Immune-mediated rippling muscle disease with myasthenia gravis: A report of seven patients with long-term follow-up in two

2009 ◽  
Vol 19 (3) ◽  
pp. 223-228 ◽  
Author(s):  
Benedikt Schoser ◽  
Saiju Jacob ◽  
David Hilton-Jones ◽  
Wolfgang Müller-Felber ◽  
Christian Kubisch ◽  
...  
Keyword(s):  
Myasthenia Gravis ◽  
Muscle Disease ◽  
Immune Mediated ◽  
Long Term Follow Up

scholarly journals Immune-mediated monofocal motor neuropathy: Report of two cases with long-term follow up and review of six published cases

2014 ◽  
Vol 2 (3) ◽  
pp. 76-81
Author(s):  
Bamidele O Adeyemo ◽  
James Andriotakis ◽  
Michael Dreyer ◽  
Pantelis P Pavlakis ◽  
Peter Siao
Keyword(s):  
Motor Neuropathy ◽  
Immune Mediated ◽  
Long Term Follow Up

scholarly journals Open ADAMTS13, induced by antibodies, is a biomarker for subclinical immune-mediated thrombotic thrombocytopenic purpura

Blood ◽  
2020 ◽  
Author(s):  
Elien Roose ◽  
An-Sofie Schelpe ◽  
Edwige Tellier ◽  
György Sinkovits ◽  
Bérangère S Joly ◽  
...  
Keyword(s):  
Thrombotic Thrombocytopenic Purpura ◽  
Adamts13 Activity ◽  
Thrombocytopenic Purpura ◽  
Follow Up Study ◽  
Open Conformation ◽  
Immune Mediated ◽  
Long Term Follow Up ◽  
Donor Plasma

Recently, we showed that during the acute phase of immune-mediated thrombotic thrombocytopenic purpura (iTTP), ADAMTS13 circulates in an open conformation. Although the cause of this conformational change in acute iTTP remains elusive, ADAMTS13 is mainly closed in iTTP patients (i) in remission with an ADAMTS13 activity >50% and undetectable anti-ADAMTS13 autoantibodies, and (ii) after rituximab treatment, suggesting a role for anti-ADAMTS13 autoantibodies. Therefore, IgGs from 18 acute iTTP patients were purified and added to closed ADAMTS13 in healthy donor plasma. This resulted in open ADAMTS13 in 14/18 (78%) samples, proving that indeed anti-ADAMTS13 autoantibodies can induce an open ADAMTS13 conformation. To further elucidate the conformation of ADAMTS13 in iTTP patients, we studied a novel iTTP patient cohort (n=197) that also included plasma samples of iTTP patients in remission where ADAMTS13 activity was <50%. The open ADAMTS13 conformation was not only found during acute iTTP but also in patients in remission with an ADAMTS13 activity <50% and in half of the patients with an ADAMTS13 activity >50%, although free anti-ADAMTS13 autoantibodies were not always detected. Thus open ADAMTS13 is not only a hallmark of acute iTTP, but also a novel biomarker to detect subclinical iTTP in patients in remission. Finally, a long term follow-up study in one iTTP patient showed that the open conformation precedes a severe drop in ADAMTS13 activity. In conclusion, we have shown that anti-ADAMTS13 autoantibodies from iTTP patients induce an open ADAMTS13 conformation. Most importantly, an open ADAMTS13 conformation is a biomarker for subclinical iTTP and could become an important tool in TTP management.

scholarly journals Myasthenia gravis and thymus: long-term follow-up screening of thymectomized and non-thymectomized patients

2013 ◽  
Vol 71 (7) ◽  
pp. 462-464 ◽  
Author(s):  
Paulo José Lorenzoni ◽  
Lucas Pires Augusto ◽  
Cláudia Suemi Kamoi Kay ◽  
Rosana Herminia Scola ◽  
Lineu Cesar Werneck
Keyword(s):  
Myasthenia Gravis ◽  
Recurrence Rate ◽  
Progressive Increase ◽  
Clinical Deterioration ◽  
Initial Screening ◽  
Long Term Follow Up ◽  
Study Support ◽  
Anti Acetylcholine

Thymoma screening is recommended at the onset of myasthenia gravis (MG) or when patients with MG present with clinical deterioration or a progressive increase of anti-acetylcholine receptor antibody. However, it is unknown if it is necessary to repeat the screening of thymoma at fixed intervals, even in the absence of MG deterioration, when the initial screening is negative. We analyzed the recurrence rate and incidence of new thymoma in a series of patients with well-controlled MG. The sample consisted of 53 patients, aged 17 to 72 years, and the follow-up varied between 75 and 472 months. The chest computerized tomography detected thymus abnormalities in eight patients at the initial screening and no abnormalities in all patients at a second screening after five years. The findings of this study support the classical opinion that screening for thymoma should be recommended only if there is clinical deterioration due to the disease.

scholarly journals Preemptive rituximab prevents long-term relapses in immune-mediated thrombotic thrombocytopenic purpura

Blood ◽  
2018 ◽  
Vol 132 (20) ◽  
pp. 2143-2153 ◽  
Author(s):  
Matthieu Jestin ◽  
Ygal Benhamou ◽  
An-Sofie Schelpe ◽  
Elien Roose ◽  
François Provôt ◽  
...  
Keyword(s):  
Thrombotic Thrombocytopenic Purpura ◽  
Relapse Rate ◽  
Thrombocytopenic Purpura ◽  
Immune Mediated ◽  
Key Points ◽  
Long Term Follow Up ◽  
Adamts13 Deficiency ◽  
Risk Balance

Key Points TTP patients who display persistent and severe ADAMTS13 deficiency after remission have a relapse rate of 74% during long-term follow-up. Preemptive rituximab can decrease TTP relapses in 85% of patients with a favorable benefit-risk balance.

Long-Term Follow-Up After Robotic Thymectomy for Nonthymomatous Myasthenia Gravis

2011 ◽  
Vol 92 (3) ◽  
pp. 1018-1023 ◽  
Author(s):  
Richard K. Freeman ◽  
Anthony J. Ascioti ◽  
Jaclyn M. Van Woerkom ◽  
Amy Vyverberg ◽  
Robert J. Robison
Keyword(s):  
Myasthenia Gravis ◽  
Long Term Follow Up ◽  
Robotic Thymectomy

scholarly journals An Ocular Form of Myasthenia Gravis with a High Titer of Anti-MuSK Antibodies during a Long-term Follow-up

2012 ◽  
Vol 51 (21) ◽  
pp. 3077-3079 ◽  
Author(s):  
Ai Hosaka ◽  
Hiroshi Takuma ◽  
Kiyoe Ohta ◽  
Akira Tamaoka
Keyword(s):  
Myasthenia Gravis ◽  
High Titer ◽  
Long Term Follow Up

scholarly journals Long-Term Follow-Up of Strabismus Surgery for Patients With Ocular Myasthenia Gravis

2013 ◽  
Vol 33 (1) ◽  
pp. 40-44 ◽  
Author(s):  
Jason H. Peragallo ◽  
Federico G. Velez ◽  
Joseph L. Demer ◽  
Stacy L. Pineles
Keyword(s):  
Myasthenia Gravis ◽  
Strabismus Surgery ◽  
Ocular Myasthenia ◽  
Ocular Myasthenia Gravis ◽  
Long Term Follow Up
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