Diagnostic value of 18-F fluorodeoxyglucose PET/CT and bone scan in Schnitzler syndrome

Alix, L.; Néel, A.; Cador, B.; Смаил, А.; Serratrice, Jacques; Closs-Prophette, F.; Jégo, Patrick; Devillers, Anne; Decaux, Olivier

doi:10.1080/08916934.2019.1680649

Cited by 10 publications

(11 citation statements)

References 23 publications

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“…Moreover, bone scans may be well positioned to distinguish SchS relapse from other aetiologies of bone, joint, or muscle pain. 22 In our case, the scintigraphy showed an increased radiotracer uptake, which correlated with bone pain, but the alkaline phosphatase was normal. Urticaria is a common symptom in SchS and has to be differentiated from chronic spontaneous urticaria (CSU).…”

Section: Dovepressmentioning

confidence: 42%

<p>Schnitzler Syndrome in a 27-Year-Old Man: Diagnostic and Therapeutic Dilemma in Adult Auto-Inflammatory Syndromes A Case Report and Literature Review</p>

Więsik-Szewczyk

Felis-Giemza

Dziuk

et al. 2020

IJGM

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A 32-year-old-man, with a history of chronic urticaria from the age of 27, diagnosed with an adult-onset Still's disease and received a low dose of glucocorticoids, methotrexate and tocilizumab. Despite the long-term combined treatments, he suffered from chronic urticaria, lowgrade fever and bone pain. He was found to have high inflammatory markers, hypogammaglobulinemia, monoclonal IgM-kappa light chain in serum and increased radiotracer uptake in the whole bone scintigraphy. No pathological variants for monogenic autoinflammatory diseases were present in the genome exome sequencing. These investigations confirmed the diagnosis of Schnitzler syndrome, which is an exception before the age of 35. Switching from tocilizumab to interleukin 1 receptor inhibitor, anakinra led to a full clinical response and normalisation of inflammatory markers. Patients with a history of fever and chronic urticaria are routinely tested for monoclonal gammopathy in the context of malignancy, but it should also be considered as a sign of the autoinflammatory syndrome. The Schnitzler syndrome and the adult-onset Still's disease share common features, so the diagnosis requires a thorough investigation to establish an optimal treatment. In the diagnostic algorithm, monoclonal gammopathy is usually considered red flag for malignancy but might be overlooked as a criterion of Schnitzler syndrome, particularly in young adults. We confirm that the interleukin 1 inhibitor should be the first line of therapy in Schnitzler syndrome, and in the presented case we found it more effective than the interleukin 6 blockade. The main goal of this paper is to increase awareness of Schnitzler syndrome among health care professionals. We aim to present features which can be helpful in differential diagnosis.

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Section: Dovepressmentioning

confidence: 42%

<p>Schnitzler Syndrome in a 27-Year-Old Man: Diagnostic and Therapeutic Dilemma in Adult Auto-Inflammatory Syndromes A Case Report and Literature Review</p>

Więsik-Szewczyk

Felis-Giemza

Dziuk

et al. 2020

IJGM

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“…Increased radiotracer uptake appeared to be more sensitive in detecting lower extremity inflammation than PET/CT. Moreover, radiotracer uptake in bone scan correlated with disease activity, but PET/CT did not appear to be useful for diagnosis or disease activity follow-up [21] . The diagnostic value of bone scan and correlation with disease activity of Schnitzler syndrome were also confirmed by another study of 25 cases [22] .…”

