Helping You Find Full Text Journal Articles

Search Results:

'Schnitzler Syndrome' (299)



View Full Text PDF Listings View primary source full text article PDFs.

Dec
1969

The paper reviews the publications dealing with Schnitzler syndrome, a rare autoinflammatory disease, and describes the authors' own clinical observation. It describes the first Russian experience in successfully using the interleukin-1 inhibitor canakinumab to treat this disease.

View Full Text PDF Listings View primary source full text article PDFs.

Dec
2017

The Schnitzler syndrome (SS) is a rare and underdiagnosed entity that associates a chronic urticarial rash, monoclonal IgM (or sometimes IgG) gammopathy and signs and symptoms of systemic inflammation. During the past 45 years, the SS has evolved from an elusive little-known disorder to the paradigm of a late-onset acquired auto-inflammatory syndrome. Though there is no definite proof of its precise pathogenesis, it should be considered as an acquired disease involving abnormal stimulation of the innate immune system, which can be reversed by the interleukin-1 receptor antagonist anakinra.

View Full Text PDF Listings View primary source full text article PDFs.

Jan
2018

Chronic urticaria may often be associated with interleukin (IL)-1-mediated autoinflammatory disease, which should be suspected if systemic inflammation signs are present. Here, we report a case of Schnitzler's syndrome without monoclonal gammopathy treated successfully with the IL-1 receptor antagonist anakinra. A 69-year-old man suffered from a pruritic urticarial rash for 12 years.

View Full Text PDF Listings View primary source full text article PDFs.

View Full Text PDF Listings View primary source full text article PDFs.

View Full Text PDF Listings View primary source full text article PDFs.

View Full Text PDF Listings View primary source full text article PDFs.

Sep
2017


View Full Text PDF Listings View primary source full text article PDFs.

Sep
2017

This article provides description about acute toxicity and early follow-up of one patient treated for breast cancer and Schnitzler syndrome. There are no previously reported cases exploring this interaction on medical literature. The expected radiodermitis to occur in the region treated with radiotherapy along with urticarial-like lesions might be challenging in view of the interaction between symptoms and therapeutic measures.

View Full Text PDF Listings View primary source full text article PDFs.

Sep
2017

Hantavirus disease is characterized by endothelial dysfunction. Angiopoietin-1 (Ang-1) and its antagonist angiopoietin-2 (Ang-2) play a key role in the control of capillary permeability. Ang-1 is responsible for maintenance of cell-to-cell contacts whereas Ang-2 destabilizes monolayers.

View Full Text PDF Listings View primary source full text article PDFs.

View Full Text PDF Listings View primary source full text article PDFs.

Aug
2017

Schnitzler Syndrome: a Review.

Curr Rheumatol Rep 2017 Aug;19(8):46
L Gusdorf, D Lipsker
We focus on recent advances in diagnosis and therapeutic strategies, as well as on pathogenesis of Schnitzler syndrome.
New diagnostic criteria were established, and their external validity was assessed in a retrospective cohort study. The cytokine interleukin-1 (IL-1) plays a crucial role in the pathogenesis of the Schnitzler syndrome, and this explains the spectacular efficiency of IL-1 blocking therapies.

View Full Text PDF Listings View primary source full text article PDFs.

Jun
2017

Schnitzler syndrome (SS) is an autoinflammatory dermatosis that often goes undiagnosed for 5-6 years. Patients typically carry a diagnosis of urticaria; however, their cutaneous symptoms fail to respond to typical urticaria therapies and lack symptoms such as pruritus. Additionally, patients with SS may see multiple providers for nonspecific complaints of fever, lymphadenopathy, arthralgias, and bone pain.


View Full Text PDF Listings View primary source full text article PDFs.

View Full Text PDF Listings View primary source full text article PDFs.

Sep
2017


View Full Text PDF Listings View primary source full text article PDFs.

Dec
1969

Biologic Therapy in the Treatment of Chronic Skin Disorders.

Immunol Allergy Clin North Am 2017 05 1;37(2):315-327. Epub 2017 Mar 1.
James M Fernandez, Anthony P Fernandez, David M Lang
Understanding of the immunologic pathways involved in the pathogenesis of skin-related diseases is constantly advancing. Several biologic agents play important therapeutic roles for management of patients with chronic urticaria, atopic dermatitis, and psoriasis, particularly omalizumab for antihistamine-resistant chronic urticaria, interleukin (IL)-1 inhibitors for cryopyrin-associated periodic syndrome and Schnitzler syndrome, dupilumab for recalcitrant atopic dermatitis, and IL-17 inhibitors for psoriasis. The therapeutic utility of biologic agents for patients with immune-related dermatologic disorders is likely to expand in the future.

