scholarly journals Schnitzler’s Syndrome: A Diagnostic Consideration in Evaluating the Constellation of Monoclonal Gammopathy and Chronic Urticaria

2021 ◽  
Vol 10 (3) ◽  
pp. 143-146
Author(s):  
Jyothika Mamadgi ◽  
Laila Babar ◽  
Rama Bhagavatula ◽  
Santhosh Sadashiv ◽  
Kossivi Dantey ◽  
...  
Keyword(s):  
Chronic Urticaria ◽  
Monoclonal Gammopathy ◽  
Schnitzler’S Syndrome

scholarly journals A case of Schnitzler’s syndrome without monoclonal gammopathy successfully treated with canakinumab

2021 ◽  
Vol 22 (1) ◽  
Author(s):  
Yuya Fujita ◽  
Tomoyuki Asano ◽  
Akira Sakai ◽  
Natsumi Norikawa ◽  
Toshiyuki Yamamoto ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Chronic Urticaria ◽  
Monoclonal Gammopathy ◽  
Bone Pain ◽  
Periodic Fever ◽  
Interleukin 1 ◽  
Clinical Manifestations ◽  
Magnetic Resonance Imaging Examination ◽  
Amyloid A ◽  
Schnitzler’S Syndrome

Abstract Background Schnitzler’s syndrome (SchS) is a rare autoinflammatory syndrome with diagnostic challenge and be characterized by chronic urticaria, a monoclonal gammopath, periodic fever and bone pain. In addition to the monoclonal gammopathy, bone abnormalities are often found at the site of bone pain in patients with SchS. The remarkable efficacy of interleukin-1 (IL-1) inhibition was also demonstrated in this syndrome. Case presentation We describe a case of refractory chronic urticaria presenting with clinical manifestations consistent with SchS without monoclonal gammopathy. A 43-year-old female patient suffering from recurring of urticaria with periodic fever as well as bone pain for the past 4 years. The patient had leukocytosis and elevated levels of C-reactive protein (CRP) and serum amyloid A (SAA). PET/CT (positron emission tomography/computed tomography) and MRI (magnetic resonance imaging) examination revealed hyper-metabolism areas in both femoral bone marrow. Although bone marrow histology revealed no abnormality, urticarial skin lesions shows neutrophilic infiltrations without evidence of vasculitis. We could not exclude the possibility of SchS. The patient had been treated with antihistamines, steroids, omarizumab, colchicine and cyclosporine A, no therapeutic effect was observed. She was started on canakinumab 150 mg subcutaneous injection with 4 weeks interval. Within 48 h after the first injection, the urticarial rash disappeared, and febrile attack and bone pain had not recurred. Elevated levels of serum CRP and SAA were normalized within a week after the first injection of canakinumab. Conclusions The current case suggests an important role for IL-1 as a mediator in the pathophysiology of SchS-like refractory urticaria with bine pain. It had been presumed that monoclonal gammopathy may not always present in SchS. It is important to avoid delay in diagnosis and initiation of proper treatment in SchS or autoinflammatory conditions resembling SchS.

scholarly journals A Case of Schnitzler's Syndrome without Monoclonal Gammopathy-Associated Chronic Urticaria Treated with Anakinra

2018 ◽  
Vol 59 (1) ◽  
pp. 154 ◽  
Author(s):  
Min Joo Ahn ◽  
Ji Eun Yu ◽  
Jiung Jeong ◽  
Da Woon Sim ◽  
Young-Il Koh
Keyword(s):  
Chronic Urticaria ◽  
Monoclonal Gammopathy ◽  
Schnitzler’S Syndrome

scholarly journals FRI0480 SCHNITZLER’S SYNDROME: DESCRIPTION OF AN ITALIAN MULTICENTER COHORT

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 837.1-837
Author(s):  
F. Crisafulli ◽  
P. Airò ◽  
F. Franceschini ◽  
A. Tincani ◽  
M. Frassi
Keyword(s):  
Monoclonal Gammopathy ◽  
Interleukin 1 ◽  
Diagnostic Delay ◽  
Injection Site Reaction ◽  
Lymphoproliferative Disease ◽  
Severe Allergic Reaction ◽  
Intermittent Fever ◽  
Multicenter Cohort ◽  
Laboratory Features ◽  
Schnitzler’S Syndrome

