Schnitzler's syndrome: diagnosis, treatment, and follow‐up

Simon, Anna; Asli, Bouchra; Braun‐Falco, Markus; Koning, Heleen D. de; Fermand, Jean‐Paul; Grattan, C; Krause, Karoline; Lachmann, HJ; Lenormand, C.; Martínez‐Taboada, Víctor M.; Maurer, Marcus; Peters, Margot S.; Rizzi, Rita; Rongioletti, Franco; Ruzicka, Thomas; Schnitzler, L; Schubert, Bernd; Sibilia, Jean; Lipsker, Dan

doi:10.1111/all.12129

Cited by 224 publications

(300 citation statements)

References 46 publications

Supporting

Mentioning

245

Contrasting

Unclassified

Order By: Relevance

“…Nevertheless, their validity has never been formally evaluated. In 2012 (5), an expert conference was held in Strasbourg and allowed the elaboration of new set of diagnostic criteria known as the 'Strasbourg criteria', summarized in Table 2. The Strasbourg criteria formally allowed the presence of a monoclonal IgG component, which the previous criteria did not, and introduced the notion of dermal neutrophilic infiltrate on skin biopsy.…”

mentioning

confidence: 99%

See 1 more Smart Citation

Schnitzler syndrome: validation and applicability of diagnostic criteria in real-life patients

Gusdorf

Asli

Barbarot

et al. 2016

Allergy

Self Cite

View full text Add to dashboard Cite

Background: Schnitzler syndrome is characterized by an urticarial rash, a monoclonal gammopathy, and clinical, histological, and biological signs of neutrophilmediated inflammation. The aim of this study was to assess the applicability and validity of the existing diagnostic criteria in real-life patients. Methods: 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. We compared the sensitivities and specificities and calculated the positive and negative predictive values of the Lipsker and of the Strasbourg criteria for the patients with Schnitzler syndrome and for the controls. We included 42 patients with Schnitzler syndrome, 12 with adult-onset Still's disease, 7 with cryopyrin-associated periodic disease, 9 with Waldenstr€ om disease, and 10 with chronic spontaneous urticaria. Results: All patients with Schnitzler syndrome met the Lipsker criteria. According to the Strasbourg criteria, 34 patients had definite Schnitzler syndrome, five had probable Schnitzler syndrome, and three did not meet the criteria. One control met the Lipsker criteria and had probable Schnitzler syndrome according to the Strasbourg criteria. Sensitivity and specificity of the Lipsker criteria were 100% and 97%, respectively. For the Strasbourg criteria, sensitivity for definite and probable diagnosis was 81% and 93%, respectively, with a corresponding specificity of 100% and 97%. Conclusion: Diagnostic criteria currently in use to diagnose Schnitzler syndrome are reliable. More investigations must be done to attest their efficiency in patients with recent-onset manifestations.

show abstract

mentioning

confidence: 99%

“…Indeed, in 1995 the first case of Schnitzler syndrome with a monoclonal IgG component was described (6). The experts also felt the need to have the possibility to distinguish between patients with definite Schnitzler syndrome and probable Schnitzler syndrome (5).…”

mentioning

confidence: 99%

Schnitzler syndrome: validation and applicability of diagnostic criteria in real-life patients

Gusdorf

Asli

Barbarot

et al. 2016

Allergy

Self Cite

View full text Add to dashboard Cite

show abstract

“…2,3 The first patient we described had a latency of more than eight years before the correct diagnosis was established.…”

Section: Discussionmentioning

confidence: 99%

“…1 The disease is thought to be an adult onset autoinflammatory syndrome with unknown etiology and unknown pathogenesis. 2,3 The diagnosis is reached after excluding all other more common causes 4 taking into account the Strasbourg diagnostic criteria (Table 1). 2 Treatment strategies depend on several factors, the most important being the general condition of the patient and levels of acute phase reactants.…”

confidence: 99%

“…2,3 The diagnosis is reached after excluding all other more common causes 4 taking into account the Strasbourg diagnostic criteria (Table 1). 2 Treatment strategies depend on several factors, the most important being the general condition of the patient and levels of acute phase reactants. In this article, we aimed to evaluate the different treatment strategies by comparing the clinical courses of two patients, one achieving remission with colchicine and the other with interleukin 1 (IL-1) receptor blockade after failing to respond to the conventional immunosuppressants.…”

confidence: 99%

See 1 more Smart Citation

Different Therapeutic Paths (Colchicine vs. Anakinra) in Two Patients With Schnitzler’s Syndrome

et al. 2016

View full text Add to dashboard Cite

Autoinflammatory Diseases Presenting in the Skin

Lipsker

Grattan

Lovell

2016

Rook's Textbook of Dermatology, Ninth Edition

View full text Add to dashboard Cite

Autoinflammatory diseases comprise a heterogeneous group of inherited monogenic and acquired polygenic multisystem disorders that may present in the skin and are therefore of importance to dermatologists. The inherited monogenic disorders typically present in childhood. Those that present with urticarial rashes are particularly important to differentiate from chronic urticaria since the pathogenesis, prognosis and treatment are completely different. Cryopyrin‐associated periodic syndrome should be suspected in children with a family history of chronic urticaria that does not respond to H 1 antihistamines and who have systemic symptoms of malaise and fever with persistently raised inflammatory indices. Autoinflammatory syndromes also may present with unexplained fever, erysipelas‐like rash (familial Mediterranean fever), oedema (tumour necrosis factor receptor‐associated periodic syndrome), pustulosis and lipoatrophy (chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature), an aseptic pustular dermatosis (deficiency of interleukin 1 receptor antagonist) or pyoderma gangrenosum (pyogenic sterile arthritis, acne and pyoderma gangrenosum). Acquired autoinflammatory syndromes which present in adult life include Schnitzler syndrome and adult Still disease; the differential diagnosis includes urticarial vasculitis.

show abstract

Schnitzler's syndrome: diagnosis, treatment, and follow‐up

Cited by 224 publications

References 46 publications

Schnitzler syndrome: validation and applicability of diagnostic criteria in real-life patients

Schnitzler syndrome: validation and applicability of diagnostic criteria in real-life patients

Different Therapeutic Paths (Colchicine vs. Anakinra) in Two Patients With Schnitzler’s Syndrome

Autoinflammatory Diseases Presenting in the Skin

Contact Info

Product

Resources

About