View Full Text PDF Listings View primary source full text article PDFs.

May
2017

Disorders characterized by predominant or exclusive dermal inflammation.

Semin Diagn Pathol 2017 May 14;34(3):273-284. Epub 2016 Dec 14.
Mark R Wick
Some cutaneous inflammatory disorders are typified by a predominant or exclusive localization in the dermis. They can be further subdivided by the principal cell types into lymphocytic, neutrophilic, and eosinophilic infiltrates, and mixtures of them are also seen in a proportion of cases. This review considers such conditions.

View Full Text PDF Listings View primary source full text article PDFs.

Dec
1969

Accumulation of ammonium and glutamine in blood and brain is a key factor in hepatic encephalopathy (HE) - a neuropsychiatric syndrome characterized by various cognitive and motor deficits. MRI imaging identified abnormalities notably in the basal ganglia of HE patients, including its major input station, the striatum. While neurotoxic effects of ammonia have been extensively studied, glutamine is primarily perceived as "detoxified" form of ammonia.

View Full Text PDF Listings View primary source full text article PDFs.

Nov
2016

Hantavirus disease belongs to the emerging infections. The clinical picture and severity of infections differ between hantavirus species and may even vary between hantavirus genotypes. The mechanisms that lead to the broad variance of severity in infected patients are not completely understood.

View Full Text PDF Listings View primary source full text article PDFs.

Nov
2016

Inflammasomes and dermatology.

An Bras Dermatol 2016 Sep-Oct;91(5):566-578
Daniel Coelho de Sá, Cyro Festa
Inflammasomes are intracellular multiprotein complexes that comprise part of the innate immune response. Since their definition, inflammasome disorders have been linked to an increasing number of diseases. Autoinflammatory diseases refer to disorders in which local factors lead to the activation of innate immune cells, causing tissue damage when in the absence of autoantigens and autoantibodies.

View Full Text PDF Listings View primary source full text article PDFs.

Apr
2017

Schnitzler syndrome is an adult-onset autoinflammatory disease characterized by urticarial exanthema and monoclonal gammopathy accompanied by systemic symptoms such as fever, bone, and muscle pain. Up to now, approved treatment options are not available.
We assessed effects of the anti-IL-1β mAb canakinumab on the clinical signs and symptoms of Schnitzler syndrome.

View Full Text PDF Listings View primary source full text article PDFs.

Apr
2017

Schnitzlers syndrome is an acquired auto-inflammatory disease of still unclear origin. The Strasbourg criteria were adopted (non-infectious fever, chronic urticaria, changes in the bone structure, leukocytosis and higher values of inflammatory markers - CRP and presence of monoclonal immunoglobulin mostly of type IgM, very rarely of IgG) to establish this diagnosis. The first-choice therapy for this disease is the blocking of interleukin-1 effects.

View Full Text PDF Listings View primary source full text article PDFs.

Apr
2017

The Schnitzler syndrome is a rare inflammatory disorder, with a chronic urticaria-like rash and an IgM (rarely IgG) monoclonal gammopathy as cardinal features. Interleukin-1 β is regarded as the key mediator and the interleukin-1 receptor antagonist anakinra has been proposed as first-line treatment. This case series of eleven patients is intended to enhance disease awareness and to compare our centre's experience with that of literature.

View Full Text PDF Listings View primary source full text article PDFs.

Feb
2017

Schnitzler syndrome is characterized by an urticarial rash, a monoclonal gammopathy, and clinical, histological, and biological signs of neutrophil-mediated inflammation. The aim of this study was to assess the applicability and validity of the existing diagnostic criteria in real-life patients.
This multicentric study was conducted between 2009 and 2014 in 14 hospitals in which patients with Schnitzler syndrome or controls with related disorders were followed up.

View Full Text PDF Listings View primary source full text article PDFs.

Jan
2017

Monoclonal gammopathy of cutaneous significance: review of a relevant concept.

J Eur Acad Dermatol Venereol 2017 Jan 8;31(1):45-52. Epub 2016 Aug 8.
D Lipsker
Some dermatologic entities are strongly associated with the presence of a monoclonal gammopathy. They should be referred to as monoclonal gammopathy of cutaneous significance (MGCS). A short review of the main entities that fit into the spectrum of MGCS is provided.

View Full Text PDF Listings View primary source full text article PDFs.

Oct
2016

Clinical differentiation of parkinsonian syndromes is still challenging.
A fully automated method for quantitative MRI analysis using atlas-based volumetry combined with support vector machine classification was evaluated for differentiation of parkinsonian syndromes in a multicenter study.
Atlas-based volumetry was performed on MRI data of healthy controls (n = 73) and patients with PD (204), PSP with Richardson's syndrome phenotype (106), MSA of the cerebellar type (21), and MSA of the Parkinsonian type (60), acquired on different scanners.

