Schnitzler syndrome: clinical features and histopathology

Dingli, David; Camilleri, Michael

doi:10.2147/plmi.s69414

Cited by 5 publications

(4 citation statements)

References 42 publications

(90 reference statements)

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…The Strasbourg criteria as described in box 1 include monoclonal IgG or IgM and urticarial rash as the major criteria with recurrent fever >38ºC, abnormal bone remodelling with or without bone pain, neutrophilic dermal infiltrate on skin biopsy, leucocytosis and elevated CRP constituting the minor criteria 2. Definite diagnosis is made with two major criteria and two minor criteria if IgM positive and three minor criteria if IgG present 2. When bone marrow biopsy is completed, there is minimal or no lymphoplasmacytic infiltration seen on pathology 2.…”

Section: Introductionmentioning

confidence: 99%

“…Definite diagnosis is made with two major criteria and two minor criteria if IgM positive and three minor criteria if IgG present 2. When bone marrow biopsy is completed, there is minimal or no lymphoplasmacytic infiltration seen on pathology 2. As a caveat to the use of diagnostic criteria in a rare disease such as Schnitzler syndrome, the clinician must also thoroughly exclude other potential diagnoses.…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Schnitzler syndrome

et al. 2023

View full text Add to dashboard Cite

A woman in her late 40s with a history of psoriatic arthritis presented to us with fever, migratory rash, cervical and axillary lymphadenopathy, and generalised myalgia. Her symptoms did not improve with steroids and her inflammatory markers were in the range of 200 mg/dL for C-reactive protein, erythrocyte sedimentation rate of 71 mm/hour and ferritin of 4000 ng/mL. Infectious workup was negative. Haematological malignancy and autoimmune conditions were among the top differentials, and she was eventually diagnosed with Schnitzler syndrome. A multidisciplinary team consisting of internal medicine, rheumatology, infectious disease and haematology-oncology specialists was involved in the care of this patient. We highlight the diagnostic schema that was followed for this rare and unique constellation of symptoms.

show abstract

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Schnitzler syndrome

et al. 2023

View full text Add to dashboard Cite

show abstract

“…Schnitzler syndrome (ShS) is a rare, acquired systemic autoinflammatory disease characterized by recurrent fever, urticaria, and monoclonal gammopathy. Around 15-20% develop overt lymphoproliferative disease, typically Waldenstrom’s macroglobulinaemia [ 69 ]. Patients usually present in the fifth decade, without a positive family history [ 70 ].…”

Section: Introductionmentioning

confidence: 99%

Autoinflammatory manifestations in adult patients

Rodolfi

Nasone

Folci

et al. 2022

Clinical and Experimental Immunology

View full text Add to dashboard Cite

Summary Autoinflammatory diseases represent a family of immune-mediated conditions characterized by the unchecked activation of innate immunity. These conditions share common clinical features such as recurrent fever, inflammatory arthritis and elevation of acute phase reactants, in the absence of an identified infectious etiology, generally without detectable serum autoantibodies, with variable response to glucocorticoids and in some cases colchicine, which represented the mainstay of treatment until cytokine blockade therapies became available. The first autoinflammatory diseases to be described were monogenic disorders caused by missense mutations in inflammasome components and were recognized predominantly during childhood or early adulthood. However, the progress of genetic analyses and a more detailed immunological phenotyping capacity led to the discovery a wide spectrum of diseases, often becoming manifest or being diagnosed in the adult population. The beneficial role of targeting hyperinflammation via interleukin 1 in complex non-immune mediated diseases is a field of growing clinical interest. We provide an overview of the autoinflammatory diseases of interest to physicians treating adult patients and to analyze the contribution of hyperinflammation in non-immune-mediated diseases; the result is intended to provide a roadmap to orient scientists and clinicians in this broad area.

show abstract

“…Es necesario controlar el recuento de neutrófilos. Hasta la fecha, es el único tratamiento que ha demostrado una eficacia regular en este síndrome.ParaDingli & Camilleri, (2015) es importante cuestionar el diagnóstico del síndrome de Schnitzler, en pacientes que no responden a este fármaco. Sin embargo, en ocasiones, los pacientes pueden responder a dosis más altasde anakinra.…”

unclassified

Revisión bibliográfica del síndrome de Schnitzler

Serrano Wiesner,

Fernández Véliz,

Pasquel Morán

et al. 2023

RECIMUNDO

View full text Add to dashboard Cite

El síndrome de Schnitzler es una constelación única y rara, de signos y síntomas, clínicos y biológicos, que incluyen la manifestación de urticaria crónica, fiebre, dolor óseo, artralgia y una gammapatía monoclonal de tipo IgM. El diagnóstico, en muchas ocasiones, se establece con dificultad, en consecuencia, ocurre un retraso en el mismo, sumando al caso una morbilidad importante. La mayoría de los tratamientos no son eficaces. En este sentido, la presente revisión pretende actualizar y resumir los aspectos generales del síndrome de Schnitzler, especialmente el horizonte diagnóstico y terapéutico. El enfoque metodológico utilizado para la investigación es de tipo bibliográfico – documental, el cual se apoya en diversas bases de datos para obtener información relevante en base al tema de estudio. El diagnóstico se establece mediante la combinación de los hallazgos clínicos, de laboratorio y de imágenes. Los criterios de Estrasburgo para el diagnóstico son ampliamente usados en la actualidad, por cuanto han demostrado ser más prácticos. Estos criterios requieren únicamente la coexistencia de 2 criterios mayores (exantema urticarial crónico y gamapatía monoclonal IgM o IgG) y al menos 2 criterios menores (fiebre intermitente, artralgia o artritis, dolor óseo, linfadenopatía palpable, esplenomegalia o hepatomegalia, velocidad de sedimentación globular elevada, leucocitosis y anormalidades óseas). Así mismo, es importante realizar el diagnóstico diferencial. En la actualidad se dispone de más de 35 modalidades para el tratamiento de esta patología, con diferentes niveles de eficacia, siendo los más efectivos las terapias bloqueantes de IL-1, y entre ellas, el antagonista del receptor de IL-1 Anakinra.

show abstract

Schnitzler syndrome: clinical features and histopathology

Cited by 5 publications

References 42 publications

Schnitzler syndrome

Schnitzler syndrome

Autoinflammatory manifestations in adult patients

Revisión bibliográfica del síndrome de Schnitzler

Contact Info

Product

Resources

About