Institute and State University, Blacksburg, VA 24061, USA
²Edward Via College of Osteopathic Medicine, Blacksburg, VA 24060, USA

Received 12 February 2013; Revised 20 May 2013; Accepted 12 June 2013

Academic Editor: Richard J. Quigg

Copyright © 2013 Cristen B. Chafin and Christopher M. Reilly. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by the deposition of immune complexes due to widespread loss of immune tolerance to nuclear self-antigens. Deposition in the renal glomeruli results in the development of lupus nephritis (LN), the leading cause of morbidity and mortality in SLE. In addition to the well-recognized genetic susceptibility to SLE, disease pathogenesis is influenced by epigenetic regulators such as microRNAs (miRNAs). miRNAs are small, noncoding RNAs that bind to the 3′ untranslated region of target mRNAs resulting in posttranscriptional gene modulation. miRNAs play an important and dynamic role in the activation of innate immune cells and are critical in regulating the adaptive immune response. Immune stimulation and the resulting cytokine milieu alter miRNA expression while miRNAs themselves modify cellular responses to stimulation. Here we examine dysregulated miRNAs implicated in LN pathogenesis from human SLE patients and murine lupus models. The effects of LN-associated miRNAs in the kidney, peripheral blood mononuclear cells, macrophages, mesangial cells, dendritic cells, and splenocytes are discussed. As the role of miRNAs in immunopathogenesis becomes delineated, it is likely that specific miRNAs may serve as targets for therapeutic intervention in the treatment of LN and other pathologies.

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Acute eosinophilic pneumonia leading to acute respiratory failure in a current systemic corticosteroid user

Case Report  Open Access

|    Allergy Asthma Immunol Res. 2013 Jul;5(4):242-244. English. Published online 2013 April 05.  http://dx.doi.org/10.4168/aair.2013.5.4.242  Copyright © 2013 The Korean Academy of Asthma, Allergy and Clinical Immunology • The Korean Academy of Pediatric Allergy and Respiratory Disease Acute Eosinophilic Pneumonia Leading to Acute Respiratory Failure in a Current Systemic Corticosteroid User Hwa Yong Shin,1 Ju Won Choe,2 Minsuk Kwon,3 Ju Young Jang,3 Jae Woo Jung,3 Jae Chol Choi,3 Jong Wook Shin,3 In Won Park,3 Byoung Whui Choi,3 and Jae Yeol Kim3 1Department of Anesthesiology & Pain Medicine, Chung-Ang University College of Medicine, Seoul, Korea. 2Department of Chest Surgery, Chung-Ang University College of Medicine, Seoul, Korea. 3Department of Internal Medicine, Chung-Ang University College of Medicine, Seoul, Korea. Correspondence to: Jae Yeol Kim, MD, PhD, Department of Internal Medicine, Chung-Ang University Hospital, 102 Heukseok-ro, Dongjak-gu, Seoul 156-755, Korea. Tel: +82-2-6299-1396, Fax: +82-2-825-7571, Email: jykimmd@cau.ac.kr Received August 20, 2012; Revised October 11, 2012; Accepted November 06, 2012. This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. Abstract A 69-year-old female patient visited the emergency room with fever (38.3℃) and dyspnea. She had been taking prednisolone (5 mg once per day) and methotrexate (2.5 mg once per week) for rheumatoid arthritis for 2 years. Chest computed tomography (CT) showed bilateral, multifocal ground glass opacity with interlobular septal thickening. Peripheral blood leukocyte count was 6,520/mm3 (neutrophils, 77.4%; eosinophils, 12.1%). During the night, mechanical ventilation was initiated due to the development of severe hypoxemia. Bronchoalveolar lavage fluid showed a high proportion of eosinophils (49%). Her symptoms improved dramatically after commencement of intravenous methylprednisolone therapy. This is the first report of idiopathic acute eosinophilic pneumonia developing in a current user of systemic corticosteroids. Keywords: Acute eosinophilic pneumonia, respiratory failure, corticosteroid.

