Abstract
ObjectiveTo report on the characteristics and long-term course of rheumatic manifestations in Schnitzler syndrome (SchS).MethodsA retrospective cohort study of patients with SchS followed between 2000 and 2020. Inclusion criteria included a diagnosis of SchS (Strasbourg criteria). All available bone scans were reviewed and scored according to the intensity and number of pathological sites. The scintigraphic score was compared with the clinical activity score, CRP level, and treatments.ResultsTwenty-five patients were included. Median age at diagnosis was 68 years. Eighty patients (72%) had SchS-related rheumatic pain. Most patients had a long-standing isolated rash before constitutional and/or rheumatic symptoms appeared. The monoclonal component level was usually very low (IgMκ in 22/25). Rheumatic pain predominated around the knees. Bone scans revealed abnormal tracer uptake in 15/18 (85%). The scintigraphic score correlated with clinical activity (r = 0.4, p < 0.02) and CRP level (r = 0.47, p < 0.01). The scintigraphic score was lower in patients receiving corticosteroids or IL1Ra (interleukin 1 receptor antagonist) than in untreated patients (median scores:2, 0, and 13, respectively; p < 0.05). Two patients developed Waldenström macroglobulinemia. Of the 22 surviving patients, median age at follow-up was 76 years. IL1Ra was used in 13 patients, with dramatic efficacy on both symptoms and bone scan features.ConclusionsRheumatic manifestations are very prevalent in SchS. However, bone pain can be misleading and contribute to misdiagnosis. Bone scan abnormalities are very prevalent and correlate with disease activity and treatments. IL1-Ra has a dramatic and durable efficacy but may not be required in every patient early on.
Highlights
Schnitzler’s syndrome (SchS) is a rare adult-onset inflammatory disease first described in 1972 by Liliane Schnitzler, a French dermatologist [1, 2]
SchS is characterized by the association of urticarial rash, monoclonal gammopathy, and a variable combination of constitutional symptoms, bone pain, osteosclerosis, and/or elevated inflammatory markers (erythrocyte sedimentation rate (ESR) or C-reactive protein (CRP))
Most patients (n = 21/25, 83%) had a long-lasting, recurrent, or chronic rash frequently labeled as chronic spontaneous urticaria, before other symptoms of the disease developed
Summary
Schnitzler’s syndrome (SchS) is a rare adult-onset inflammatory disease first described in 1972 by Liliane Schnitzler, a French dermatologist [1, 2]. SchS is characterized by the association of urticarial rash, monoclonal gammopathy (overwhelmingly IgMκ), and a variable combination of constitutional symptoms (fever, fatigue, weight loss), bone pain, osteosclerosis, and/or elevated inflammatory markers (erythrocyte sedimentation rate (ESR) or C-reactive protein (CRP)). IL-1 blockade (mostly with anakinra, offlabel) have proved remarkably effective. It is only suspensive [3,4,5]. Diagnostic criteria were proposed in 2001 by Lipsker et al, with an urticarial rash and IgM component as 2 mandatory criteria [6]. The same group proposed and validated the so-called Strasbourg criteria, distinguishing patients with definite and probable SchS [7]
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