Treatment response to cyclophosphamide, rituximab, and bortezomib in chronic immune-mediated sensorimotor neuropathies: a retrospective cohort study
Background: Up to 20% of patients with chronic immune-mediated sensorimotor neuropathies (CIN) do not respond adequately to first-line therapies. However, studies on further treatment are scarce. Methods: We analyzed retrospectively 200 CIN patients regarding disease characteristics and response to...
| Main Authors: | , , , , , , , , , , , , , |
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| Format: | Article |
| Language: | English |
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SAGE Publishing
2021-03-01
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| Series: | Therapeutic Advances in Neurological Disorders |
| Online Access: | https://doi.org/10.1177/1756286421999631 |
| _version_ | 1819141350403080192 |
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| author | Jeremias Motte Anna Lena Fisse Nuray Köse Thomas Grüter Hannah Mork Diamantis Athanasopoulos Miriam Fels Susanne Otto Ines Siglienti Christiane Schneider-Gold Kerstin Hellwig Min-Suk Yoon Ralf Gold Kalliopi Pitarokoili |
| author_facet | Jeremias Motte Anna Lena Fisse Nuray Köse Thomas Grüter Hannah Mork Diamantis Athanasopoulos Miriam Fels Susanne Otto Ines Siglienti Christiane Schneider-Gold Kerstin Hellwig Min-Suk Yoon Ralf Gold Kalliopi Pitarokoili |
| author_sort | Jeremias Motte |
| collection | DOAJ |
| description | Background: Up to 20% of patients with chronic immune-mediated sensorimotor neuropathies (CIN) do not respond adequately to first-line therapies. However, studies on further treatment are scarce. Methods: We analyzed retrospectively 200 CIN patients regarding disease characteristics and response to therapy with cyclophosphamide (CYP), rituximab (RTX), and bortezomib (BTZ). Treatment response was defined as improvement or stabilization of inflammatory neuropathy cause and treatment overall disability score (INCAT-ODSS). Results: A total of 48 of 181 patients (26.5%) received therapy with CYP, RTX, or BTZ. The most frequently and first used therapy was CYP (69%). More than 40% of patients needed a second or third treatment. Overall, 71 treatments were applied in 48 patients. The combination of up to all three treatments enhanced the response-rate to 90%. Treatment within 24 months after initial diagnosis resulted in significantly higher response rate than late treatment (79% versus 50 %, p = 0.04, χ 2 -test, n = 46) and in lower disability in long-term follow up (INCAT-ODSS 3.8 versus 5.8, p = 0.02, t -test, n = 48). Patients with Lewis-Sumner syndrome ( n = 9) and autoantibody mediated neuropathies ( n = 13) had excellent response rates after treatment with RTX (90–100%). In contrast, typical chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) showed a response rate of 64% in CYP, 64% in RTX, and 75% in BTZ. Conclusion: Treatment with CYP, RTX, or BTZ was effective in this cohort of CIN refractory to first-line treatment. Our data increase evidence for an early use of these therapies. High efficacy of RTX in Lewis-Sumner syndrome in contrast to typical CIDP suggests a distinct pathophysiology. |
| first_indexed | 2024-12-22T11:53:03Z |
| format | Article |
| id | doaj.art-2623d273ea6145de97fa1f67d8e85186 |
| institution | Directory Open Access Journal |
| issn | 1756-2864 |
| language | English |
| last_indexed | 2024-12-22T11:53:03Z |
| publishDate | 2021-03-01 |
| publisher | SAGE Publishing |
| record_format | Article |
| series | Therapeutic Advances in Neurological Disorders |
| spelling | doaj.art-2623d273ea6145de97fa1f67d8e851862022-12-21T18:26:55ZengSAGE PublishingTherapeutic Advances in Neurological Disorders1756-28642021-03-011410.1177/1756286421999631Treatment response to cyclophosphamide, rituximab, and bortezomib in chronic immune-mediated sensorimotor neuropathies: a retrospective cohort studyJeremias MotteAnna Lena FisseNuray KöseThomas GrüterHannah MorkDiamantis AthanasopoulosMiriam FelsSusanne OttoInes SiglientiChristiane Schneider-GoldKerstin HellwigMin-Suk YoonRalf GoldKalliopi PitarokoiliBackground: Up to 20% of patients with chronic immune-mediated sensorimotor neuropathies (CIN) do not respond adequately to first-line therapies. However, studies on further treatment are scarce. Methods: We analyzed retrospectively 200 CIN patients regarding disease characteristics and response to therapy with cyclophosphamide (CYP), rituximab (RTX), and bortezomib (BTZ). Treatment response was defined as improvement or stabilization of inflammatory neuropathy cause and treatment overall disability score (INCAT-ODSS). Results: A total of 48 of 181 patients (26.5%) received therapy with CYP, RTX, or BTZ. The most frequently and first used therapy was CYP (69%). More than 40% of patients needed a second or third treatment. Overall, 71 treatments were applied in 48 patients. The combination of up to all three treatments enhanced the response-rate to 90%. Treatment within 24 months after initial diagnosis resulted in significantly higher response rate than late treatment (79% versus 50 %, p = 0.04, χ 2 -test, n = 46) and in lower disability in long-term follow up (INCAT-ODSS 3.8 versus 5.8, p = 0.02, t -test, n = 48). Patients with Lewis-Sumner syndrome ( n = 9) and autoantibody mediated neuropathies ( n = 13) had excellent response rates after treatment with RTX (90–100%). In contrast, typical chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) showed a response rate of 64% in CYP, 64% in RTX, and 75% in BTZ. Conclusion: Treatment with CYP, RTX, or BTZ was effective in this cohort of CIN refractory to first-line treatment. Our data increase evidence for an early use of these therapies. High efficacy of RTX in Lewis-Sumner syndrome in contrast to typical CIDP suggests a distinct pathophysiology.https://doi.org/10.1177/1756286421999631 |
| spellingShingle | Jeremias Motte Anna Lena Fisse Nuray Köse Thomas Grüter Hannah Mork Diamantis Athanasopoulos Miriam Fels Susanne Otto Ines Siglienti Christiane Schneider-Gold Kerstin Hellwig Min-Suk Yoon Ralf Gold Kalliopi Pitarokoili Treatment response to cyclophosphamide, rituximab, and bortezomib in chronic immune-mediated sensorimotor neuropathies: a retrospective cohort study Therapeutic Advances in Neurological Disorders |
| title | Treatment response to cyclophosphamide, rituximab, and bortezomib in chronic immune-mediated sensorimotor neuropathies: a retrospective cohort study |
| title_full | Treatment response to cyclophosphamide, rituximab, and bortezomib in chronic immune-mediated sensorimotor neuropathies: a retrospective cohort study |
| title_fullStr | Treatment response to cyclophosphamide, rituximab, and bortezomib in chronic immune-mediated sensorimotor neuropathies: a retrospective cohort study |
| title_full_unstemmed | Treatment response to cyclophosphamide, rituximab, and bortezomib in chronic immune-mediated sensorimotor neuropathies: a retrospective cohort study |
| title_short | Treatment response to cyclophosphamide, rituximab, and bortezomib in chronic immune-mediated sensorimotor neuropathies: a retrospective cohort study |
| title_sort | treatment response to cyclophosphamide rituximab and bortezomib in chronic immune mediated sensorimotor neuropathies a retrospective cohort study |
| url | https://doi.org/10.1177/1756286421999631 |
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