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

Ahn, Min Joo; Yu, Ji Eun; Jeong, Ji-Ung; Sim, Da Woon; Koh, Young Il

doi:10.3349/ymj.2018.59.1.154

Cited by 9 publications

(4 citation statements)

References 13 publications

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“…A few cases have shown that monoclonal is not detected in the early stage of this syndrome [ 14 ]. However, monoclonal gammopathy has been reported to be still absent during the entire disease course [ 15 ]. The presence of monoclonal IgM is a mandatory criterion for diagnosing Schnitzler’s syndrome [ 3 ]; however, not all patients had monoclonal IgM at the initiation stage of SchS [ 4 ].…”

Section: Discussionmentioning

confidence: 99%

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

Fujita

Asano

Sakai

et al. 2021

BMC Musculoskelet Disord

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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.

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Section: Discussionmentioning

confidence: 99%

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

Fujita

Asano

Sakai

et al. 2021

BMC Musculoskelet Disord

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“…Schnitzler`s syndrome without IgG or IgM gammopathy described as Schnitzler-like syndrome in the literature is a potentially underdiagnosed entity (13)(14)(15). But the spectrum of Schnitzler-like syndromes may be not restricted to autoinflammatory symptoms with urticarial rash lacking gammopathy (20).…”

Section: Discussionmentioning

confidence: 99%

Case report: Schnitzler-like syndrome without monoclonal gammopathy

et al. 2023

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Schnitzler syndrome is a rare autoinflammatory disorder characterized by urticarial rash, joint pain, recurrent fever, leucocytosis, elevated C-reactive protein (CRP) and serum amyloid A (SAA), and monoclonal IgM or IgG gammopathy. According to the Strasbourg criteria, both urticarial rash and gammopathy are mandatorily required for the diagnosis of Schnitzler’s syndrome. However, incomplete variants lacking either skin symptoms or monoclonal gammopathy have also been described. Here, we report a case in which the diagnosis of Schnitzler-like syndrome was made despite the absence of gammopathy, based on neutrophilic dermal inflammation, episodic and excessive increase in inflammatory parameters, and prompt response to anakinra, a soluble IL1 receptor antagonist (sIL-1RA). In addition, we detected neutrophil epitheliotropism, which is highly suggestive of autoinflammatory disease. Using whole-exome sequencing, we were unable to find a causative pathogenic mutation but did find several mutations possibly related to the inflammatory processes in this patient. This and other cases highlight that the existing Strasbourg criteria are too strict to capture Schnitzler-like syndromes that may respond well and rapidly to IL1 inhibition. Recurrent episodes of disease with normalization of inflammatory symptoms in the interval, rapid response to anakinra, and neutrophilic epitheliotropism in a lesional skin biopsy may help confirm the diagnosis of Schnitzler-like syndrome.

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“…However, to our knowledge, this is the first report of SchS without monoclonal gammopathy in Italy. We retrieved only 7 further case reports of patients diagnosed with SchS without monoclonal gammopathy [10][11][12][13][14][15].…”

Section: Discussionmentioning

confidence: 99%

Efficacy of interleukin-1 blockade in Schnitzler’s syndrome without detectable monoclonal gammopathy: a case-based review

2020

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Schnitzler’s syndrome (SchS) is a rare autoinflammatory disorder characterized by urticarial rash and monoclonal gammopathy which is currently regarded as IL-1 mediated disease. We present the case of a 21-year-old woman presenting with urticarial rash, arthralgias, and elevated inflammatory markers. She has been suffering these symptoms for 2 years and was treated with antihistamines, omalizumab, steroids, and non-steroidal anti-inflammatory drugs (NSAIDs) without success. After an extensive diagnostic workout, we suspected SchS even without monoclonal gammopathy, and started Anakinra 100 mg daily with a dramatic response and achieving complete remission after 48 h of the beginning of the treatment, so we decided to confirm SchS diagnosis. We performed a search of the literature and found seven more cases of patients diagnosed with SchS without monoclonal gammopathy at the presentation. Five were treated with IL-1 blocking therapies and all achieved remission. We, therefore, prompt the possible role of IL-1 blockade therapy remission as support in diagnosing SchS without monoclonal gammopathy.

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

Cited by 9 publications

References 13 publications

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

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

Case report: Schnitzler-like syndrome without monoclonal gammopathy

Efficacy of interleukin-1 blockade in Schnitzler’s syndrome without detectable monoclonal gammopathy: a case-based review

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