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Primary progressive multiple sclerosis

MedGen UID:
155968
•Concept ID:
C0751964
•
Disease or Syndrome
Synonyms: Multiple Sclerosis, Primary Progressive; Primary Progressive Multiple Sclerosis
SNOMED CT: Primary progressive multiple sclerosis (428700003); Multiple sclerosis (MS) primary progressive (428700003)
 
Monarch Initiative: MONDO:0000451

Definition

A multiple sclerosis that is characterized by steady worsening of neurologic functioning, without any distinct relapses or periods of remission. The rate of progression may vary over time, with occasional plateaus or temporary improvements, but the progression is continuous. [from MONDO]

Professional guidelines

PubMed

Pathophysiology, Diagnosis, Treatment and Emerging Neurotherapeutic Targets for Progressive Multiple Sclerosis: The Age of PIRA.
Carlson AK, Fox RJ
Neurol Clin 2024 Feb;42(1):39-54. Epub 2023 Aug 21 doi: 10.1016/j.ncl.2023.07.002. PMID: 37980122
Progressive multiple sclerosis: from pathogenic mechanisms to treatment.
Correale J, Gaitán MI, Ysrraelit MC, Fiol MP
Brain 2017 Mar 1;140(3):527-546. doi: 10.1093/brain/aww258. PMID: 27794524
Classification, diagnosis, and differential diagnosis of multiple sclerosis.
Katz Sand I
Curr Opin Neurol 2015 Jun;28(3):193-205. doi: 10.1097/WCO.0000000000000206. PMID: 25887774

Curated

UK NICE Guideline NG220, Multiple sclerosis in adults: management, 2022

Recent clinical studies

Therapy

Real-world evaluation of ocrelizumab in multiple sclerosis: A systematic review.
Montalban X, Matthews PM, Simpson A, Petrie JL, Sammon C, Ramagopalan S, Disanto G, Kuhle J
Ann Clin Transl Neurol 2023 Mar;10(3):302-311. Epub 2023 Feb 2 doi: 10.1002/acn3.51732. PMID: 36728340Free PMC Article
Ocrelizumab: A Review in Multiple Sclerosis.
Lamb YN
Drugs 2022 Feb;82(3):323-334. Epub 2022 Feb 22 doi: 10.1007/s40265-022-01672-9. PMID: 35192158Free PMC Article
Multiple Sclerosis.
Olek MJ
Ann Intern Med 2021 Jun;174(6):ITC81-ITC96. Epub 2021 Jun 8 doi: 10.7326/AITC202106150. PMID: 34097429
Systematic Review of Safety and Efficacy of Rituximab in Treating Immune-Mediated Disorders.
Kaegi C, Wuest B, Schreiner J, Steiner UC, Vultaggio A, Matucci A, Crowley C, Boyman O
Front Immunol 2019;10:1990. Epub 2019 Sep 6 doi: 10.3389/fimmu.2019.01990. PMID: 31555262Free PMC Article
Ocrelizumab versus Placebo in Primary Progressive Multiple Sclerosis.
Montalban X, Hauser SL, Kappos L, Arnold DL, Bar-Or A, Comi G, de Seze J, Giovannoni G, Hartung HP, Hemmer B, Lublin F, Rammohan KW, Selmaj K, Traboulsee A, Sauter A, Masterman D, Fontoura P, Belachew S, Garren H, Mairon N, Chin P, Wolinsky JS; ORATORIO Clinical Investigators
N Engl J Med 2017 Jan 19;376(3):209-220. Epub 2016 Dec 21 doi: 10.1056/NEJMoa1606468. PMID: 28002688

Prognosis

Paraneoplastic/autoimmune myelopathies.
Montalvo M, Flanagan EP
Handb Clin Neurol 2024;200:193-201. doi: 10.1016/B978-0-12-823912-4.00017-7. PMID: 38494277
Blood neurofilament light levels predict non-relapsing progression following anti-CD20 therapy in relapsing and primary progressive multiple sclerosis: findings from the ocrelizumab randomised, double-blind phase 3 clinical trials.
Bar-Or A, Thanei GA, Harp C, Bernasconi C, Bonati U, Cross AH, Fischer S, Gaetano L, Hauser SL, Hendricks R, Kappos L, Kuhle J, Leppert D, Model F, Sauter A, Koendgen H, Jia X, Herman AE
EBioMedicine 2023 Jul;93:104662. Epub 2023 Jun 22 doi: 10.1016/j.ebiom.2023.104662. PMID: 37354600Free PMC Article
Patient selection for trials.
Wolinsky JS
Mult Scler 2017 Oct;23(12):1636-1641. doi: 10.1177/1352458517729458. PMID: 29041867
Multiple sclerosis - etiology and diagnostic potential.
Kamińska J, Koper OM, Piechal K, Kemona H
Postepy Hig Med Dosw (Online) 2017 Jun 30;71(0):551-563. doi: 10.5604/01.3001.0010.3836. PMID: 28665284
Rituximab in patients with primary progressive multiple sclerosis: results of a randomized double-blind placebo-controlled multicenter trial.
Hawker K, O'Connor P, Freedman MS, Calabresi PA, Antel J, Simon J, Hauser S, Waubant E, Vollmer T, Panitch H, Zhang J, Chin P, Smith CH; OLYMPUS trial group
Ann Neurol 2009 Oct;66(4):460-71. doi: 10.1002/ana.21867. PMID: 19847908