Section: Classical Schnitzler Syndromementioning

confidence: 94%

Schnitzler syndrome and Schnitzler-like syndromes

Chu

2022

Chinese Medical Journal

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Schnitzler syndrome is a rare disease of adult-onset with main features including chronic urticarial rash, recurrent fever, arthralgia or arthritis, monoclonal gammopathy of undetermined significance (MGUS), and marked systemic inflammation. Schnitzler syndrome is often underdiagnosed. Patients with Schnitzler syndrome may present to dermatologists and allergists for urticaria, hematologists for MGUS, or rheumatologists for arthritis. It is important to recognize Schnitzler syndrome for its remarkable response to interleukin (IL)-1 blockade. Besides, many cases of Schnitzler-like syndromes do not meet the diagnostic criteria of classical Schnitzler syndrome but display excellent response to IL-1 inhibitors. The overly produced IL-1 is the result of a somatic mosaic gain of function mutation of NLRP3 (nucleotide-binding oligomerization domain [NOD]-like receptor [NLR] family pyrin domain containing 3) gene in some patients with Schnitzler-like syndromes. Inflammasome activation is evident in patients with classical Schnitzler syndrome although no NLRP3 gene mutation is identified. Collectively, Schnitzler syndrome and Schnitzler-like syndromes represent a spectrum of IL-1 mediated adult-onset autoinflammatory diseases.

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“…We performed a retrospective study of 6 patients (5 men, 1 woman) diagnosed with Schnitzler´s syndrome fulfilling the Strasbourg criteria who had been treated at our centre in the University Hospital Brno from 2007 to 2021. Median age at diagnosis was 54 (45-67) years, median follow up was 8 (3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14) years. All 6 patients had IgM κ monoclonal gammopathy, increased CRP and/or erythrocyte sedimentation rate and arthralgia or bone pain, 4 patients suffered from fever, three had leucocytosis ≥ 10 × 10 9 /L and lymphadenopathy was found in one patient.…”

Section: Transformation Of Igm-mgus Into Waldenström´s Macroglobulinemia In Two Of Six Patients Treated For Schnitzler´s Syndromementioning

confidence: 99%

“…Medián věku pacientů (5 mužů 1 žena) v době stanovení diagnózy byl 54 (45-67) let. Medián sledování je 8 (3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14) roků. Všech šest pacientů mělo M-IgM, bolesti kloubů anebo bolesti kostí, zvýšenou hodnotu sedimentace erytrocytů a CRP, čtyři měli horečky, tři měli leukocytózu ≥ 10 × 10 9 /l, lymfadenopatii měl jen jeden pacient.…”

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“…VNITŘNÍ LÉKAŘSTVÍ / Vnitř Lék 2021; 67(e3): e15-e23 / www.casopisvnitrnilekarstvi.cz Dnes se do popředí zájmu dostává PET/CT vyšetření. Ve studii 10 pacientů bylo nejčastější anomálií difuzně zvýšené vychytávání18 FDG v kostní dřeni a/nebo zvýšené vychytávání18 FDG ve femurech, ale nepozorovali korelaci s léčebnou odpovědí, zatímco výsledky kostní scintigrafie korelovaly aktivitou nemoci(14). Je to vysvětlitelné faktem, že18 FDG-PET/CT, které bylo použito v této práci, odráží aktivitu buněčného metabolismu, zatímco scintigrafie skeletu odráží míru vychytávání kalcia ve skeletu.U našich 5 pacientů bylo také provedeno18 FDG-PET/CT…”

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Transformation of IgM-MGUS into Waldenström´s macroglobulinemia in two of six patients treated for Schnitzler´s syndrome

Adam¹,

Tomíška²,

Řehák³

et al. 2021

Vnitř Lék

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Diagnostic value of 18-F fluorodeoxyglucose PET/CT and bone scan in Schnitzler syndrome

Cited by 10 publications

References 23 publications

<p>Schnitzler Syndrome in a 27-Year-Old Man: Diagnostic and Therapeutic Dilemma in Adult Auto-Inflammatory Syndromes A Case Report and Literature Review</p>

<p>Schnitzler Syndrome in a 27-Year-Old Man: Diagnostic and Therapeutic Dilemma in Adult Auto-Inflammatory Syndromes A Case Report and Literature Review</p>

Schnitzler syndrome and Schnitzler-like syndromes

Transformation of IgM-MGUS into Waldenström´s macroglobulinemia in two of six patients treated for Schnitzler´s syndrome

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