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1.
J Clin Med ; 9(6)2020 Jun 25.
Article in English | MEDLINE | ID: mdl-32630482

ABSTRACT

Immune thrombocytopenia (ITP) is a rare autoimmune disease due to autoantibodies targeting platelet glycoproteins (GP). The mechanism of platelet destruction could differ depending on the specificity of antiplatelet antibodies: anti-GPIIb/IIIa antibodies lead to phagocytosis by splenic macrophages, in a Fcγ receptor (FcγR)-dependent manner while anti-GPIb/IX antibodies induce platelet desialylation leading to their destruction by hepatocytes after binding to the Ashwell-Morell receptor, in a FcγR-independent manner. Considering the FcγR-dependent mechanism of action of intravenous immunoglobulins (IVIg), we assumed that the response to IVIg could be less efficient in the presence of anti-GPIb/IX antibodies. We conducted a multicentric, retrospective study including all adult ITP patients treated with IVIg who had antiplatelet antibodies detected between January 2013 and October 2017. Among the 609 identified, 69 patients were included: 17 had anti-GPIb/IX antibodies and 33 had anti-GPIIb/IIIa antibodies. The response to IVIg was not different between the patients with or without anti-GPIb/IX (88.2% vs. 73.1%). The response to IVIg was better in the case of newly diagnosed ITP (odds ratio (OR) = 5.4 (1.2-24.7)) and in presence of anti-GPIIb/IIIa (OR = 4.82 (1.08-21.5)), while secondary ITP had a poor response (OR = 0.1 (0.02-0.64)). In clinical practice, the determination of antiplatelet antibodies is therefore of little value to predict the response to IVIg.

2.
J Rheumatol ; 46(11): 1535-1539, 2019 11.
Article in English | MEDLINE | ID: mdl-30877203

ABSTRACT

OBJECTIVE: To describe a new autoinflammatory syndrome with recurrent fever and monoclonal gammopathy that differs from Schnitzler syndrome. METHODS: We conducted a retrospective study of patients with monoclonal gammopathy and recurrent fever of unknown origin. RESULTS: Five patients were studied; median age at onset of symptoms was 44 years. Median frequency of fever attacks was 6 episodes per year. In the absence of treatment, the median duration of fevers was 3 days. CONCLUSION: This new autoinflammatory syndrome is defined by an association among monoclonal gammopathy, arthralgias, and recurrent fever.


Subject(s)
Arthralgia/complications , Fever/complications , Schnitzler Syndrome/complications , Adult , Aged , Antibodies, Monoclonal/blood , Arthralgia/drug therapy , C-Reactive Protein/analysis , Female , Fever/drug therapy , Follow-Up Studies , Humans , Immunoglobulin G/blood , Immunoglobulin M/blood , Interleukin 1 Receptor Antagonist Protein/therapeutic use , Male , Middle Aged , Recurrence , Retrospective Studies , Schnitzler Syndrome/drug therapy , Syndrome , Treatment Outcome
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