View Full Text PDF Listings View primary source full text article PDFs.

View Full Text PDF Listings View primary source full text article PDFs.

Aug
2016

Old Dogs, New Tricks: Monogenic Autoinflammatory Disease Unleashed.

Annu Rev Genomics Hum Genet 2016 Aug 2;17:245-72. Epub 2016 Jun 2.
Monique Stoffels, Daniel L Kastner
Autoinflammatory diseases are inborn disorders of the innate immune system characterized by episodes of systemic inflammation that are mediated largely by myeloid cells. The field of autoinflammatory diseases has been established since 1999, following the identification of the first genes underlying periodic fever syndromes. This review focuses on developments that have transformed the field in the last two years.

View Full Text PDF Listings View primary source full text article PDFs.

Nov
2016

Schnitzler syndrome (SS) is a rare autoinflammatory disorder characterized by a recurrent urticarial rash and a monoclonal immunoglobulin M gammopathy, as well as 2 of the following minor criteria: recurrent fever (>38°C), objective signs of abnormal bone remodeling, elevated C-reactive protein level or leukocytosis, and a neutrophilic infiltrate on skin biopsy. Alternatively, a monoclonal immunoglobulin G gammopathy may be present along with 3 minor criteria for diagnosis.
To report a rare case of SS without monoclonal gammopathy and inform physicians of this possible clinical presentation so that treatment is not delayed.

View Full Text PDF Listings View primary source full text article PDFs.

View Full Text PDF Listings View primary source full text article PDFs.

Jul
2016

Schnitzler syndrome (SchS) is an acquired autoinflammatory disease characterized by chronic urticarial rash in association with monoclonal gammopathy. Patients may progress to lymphoproliferative disorders, but the associated factors and exact risk of progression are still not well defined.
To characterize the clinical findings, laboratory abnormalities and histopathology of patients with SchS and their respective outcomes.

View Full Text PDF Listings View primary source full text article PDFs.

Jun
2016

Plasma cell proliferative disorders cause rare but extremely varied dermatologic manifestations that may occur as an accompaniment to established diagnoses, or may be a first clue of an underlying neoplasm in the setting of clinical suspicion. In some instances skin lesions result from aggregation of misfolded monoclonal immunoglobulins or their fragments, as in light chain-related systemic amyloidosis. On other occasions the cutaneous lesions result from deposits of malignant plasma cells or monoclonal proteins.

View Full Text PDF Listings View primary source full text article PDFs.

Oct
2016

T17 cells have so far been considered to be crucial mediators of autoimmune inflammation. Two distinct types of T17 cells have been described recently, which differed in their polarization requirement for IL-1β and in their cytokine repertoire. Whether these distinct T17 phenotypes translate into distinct T17 cell functions with implications for human health or disease has not been addressed yet.

View Full Text PDF Listings View primary source full text article PDFs.

Jun
2016

Schnitzler syndrome is a rare acquired systemic disease with a chronic evolution and difficult treatment. We report a 50-year-old woman with Schnitzler syndrome for 10 years, with major impact on her quality of life and refractory to conventional therapies. The patient was started on anakinra, an IL-1 receptor antagonist, with a rapid and sustained remission of the syndrome manifestations.

View Full Text PDF Listings View primary source full text article PDFs.

Dec
1969

To evaluate the striatal presynaptic dopamine transporter (FP-CIT-SPECT) and postsynaptic D2 receptor (IBZM-SPECT) binding in patients with corticobasal syndrome (CBS).
FP-CIT and IBZM are commercially available and approved SPECT tracers for in vivo molecular imaging of pre- and postsynaptic nigrostriatal neuronal degeneration, but only few data for CBS are available.
23 patients meeting clinical criteria for early- to mid-stage CBS (disease duration ≤4 years) were examined with SPECT radiotracers FP-CIT and IBZM.

View Full Text PDF Listings View primary source full text article PDFs.

View Full Text PDF Listings View primary source full text article PDFs.

Apr
2017

Azacitidine (AZA) therapy has become the recommended first-line treatment for patients with high-risk myelodysplastic syndromes (MDS) and oligoblastic (<30% bone marrow blasts) acute myeloid leukemia (AML). However, improvement of the efficacy of AZA treatment remains a challenge. We retrospectively tested the hypothesis that VitD levels (25-hydroxyvitamin D3) prior to start of first-line AZA therapy are predictive of overall survival (OS) in patients diagnosed with MDS and secondary oligoblastic AML.

View Full Text PDF Listings View primary source full text article PDFs.

