Increasing the Interval of Canakinumab Administration Effectively Supports the Remission of Schnitzler’s Syndrome

Schnitzler’s syndrome (SchS) is a rare, disabling, autoinflammatory disorder characterized by recurrent urticarial rash and monoclonal IgM gammopathy. Interleukin-1 beta (IL-1β) plays an important role in the pathophysiology of SchS. Only anecdotal reports demonstrate the efficiency and safety of human monoclonal anti-human IL-1β antibody (canakinumab) use in SchS therapy. However, there are no generally accepted recommendations concerning the scheme (or frequency) of canakinumab use for this disease. Here, we report the effective long-term treatment of SchS in a 44-year-old male with a standard canakinumab dose (150 mg) but with an increased 4-month injection interval.

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Effect of long-term treatment with platelet-activating factor (PAF-acether) on interleukin-1 (IL1) and interleukin-2 (IL2) production in the rat

Prostaglandins ◽

10.1016/0090-6980(87)90193-6 ◽

1987 ◽

Vol 34 (2) ◽

pp. 150 ◽

Cited By ~ 4

Author(s):

B. Pignol ◽

S. Hénane ◽

B. Sorlin ◽

J.M. Mencia-Huerta ◽

P. Braquet

Keyword(s):

Interleukin 1 ◽

Interleukin 2 ◽

Platelet Activating Factor ◽

Term Treatment ◽

Long Term Treatment

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

Clinical Rheumatology ◽

10.1007/s10067-020-05501-w ◽

2020 ◽

Author(s):

Riccardo Bixio ◽

Maurizio Rossini ◽

Alessandro Giollo

Keyword(s):

Complete Remission ◽

Inflammatory Markers ◽

Monoclonal Gammopathy ◽

Interleukin 1 ◽

Autoinflammatory Disorder ◽

Urticarial Rash ◽

Schnitzler’S Syndrome ◽

Inflammatory Drugs ◽

Case Based

Abstract 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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Resolution of femoral metaphyseal dysplasia in CINCA syndrome after long-term treatment with interleukin-1 blockade

Clinical Rheumatology ◽

10.1007/s10067-018-4145-8 ◽

2018 ◽

Vol 37 (7) ◽

pp. 2007-2009 ◽

Cited By ~ 1

Author(s):

Donato Rigante ◽

Raffaele Manna ◽

Elena Verrecchia ◽

Raffaella Marrocco ◽

Antonio Leone

Keyword(s):

Interleukin 1 ◽

Term Treatment ◽

Metaphyseal Dysplasia ◽

Long Term Treatment ◽

Cinca Syndrome

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Nonlinear Changes in Botulinum Toxin Treatment of Task-Specific Dystonia during Long-Term Treatment

Toxins ◽

10.3390/toxins13060371 ◽

2021 ◽

Vol 13 (6) ◽

pp. 371

Author(s):

André Lee ◽

Jabreel Al-Sarea ◽

Eckart Altenmüller

Keyword(s):

Botulinum Toxin ◽

Standard Treatment ◽

Term Treatment ◽

Long Term Treatment ◽

Median Split ◽

Injection Interval ◽

Botulinum Toxin Treatment ◽

Post Hoc ◽

First Time

Botulinum toxin (BoTX) is the standard treatment for task-specific dystonias (TSDs) such as musician’s dystonia (MD). Our aim was to assess the long-term changes in BoTX treatment in a highly homogeneous and, to our knowledge, largest group of MD patients with respect to the following parameters: (1) absolute and (2) relative BoTX dosage, (3) number of treated muscles, and (4) inter-injection interval. We retrospectively assessed a treatment period of 20 years in 233 patients, who had received a cumulative dose of 68,540 MU of BoTX in 1819 treatment sessions, performed by two neurologists. Nonlinear correlation was used to analyze changes in the parameters over the course of repeated treatments. Post-hoc we applied a median-split to classify two subgroups (high-BoTX, low-BoTX) depending on the total amount of BoTX needed during treatment. Across all patients, we found a decrease of dosage for the first approximately 25 treatments with an increase afterwards. The number of muscles and inter-injection intervals increased with time with a discrete decrease of inter-injection intervals after about 35 treatments. Subgroup differences were observed in the amount of BoTX and inter-injection intervals, with continuously increasing inter-injection intervals and decreasing BoTX dosage in the low-BTX group. Both groups showed a continuously increasing number of injected muscles. In summary, we found nonlinear changes of BoTX dosage and inter-injection intervals and a continuously increasing number of injected muscles with treatment duration in TSD-patients. Furthermore, we, for the first time, identified two subgroups with distinct differences. Increasing inter-injection intervals and decreasing BoTX dosages in the low-BoTX group indicated improvement of symptoms with continued treatment. Continually increasing BoTX dosages with unchanged inter-injection intervals in the high-BoTX group indicated deterioration.

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