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

Barešić, Marko; Mitrović, Joško; Vergles, Jadranka Morović; Anić, Branimir

doi:10.5606/archrheumatol.2016.6000

Cited by 5 publications

(6 citation statements)

References 9 publications

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“…This emphasizes the importance of increasing physicians' awareness of this syndrome, mostly because of its potential morbidity, but also because effective treatment strategies are available, with proven safety and efficacy. 12…”

Section: Discussionmentioning

confidence: 99%

Malignant Evolution of Schnitzler Syndrome to Waldenström Macroglobulinemia: a Case Report

Man

László

Horváth

et al. 2022

Journal of Interdisciplinary Medicine

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Introduction: Schnitzler syndrome (SchS), first described in 1972, is a rare autoinflammatory condition characterized by chronic urticaria and monoclonal gammopathy of IgM or, exceptionally, IgG profile. Additional features include recurrent fever, evidence of abnormal bone remodeling, a neutrophilic dermal infiltrate on skin biopsy, leukocytosis or elevated C-reactive protein, according to the Strasbourg criteria. Case Presentation: We describe the case of a 56-year-old Caucasian male patient, who suffered from chronic urticaria, moderate-grade fever, severe generalized fatigue and arthralgias. After five years of chronic disease evolution, he was referred to the hematology department where he was found to have IgM kappa light chain monoclonal gammopathy. The constellation of symptoms, a negative rheumatologic workup, and the finding of IgM monoclonal gammopathy determined the diagnosis of Schnitzler syndrome. Bone marrow biopsy proved the association of Waldenström macroglobulinemia. Conclusion: The main goal of our case report was to highlight the clinical features and treatment, with emphasis on the hematological aspects, to provide a better understanding and to raise awareness of Schnitzler syndrome among healthcare professionals.

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Section: Discussionmentioning

confidence: 99%

Malignant Evolution of Schnitzler Syndrome to Waldenström Macroglobulinemia: a Case Report

Man

László

Horváth

et al. 2022

Journal of Interdisciplinary Medicine

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“…For patients with moderate to severe symptoms and highly elevated inflammatory markers treatment with anakinra, a recombinant IL-1 antagonist is recommended [ 1 - 3 , 6 ]. Anakinra produces dramatic clinical response in all the patients with Schnitzler’s syndrome [ 7 ].…”

Section: Discussionmentioning

confidence: 99%

“…Schnitzler’s syndrome carries a good prognosis. However, 10-20% of the cases can develop a hematologic malignancy including Waldenstrom’s macroglobulinemia, IgM multiple myeloma or non-Hodgkin lymphoma [ 1 - 3 , 8 , 10 ]. The mean duration of development of this complication is about 8 years since the onset of symptoms.…”

Section: Discussionmentioning

confidence: 99%

“…Interestingly, it is noted that anakinra offsets the development of these lymphoproliferative disorders. AA amyloidosis can develop in untreated cases [ 2 , 3 , 6 , 8 ].…”

Section: Discussionmentioning

confidence: 99%

“…Schnitzler’s syndrome affects adults usually in their 50s [ 1 , 2 ]. The diagnosis is difficult to establish and is often delayed, with an average time to diagnosis being approximately 5 years [ 3 ], adding significant morbidity to the affected patients. Multiple attempts have been made to simplify the diagnostic process.…”

Section: Introductionmentioning

confidence: 99%

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

Mamadgi¹,

Babar²,

Bhagavatula³

et al. 2021

J Hematol

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Schnitzler’s syndrome is a rare clinical entity characterized by intermittent, non-pruritic urticarial rash, fevers, arthralgias, myalgias and monoclonal gammopathy, most commonly of the immunoglobulin M (IgM) subtype. Schnitzler’s syndrome should be considered in the differential diagnosis of fever of unknown origin. We report a case of a 56-year-old healthy Caucasian female, who initially presented to the primary care physician’s office with complaints of severe generalized fatigue and myalgias involving thighs and calves. Patient subsequently underwent extensive rheumatologic workup, and was treated with multiple courses of steroids with temporary resolution of symptoms. During the course of her workup she was found to have IgM kappa monoclonal gammopathy, and was referred to hematology for further evaluation. The constellation findings of fever, arthralgias, chronic intermittent non-pruritic urticaria, myalgias, and a negative rheumatologic workup in the presence of IgM monoclonal gammopathy raised the suspicion of Schnitzler’s syndrome. Following completion of additional workup, she was started on anakinra 100 mg daily with prompt resolution of her symptoms. Due to the rarity of the disease, the diagnosis of Schnitzler’s syndrome is often delayed, with an average time to diagnosis being approximately 5 years. The symptoms in most cases can be debilitating and add to significant morbidity as noted in our patient, who required bilateral hip arthroplasty at a much younger age than expected. Published reports discuss the poor quality of life associated with the delayed diagnosis and unawareness of potential end organ damage. With our case report we like to highlight the disease characteristics for an early identification to prevent further organ damage believed to be from chronic inflammation. Early diagnosis and treatment with agents such as interleukin-1 (IL-1) inhibitors can promptly provide symptomatic relief, reduce inflammation and prevent organ damage.

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Off-Label studies on anakinra in dermatology: a review

Tegtmeyer

Atassi

Zhao

et al. 2020

Journal of Dermatological Treatment

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

Cited by 5 publications

References 9 publications

Malignant Evolution of Schnitzler Syndrome to Waldenström Macroglobulinemia: a Case Report

Malignant Evolution of Schnitzler Syndrome to Waldenström Macroglobulinemia: a Case Report

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

Off-Label studies on anakinra in dermatology: a review

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