Background:Schnitzler’s syndrome is an autoinflammatory disease characterized by monoclonal gammopathy and recurrent episodes of urticaria accompanied by clinical and laboratory signs of acute inflammation. Although the exact pathogenic mechanisms have not been fully clarified, the role of Interleukin-1 seems to be central.Objectives:To describe clinical features and therapeutic approach in patients with Schnitzler’s Syndrome.Methods:Retrospective analysis of an Italian multicenter cohort. Data are expressed as the median (IQR).Results:The clinical data of 24 patients from 9 centers (median follow-up 6 years [2-10]; median age at diagnosis 56.5 years [51.25-64.25]) were collected. The median diagnostic delay was 2 years (0-10); the diagnosis was made consensually at the onset of symptoms in 4 cases. The main clinical and laboratory features are shown in Table 1. Therapeutic response was evaluable in 20 patients: all received corticosteroids (CS; 25mg/day [25-50]); in one case, a good clinical response was observed. Eight patients were initially treated with colchicine: in 3 cases it was effective in controlling symptoms and reducing the dose of CS; other 8 patients were treated with csDMARDs (n:1 [1-2]): only 1 patient had a good response to cyclosporin.Table 1.Clinical and laboratory featuresChronic Urticarial Rash, n (%)24/24 (100)Pruritus, n (%)17/24 (71)Intermittent fever, n (%)23/24 (96)Arthralgia/Arthritis, n (%)20/24 (83)Bone pain, n (%)8/24 (33)Weight loss, n (%)9/24 (38)Angioedema, n (%)4/24 (17)Lymphoadenopathy, n (%)7/24 (29)Hepatomegaly, n (%)3/24 (12)Splenomegaly, n (%)3/24 (12)Neuropathy, n (%)4/24 (17)Raised ESR or CRP, n (%)24/24 (100)Leukocytosis, n (%)17/24 (71)Anemia, n (%)9/24 (38)Monoclonal GammopathyIgG λ, n (%)5/22 (23)IgG κ, n (%)6/22 (27)IgM λ, n (%)1/22 (5)IgM κ, n (%)12/22 (55)Bence Jones Protein, n (%)6/23 (26)A bDMARD was initiated in 15 patients. In 7 of the 14 patients initially treated with anakinra this therapy was continued with benefit whereas in the other 7 patients the treatment was discontinued for primary inefficacy (1 patient), secondary inefficacy (3 patients) and adverse events (3 patients; 2 injection site reaction, 1 severe allergic reaction). After anakinra discontinuation, 5 patients were treated with canakinumab with a good response in 3 cases and a partial response in 1 case (persistent arthritis); 1 patient died during the treatment. No response was observed in 3 patients treated with TNF inhibitors as a 2ndor 3rdline bDMARDs, as well as in 1 case initially treated with tocilizumab (in which a good response was afterwards obtained with canakinumab). bDMARDs were associated with a csDMARD in 2 patients (methotrexate and methotrexate + cyclosporine).In one case monoclonal gammopathy evolved into Multiple Myeloma and the patient died 15 years after the onset of symptoms. Idiopathic myelofibrosis and myelodysplasia were found in one and in two patients, respectively.Conclusion:In most cases csDMARDs and bDMARDs like anti-IL6 and anti-TNFα were not able to control the disease. In contrast, in some cases, a good response to colchicine was observed; refractory patients may be successfully treated with anti-IL1 agents. Patients should be supervised for possible evolution towards lymphoproliferative disease.Disclosure of Interests:None declared

scholarly journals Schnitzler's Syndrome without Monoclonal Gammopathy

2005 ◽  
Vol -1 (1) ◽  
pp. 1-1 ◽  
Author(s):  
Tatiana Cristina Nogueira Varella ◽  
Marcel Yossuke Nishimura ◽  
Maria Cecília Rivitti Machado ◽  
Dewton de Moraes-Vasconcelos ◽  
Evandro Ararigboia Rivitti
Keyword(s):  
Monoclonal Gammopathy ◽  
Schnitzler’S Syndrome

The Schnitzler syndrome: Chronic urticaria and monoclonal gammopathy - an autoinflammatory syndrome?

2008 ◽  
Vol 6 (8) ◽  
pp. 626-631 ◽  
Author(s):  
Elisabeth Eiling ◽  
Johann O. Schröder ◽  
Wolfgang L. Gross ◽  
Inga Kreiselmaier ◽  
Ulrich Mrowietz ◽  
...  
Keyword(s):  
Chronic Urticaria ◽  
Monoclonal Gammopathy ◽  
Autoinflammatory Syndrome ◽  
Schnitzler Syndrome
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