Assessing the risk of attack in the management of asthma: a review and proposal for revision of the current control-centred paradigm

Online first

Clinical Review

Assessing the risk of attack in the management of asthma: a review and proposal for revision of the current control-centred paradigm

^*John D Blakey¹, Kerry Woolnough², Jodie Fellows^2,3, Samantha Walker⁴, Mike Thomas⁵, Ian D Pavord<sup>6

1</sup> Clinical Sciences, Liverpool School of Tropical Medicine, Liverpool, UK
² Severe and Brittle Asthma Unit, Heartlands Hospital, Birmingham, UK
³ Clinical Health Psychology, Birmingham & Solihull Mental Health Foundation Trust, Birmingham, UK
⁴ Asthma UK, London, UK
⁵ Centre for Academic Primary Care, University of Southampton, UK
⁶ Institute for Lung Health, Glenfield Hospital, Leicester, UK
Received 26 November 2012 • Accepted 13 April 2013 • Online 2 July 2013

Abstract
Asthma guidelines focus on day-to-day control of symptoms. However, asthma attacks remain common. They continue to cause mortality and considerable morbidity, and are a major financial burden to the UK National Health Service (NHS) and the wider community. Asthma attacks have chronic consequences, being associated with loss of lung function and significant psychological morbidity. In this article we argue that addressing daily symptom control is only one aspect of asthma treatment, and that there should be a more explicit focus on reducing the risk of asthma attacks. Management of future risk by general practitioners is already central to other conditions such as ischaemic heart disease and chronic renal impairment. We therefore propose a revised approach that separately considers the related domains of daily control and future risk of asthma attack. We believe this approach will have advantages over the current ‘stepwise’ approach to asthma management. It should encourage individualised treatment, including non-pharmacological measures, and thus may lead to more efficacious and less harmful management strategies. We speculate that this type of approach has the potential to reduce morbidity and healthcare costs related to asthma attacks.

Cite as: Blakey JD, Woolnough K, Fellows J, Walker S, Thomas M, Pavord ID. Assessing the risk of attack in the management of asthma: a review and proposal for revision of the current control-centred paradigm. Prim Care Respir J 2013; Available from: URL: http://dx.doi.org/10.4104/pcrj.2013.00063

Keywords
asthma attack, asthma control, risk, management, asthma guidelines

* Corresponding author. John D Blakey Tel: +44 (0)151 705 3246 Fax: +44 (0)151 705 3370 Email: jblakey@liverpool.ac.uk

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© 2013 Primary Care Respiratory Society UK. All rights reserved.

• Clin Exp Otorhinolaryngol
• v.6(2); Jun 2013
• PMC3687065

Clin Exp Otorhinolaryngol. 2013 June; 6(2): 73–77.

Published online 2013 June 14. doi:  10.3342/ceo.2013.6.2.73

PMCID: PMC3687065

Effects of Rhinophototherapy on Quality of Life in Persistant Allergic Rhinitis

Emel Çadalli Tatar,1 Hakan Korkmaz,2 Ünzile Akpinar Sürenoğlu,1 Güleser Saylam,1 and Ali Özdek1

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Abstract

Objectives

To investigate the effect of rhinophototherapy with medical therapy on quality of life in persistent allergic rhinitis.

Methods

A prospective, randomized study was being performed between December 2009 and March 2010. The study included 65 patients with persistent allergic rhinitis. The diagnosis was confirmed with positive skin tests. All of the patients had house dust mite allergies. We divided the patients into two groups. First group (n=33) was given topical mometasone furoate 200 mcg/day and levocetirizine 5 mg/day for a month. Rhinophototherapy was applied with the same medical therapy to the second group (n=32), twice a week for three weeks continuously. Rhinophototherapy included visible light, ultraviolet A and ultraviolet B. We evaluated patients before the treatment, at the first month and at the third month after treatment with rhinoconjunctivitis quality of life questionnaire, nasal symptom scores and visual analogue scale (VAS) scores.

Results

Improvements of all variables of the quality of life questionnaire, nasal symptom scores and VAS were statistically significant in the second group both on the first and the third months when compared with the first group.

Conclusion

Allergic rhinitis is a social problem and impairs quality of life. Rhinophototherapy with medical therapy improves the quality of life in allergic rhinitis.

Keywords: Allergic rhinitis, Rhinophototherapy, Quality of life, Symptoms scores, Visual analogue scale

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