Immunoglobulin Therapy in Muscle Diseases
and Myasthenia
Massimiliano Filosto
Clinica Neurologica
Centro per lo Studio delle Malattie Neuromuscolari e delle Neuropatie
Azienda Ospedaliera “Spedali Civili” ed Università degli Studi di Brescia
Immunomodulatory action of IVIg
in neuromuscular diseases
Dalakas, 2004)
PATOLOGIA
PATOLOGIA
Dermatomyositis
Mononuclear cell inflammation:
Stains for CD4 and, in some regions,
CD20 (B-cells)
Commonly perivascular.
Necrotic capillary
PATOLOGIA
Dermatomyositis
PATOLOGIA
Dermatomyositis
Dalakas, 1993
PATOLOGIA
Dermatomyositis
Continue…
PATOLOGIA
Dermatomyositis
GENES
DM
IBM
Chemokine
Mig/CXCL9
Anosmin1/KAL-1
Dalakas, 2004
PATOLOGIA
Polymyositis
PATOLOGIA
Polymyositis
No specific polymyositis RCT are currently available.
Many case reports.
Some DM/PM studies.
PATOLOGIA
Myasthenia Gravis
- Changes in serum anti Ach-R ab
- Effective dosage
- Anti MUSK and serum negative patients
Five trials: worsening
Two trials :chronic
PATOLOGIA
Myasthenia Gravis: IV Ig maintenance therapy
Comparison of Plasmapheresis and Intravenous Immunoglobulin
as Maintenance Therapies for Juvenile Myasthenia Gravis
Wendy K. M. Liew, MBChB, MRCPCH1; Christine A. Powell, MA2;
Steven R. Sloan, MD, PhD3,4; Robert C. Shamberger, MD5;
Christopher B. Weldon, MD, PhD5; Basil T. Darras, MD1; Peter
B. Kang, MD1,6
[+] Author Affiliations
.
JAMA Neurol. 2014;71(5):575-580. doi:10.1001/jamaneurol.2014.17
Conclusions and Relevance This study provides
class III evidence that PLEX and IVIG both have
high response rates as maintenance therapies and
are reasonable therapeutic options for juvenile MG.
Plasmapheresis may have a more consistent
response rate than IVIG in this setting. These
findings will provide some guidance regarding the
approach to therapy for juvenile MG, especially as
the results differ somewhat from those of studies
.
focusing on adult MG
PATOLOGIA
Predictors of response
Factors determining response to intravenous immunoglobulin (IVIg) and plasmapheresis in myasthenia gravis (MG)
have not been evaluated systematically.
Lambert-Eaton Syndrome
Post-polio syndrome
Subcutaneous Ig
PRESENTAZIONE
CLINICA
PRESENTAZIONE
CLINICA
Subcutaneous Ig
PRESENTAZIONE
CLINICA
2 casi post-polio trattati off-label con Ig e.v.
–
–
Paziente numero 1: miglioramento dell’ipostenia
con persistenza dell’efficacia nei successivi tre mesi,
successiva ricomparsa dell’ipostenia. Efficacia
duratura in cronico.
Paziente numero 2: nessuna efficacia
6 casi di Miastenia poco responsiva in terapia
cronica con Ig e.v.
•
In tutti i casi, persistente beneficio con
intervallo di somministrazione variabile tra 2 e
3 mesi
Aknowledgment
University Hospital «Spedali Civili», Brescia
Alice Todeschini
Elisa Pari
Fabrizio Rinaldi
Silvia Rota
Anna Galvagni
Irene Volonghi
Alessandro Padovani