Oct
2016

Schnitzler syndrome is a rare autoinflammatory disease, which is defined by the presence of two major criteria: chronic urticaria and monoclonal immunoglobulin M (IgM) or immunoglobulin G gammopathy, in combination with at least two additional minor criteria: recurrent fever, leukocytosis and/or elevated C-reactive protein (CRP), objective signs of abnormal bone remodelling and a neutrophilic infiltrate in skin biopsy. We report on a 68-year-old female patient with a 10-year medical history of chronic urticaria, recurrent fever, severe arthralgia and increased CRP. Over the years, multiple diagnostic investigations were performed without conclusive findings, and therapeutic attempts with anti-histamines and several immunosuppressive agents had failed.

View Full Text PDF Listings View primary source full text article PDFs.

Apr
2016

Atypical Bone Sclerosis.

Arthritis Rheumatol 2016 Apr;68(4):816
M Ricci, L G Bettazzi, M Varenna, A Marchesoni

View Full Text PDF Listings View primary source full text article PDFs.

Mar
2016

Inhibitory oscillatory mechanisms subserving tic compensation have been put forward in Tourette syndrome. Modulation of the beta rhythm (15-25 Hz) as the well-established oscillatory movement execution-inhibition indicator was tested during a cognitive-motor task in patients with Tourette syndrome.
Performing a Go/NoGo task, 12 patients with Tourette syndrome and 12 matched controls were recorded using whole-head magnetoencephalography.

View Full Text PDF Listings View primary source full text article PDFs.

Dec
1969

Delayed development of the IgM paraprotein in Schnitzler's syndrome.

Scand J Rheumatol 2015 23;44(6):521-2. Epub 2015 Sep 23.
E Mulla, R Neame

View Full Text PDF Listings View primary source full text article PDFs.

View Full Text PDF Listings View primary source full text article PDFs.

Jan
2016

Neutrophilic urticarial dermatosis (NUD) comprises a particular autoinflammatory condition within the spectrum of aseptic neutrophilic dermatoses characterized by a distinct urticarial eruption clinically and a neutrophilic dermatosis histopathologically.
In this study, we reviewed skin biopsies of lesional skin of patients seen in our outpatient clinic for autoimmune dermatoses and in allergy department from 1982 to 2014 that fulfilled these criteria.
A total of 77 biopsies from 50 patients were analyzed histopathologically.

View Full Text PDF Listings View primary source full text article PDFs.

Jul
2015

Schnitzler syndrome (SS) is a rare autoinflammatory disorder characterized by a chronic urticarial rash and a monoclonal immunoglobulin M gammopathy, accompanied by recurrent fever, lymphadenopathy, arthralgia or arthritis, hepato- or splenomegaly and elevated levels of markers of systemic inflammation. Because patients often present to various specialists with different symptoms the syndrome is often undiagnosed, and it can take years before the correct diagnosis is made. Treatment with interleukin-1 receptor antagonists has a rapid effect on SS.

View Full Text PDF Listings View primary source full text article PDFs.

Jul
2015

Schnitzler's syndrome (SchS) is a disabling autoinflammatory disorder, characterized by a chronic urticarial rash, an M-protein, arthralgia, and other signs of systemic inflammation. Anti-interleukin-1 (IL-1) beta antibodies are highly effective, but the pathophysiology is still largely unknown. Here we studied the effect of in-vivo IL-1 inhibition on serum markers of inflammation and cellular immune responses.

View Full Text PDF Listings View primary source full text article PDFs.

Jun
2015

The systemic autoinflammatory syndromes often present with recurrent fevers. They have proved exceptionally informative about the innate immune system. Although extremely rare, they are important to recognise, as many can now be completely controlled by long-term drug therapies.

View Full Text PDF Listings View primary source full text article PDFs.

Jun
2015

Since 2005, an 82-year-old female patient suffered from relapsing, generalized urticarial rash with relapsing fever episodes, arthralgias and burning tibial pain sensations. She unintentionally lost about 10 kg of body weight within 9 months. Systemic treatment with desloratadine, ranitidine, prednisolone and later, ciclosporin A was started.

View Full Text PDF Listings View primary source full text article PDFs.

View Full Text PDF Listings View primary source full text article PDFs.

Dec
1969

Chronic urticaria (CU) is a common condition faced by many clinicians. CU has been estimated to affect approximately 0.5%-1% of the population, with nearly 20% of sufferers remaining symptomatic 20 years after onset.

View Full Text PDF Listings View primary source full text article PDFs.

Aug
2015

In response to recent studies, a better understanding of the risks of renal complications among African American and biologically related living kidney donors is needed.
We examined a database linking U.S.

View Full Text PDF Listings View primary source full text article PDFs.

Back to top