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 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.

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

Aseptic Suppurative Meningitis in Juvenile Boxer Dogs: Retrospective Study of 12 Cases

2006 ◽  
Vol 42 (4) ◽  
pp. 277-282 ◽  
Author(s):  
Sebastien Behr ◽  
Laurent Cauzinille
Keyword(s):  
Complete Resolution ◽  
Clinical Presentation ◽  
Immunosuppressive Treatment ◽  
Clinical Signs ◽  
Central Nervous System Inflammation ◽  
Immune Mediated ◽  
Long Term Follow Up ◽  
Boxer Dogs

Immune-mediated central nervous system inflammation is described in a series of 12 juvenile boxer dogs. A diagnosis of steroid-responsive meningitis-arteritis was made based on the clinical presentation and on diagnostic findings. The boxer breed was at a higher risk for this inflammatory condition than other breeds. Long-term follow-up (>2 years) confirmed a better prognosis in this breed than in the beagle and the Bernese mountain dog. Complete resolution of clinical signs without significant deficits or recurrences was obtained in all cases. Early clinical recognition and immunosuppressive treatment resulted in a better response and complete resolution of the disorder.

scholarly journals Successful treatment and long-term follow up of idiopathic immune-mediated polyarthritis with mycophenolate mofetil in a cat

2020 ◽  
Vol 6 (2) ◽  
pp. 205511692096399
Author(s):  
Yu Tamura ◽  
Tetsuro Nagamoto ◽  
Kazuhito Segawa ◽  
Sakurako Neo ◽  
Hirotaka Igarashi ◽  
...  
Keyword(s):  
Mycophenolate Mofetil ◽  
Synovial Fluid ◽  
Successful Treatment ◽  
Clinical Signs ◽  
Limited Information ◽  
Amyloid A ◽  
Immune Mediated ◽  
Long Term Follow Up

Case summary A 7-year-old neutered male Norwegian Forest Cat was presented with decreased appetite and activity, weight loss, fever, neutrophilia and hyperglobulinaemia. A physical examination showed painful stifle joints and enlarged popliteal lymph nodes. Blood examination showed neutrophilia, hyperglobulinaemia and increased serum amyloid A. Urinalysis, thoracic and abdominal radiographs, and abdominal ultrasonography were unremarkable. Synovial fluid from the knee joints had diminished viscosity and revealed neutrophilic inflammation on the smear. There was no evidence of infection in a microbiological culture of the synovial fluid. A diagnosis of idiopathic immune-mediated polyarthritis (IMPA) was made. Prednisolone was initiated at 2 mg/kg q24h PO and tapered with additional immunosuppressants (leflunomide, ciclosporin A and methotrexate); however, prednisolone could not be discontinued. Informed consent was obtained from the owner and mycophenolate mofetil (MMF) at a dosage of 10 mg/kg q12h PO was initiated on day 798. There were no adverse effects of MMF and prednisolone was discontinued on day 1183. Clinical signs resolved and the cat’s general condition remained stable with MMF alone at a dosage of 10 mg/kg q48h PO on day 1600. Relevance and novel information There is limited information describing feline IMPA and its treatment options other than the use of prednisolone. This is the first report of the successful treatment and long-term follow-up of feline IMPA with MMF. MMF may be a safe and effective option as an additional immunosuppressant in feline IMPA.

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