Clinical prediction guides

The effect of ibudilast on thalamic volume in progressive multiple sclerosis.
Nicholson S, Russo AW, Brewer K, Bien H, Tobyne SM, Eloyan A, Klawiter EC
Mult Scler 2023 Dec;29(14):1819-1830. Epub 2023 Nov 10 doi: 10.1177/13524585231204710. PMID: 37947294Free PMC Article
Effectiveness of Ocrelizumab in Primary Progressive Multiple Sclerosis: a Multicenter, Retrospective, Real-world Study (OPPORTUNITY).
Chisari CG, Bianco A, Brescia Morra V, Calabrese M, Capone F, Cavalla P, Chiavazza C, Comi C, Danni M, Filippi M, Iaffaldano P, Lanzillo R, Lo Fermo S, Lucisano A, Lugaresi A, Lus G, Marfia GA, Marinelli F, Mirabella M, Moiola L, Perin C, Realmuto S, Toscano S, Trojano M, Vecchio D, Patti F; Italian MS Registry
Neurotherapeutics 2023 Oct;20(6):1696-1706. Epub 2023 Aug 23 doi: 10.1007/s13311-023-01415-y. PMID: 37610702Free PMC Article
Blood neurofilament light levels predict non-relapsing progression following anti-CD20 therapy in relapsing and primary progressive multiple sclerosis: findings from the ocrelizumab randomised, double-blind phase 3 clinical trials.
Bar-Or A, Thanei GA, Harp C, Bernasconi C, Bonati U, Cross AH, Fischer S, Gaetano L, Hauser SL, Hendricks R, Kappos L, Kuhle J, Leppert D, Model F, Sauter A, Koendgen H, Jia X, Herman AE
EBioMedicine 2023 Jul;93:104662. Epub 2023 Jun 22 doi: 10.1016/j.ebiom.2023.104662. PMID: 37354600Free PMC Article
Ocrelizumab versus Placebo in Primary Progressive Multiple Sclerosis.
Montalban X, Hauser SL, Kappos L, Arnold DL, Bar-Or A, Comi G, de Seze J, Giovannoni G, Hartung HP, Hemmer B, Lublin F, Rammohan KW, Selmaj K, Traboulsee A, Sauter A, Masterman D, Fontoura P, Belachew S, Garren H, Mairon N, Chin P, Wolinsky JS; ORATORIO Clinical Investigators
N Engl J Med 2017 Jan 19;376(3):209-220. Epub 2016 Dec 21 doi: 10.1056/NEJMoa1606468. PMID: 28002688
Primary progressive multiple sclerosis.
Montalban X
Curr Opin Neurol 2005 Jun;18(3):261-6. doi: 10.1097/01.wco.0000169742.37159.e9. PMID: 15891409

Recent systematic reviews

Real-world evaluation of ocrelizumab in multiple sclerosis: A systematic review.
Montalban X, Matthews PM, Simpson A, Petrie JL, Sammon C, Ramagopalan S, Disanto G, Kuhle J
Ann Clin Transl Neurol 2023 Mar;10(3):302-311. Epub 2023 Feb 2 doi: 10.1002/acn3.51732. PMID: 36728340Free PMC Article
Ocrelizumab for multiple sclerosis.
Lin M, Zhang J, Zhang Y, Luo J, Shi S
Cochrane Database Syst Rev 2022 May 18;5(5):CD013247. doi: 10.1002/14651858.CD013247.pub2. PMID: 35583174Free PMC Article
Systematic Review of Safety and Efficacy of Second- and Third-Generation CD20-Targeting Biologics in Treating Immune-Mediated Disorders.
Kaegi C, Wuest B, Crowley C, Boyman O
Front Immunol 2021;12:788830. Epub 2022 Feb 2 doi: 10.3389/fimmu.2021.788830. PMID: 35185862Free PMC Article
Systematic Review of Safety and Efficacy of Rituximab in Treating Immune-Mediated Disorders.
Kaegi C, Wuest B, Schreiner J, Steiner UC, Vultaggio A, Matucci A, Crowley C, Boyman O
Front Immunol 2019;10:1990. Epub 2019 Sep 6 doi: 10.3389/fimmu.2019.01990. PMID: 31555262Free PMC Article
Consensus recommendations for the diagnosis and treatment of primary progressive multiple sclerosis in Latin America.
Cristiano E, Rojas JI, Abad P, Adoni T, Barahona J, Becker J, Carrá A, Flores J, Fruns M, Fernández Liguori N, Garcea O, García Bónito J, Giunta D, Gracia F, Hamuy F, Macias Islas MA, Navas C, Boschetti LO, Patrucco L, Sato DK, Correale J
J Neurol Sci 2018 Oct 15;393:4-13. Epub 2018 Jul 31 doi: 10.1016/j.jns.2018.07.024. PMID: 30096568

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    Clinical resources

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      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Curated

    • NICE, 2022
      UK NICE Guideline NG220, Multiple sclerosis in adults: management, 2022

    Consumer resources

    Reviews

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