Imaging findings in 22 cases of Schnitzler syndrome: characteristic para-articular osteosclerosis, and the “hot knees” sign differential diagnosis

@article{Niederhauser2014ImagingFI,
  title={Imaging findings in 22 cases of Schnitzler syndrome: characteristic para-articular osteosclerosis, and the “hot knees” sign differential diagnosis},
  author={Blake D. Niederhauser and David Dingli and Robert A. Kyle and Michael D. Ringler},
  journal={Skeletal Radiology},
  year={2014},
  volume={43},
  pages={905-915}
}
Background and purposeSchnitzler syndrome is characterized by chronic urticaria, monoclonal gammopathy, and a risk of developing lymphoproliferative disorders. [] Key MethodMaterials and methodsClinical and pathological chart review identified 22 patients meeting Strasbourg criteria for Schnitzler syndrome.
Rheumatic involvement and bone scan features in Schnitzler syndrome: initial and follow-up data from a single-center cohort of 25 patients
Objective To report on the characteristics and long-term course of rheumatic manifestations in Schnitzler syndrome (SchS). Methods A retrospective cohort study of patients with SchS followed between
Correlative bone imaging in a case of Schnitzler's syndrome and brief review of the literature.
TLDR
The bone scintigraphy pattern may be very suggestive of the correct diagnosis and of bone involvement in this syndrome and lead to a differential diagnosis including infiltrative diseases and dysplastic diseases.
Diagnostic value of 18-F fluorodeoxyglucose PET/CT and bone scan in Schnitzler syndrome
TLDR
Bone scans may be well positioned to distinguish Schnitzler syndrome relapse from other aetiologies of bone, joint, or muscle pain, and PET/CT does not appear to be useful for the diagnosis and follow-up of Schnitzlers syndrome.
Schnitzler syndrome
TLDR
A 63-year-old man presented with non-pruritic urticaria for 14 months, fever of unknown origin for nearly 12 months, and arthralgias of both lower limbs for 10 months of Schnitzler syndrome, and was given prednisone 30 mg per day.
Chronic urticarial eruption associated with monoclonal gammopathy
TLDR
Schnitzler syndrome is a rare acquired autoinflammatory disease characterized by monoclonal immunoglobulin M gammopathy and urticaria and Anakinra, an interleukin-1 receptor antagonist, is the first-line treatment and has been shown to provide fast and sustained symptom relief.
Schnitzler Syndrome: the paradigm of an acquired adult-onset auto-inflammatory disease.
  • L. Gusdorf, D. Lipsker
  • Medicine, Biology
    Giornale italiano di dermatologia e venereologia : organo ufficiale, Societa italiana di dermatologia e sifilografia
  • 2020
TLDR
The evolution is chronic and about 15-20% of patients may develop lymphoproliferative disease, in particular Waldenström disease, a proportion similar to patients with IgM monoclonal gammopathy of undetermined significance, and more rarely AA-amyloidosis.
Schnitzler's syndrome: lessons from 281 cases.
TLDR
This review summarizes the clinical features, efficacy of therapies, and follow-up data of the 281 cases that have been reported to date of Schnitzler’s syndrome.
Schnitzler’s syndrome: lessons from 281 cases
TLDR
This review summarizes the clinical features, efficacy of therapies, and follow-up data of the 281 cases that have been reported to date of Schnitzler’s syndrome.
Transformation of IgM-MGUS into Waldenström´s macroglobulinemia in two of six patients treated for Schnitzler´s syndrome.
TLDR
Close monitoring is suggested of patients with Schnitzler´s syndrome to early capture potential transformation into Waldenström´s macroglobulinemia with succesful treatment of both conditions.
Schnitzler Syndrome: a Review
TLDR
The Schnitzler syndrome is now considered as a late-onset acquired autoinflammatory syndrome in which the cytokine IL-1 plays a crucial role, and the link between autoinflammation and the monoclonal component needs to be further elucidated.
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Schnitzler's Disease as an Important Differential Diagnosis of Chronic Recurrent Multifocal Osteomyelitis: A Case Report
TLDR
The reevaluation of the 13- year-old diagnosis of chronic recurrent multifocal osteomyelitis of a 58-year-old man with chronic ostealgia, night sweat, and pruritic urticarial lesions on the extremities and trunk is presented.
Bone scan appearance supportive of Schnitzler's syndrome: report of two new cases.
TLDR
The scintigraphic appearance in these 2 patients demonstrated increased concentration of the radioactive tracer in the femora, tibiae, shoulder joints, elbow joints, ankle joints, and the metatarsals and correlated with the regions of symptomatic bone pain.
Radiological aid to clinical diagnosis of Schnitzler’s syndrome: multimodality imaging approach
TLDR
Although the patient had an excellent clinical response to anakinra, the sclerotic lesion remained unchanged on follow-up X-ray examinations, and the main radiological differential diagnoses are discussed to help with the characterization of this syndrome.
Schnitzler syndrome: an under-diagnosed clinical entity
TLDR
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 Schnitzler syndrome found it to be under-recognized and patients deprived of highly effective therapy in the form of anakinra.
Schnitzler's syndrome: diagnosis, treatment, and follow‐up
TLDR
First‐line treatment in patients with significant alteration of quality of life or persistent elevation of markers of inflammation should be anakinra and follow‐up should include clinical evaluation, CBC and CRP every 3 months and MGUS as usually recommended.
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Schnitzler’s syndrome: 3-year radiological follow-up
TLDR
The imaging findings and follow-up of a new case with extensive osteosclerosis of the iliac bone, associated with inflammatory signal changes on MRI and foci of increased uptake on bone scintigraphy are reported.
Erdheim–Chester disease and Schnitzler syndrome: so near, and yet so far
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A 56-year-old male presented with diabetes insipidus, noninfectious fever, night sweats, and pain in both lower legs and pelvis compatible with Erdheim–Chester disease, and tissue samples were positive for BRAF V600E mutation on CE-IVD validated BRAF 600/601 StripAssay.
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