Improvement of recurrent urticaria in a patient with Schnitzler syndrome associated with B-cell lymphoma with combination rituximab and radiotherapy

Murota, Hiroyuki; Shoda, Yukiko; Ishibashi, Tatsuro; Sugahara, Hiroyuki; Matsumura, Itaru; Katayama, Ichiro

doi:10.1016/j.jaad.2008.12.040

Cited by 18 publications

(14 citation statements)

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“…Thus, the question whether the Schnitzler syndrome is a smoldering lymphoproliferative disorder with systemic expression, comparable to the POEMS syndrome, or a systemic disorder with an accompanying mIgM remains open. In rare instances, treatment of the underlying lymphoproliferative disorder had a beneficial effect on the Schnitzler syndrome, supporting the former point of view [60]. …”

Section: Introductionmentioning

confidence: 64%

The Schnitzler syndrome

Lipsker

2010

Orphanet J Rare Dis

156

168

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The Schnitzler syndrome is a rare and underdiagnosed entity which is considered today as being a paradigm of an acquired/late onset auto-inflammatory disease. It associates a chronic urticarial skin rash, corresponding from the clinico-pathological viewpoint to a neutrophilic urticarial dermatosis, a monoclonal IgM component and at least 2 of the following signs: fever, joint and/or bone pain, enlarged lymph nodes, spleen and/or liver, increased ESR, increased neutrophil count, abnormal bone imaging findings. It is a chronic disease with only one known case of spontaneous remission. Except of the severe alteration of quality of life related mainly to the rash, fever and pain, complications include severe inflammatory anemia and AA amyloidosis. About 20% of patients will develop a lymphoproliferative disorder, mainly Waldenström disease and lymphoma, a percentage close to other patients with IgM MGUS. It was exceedingly difficult to treat patients with this syndrome until the IL-1 receptor antagonist anakinra became available. Anakinra allows a complete control of all signs within hours after the first injection, but patients need continuous treatment with daily injections.In many aspects, the Schnitzler syndrome resembles the genetically determined auto-inflammatory syndromes involving activating mutations of the NLRP3 inflammasome. This latter point and its consequences will be addressed.

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Section: Introductionmentioning

confidence: 64%

The Schnitzler syndrome

Lipsker

2010

Orphanet J Rare Dis

156

168

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“…The major complication of SchS is the development of a hematological malignancy. This was reported in 35 SchS patients at a median follow-up of 8 years after the onset of disease [ 3 , 4 , 8 , 19 , 21 , 26 , 34 , 36 , 40 , 53 , 61 , 63 , 64 , 72 , 91 , 97 , 99 , 108 , 112 , 131 , 133 , 138 , 141 ]. Two-thirds of these malignancies concerned WM, a lymphoplasmacytic lymphoma (Table 4 ).…”

Section: Reviewmentioning

confidence: 91%

Schnitzler's syndrome: lessons from 281 cases

Koning

2014

Clinical & Translational All

101

169

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Schnitzler’s syndrome is an autoinflammatory disorder characterized by the association of a monoclonal IgM (or IgG) gammopathy, a chronic urticarial rash, and signs and symptoms of systemic inflammation, including fever, arthralgias and bone pain. It was first described in 1972. This review summarizes the clinical features, efficacy of therapies, and follow-up data of the 281 cases that have been reported to date. Also, the results of skin histology, bone imaging, laboratory investigations, and studies of the pathogenesis will be discussed, including the pivotal role of interleukin-1 beta in this disorder.Electronic supplementary materialThe online version of this article (doi:10.1186/2045-7022-4-41) contains supplementary material, which is available to authorized users.

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“…This treatment was reported to improve renal insufficiency --a complication that is not usually part of Schnitzler's syndrome --while having a slight positive effect on urticarial rash and bone pain in one patient with Schnitzler's syndrome [37], and to partially improve skin manifestations in another patient with systemic B-cell marginal zone lymphoma associated with Schnitzler's syndrome [38]. Complete remission was achieved in two other patient without associated lymphoproliferative disorder [39,40], whereas an improvement in urticarial rash was observed in another patient but only when additional radiotherapy was performed on a retroperitoneal tumor suspected of lymphomatous origin [41]. Notably, a reduction in the serum level of the IgM gammopathy was concomitantly observed in all of these cases.…”

Section: Rituximabmentioning

confidence: 99%