Plasma-exchange as a "rescue therapy" for dermato/polymyositis in acute phase. Experience in three young patients.
There are few data in the literature supporting the efficacy of plasma-exchange in dermato/polymyositis. The authors report three cases of patients with acute disease phase showing severe pharyngo-esophageal muscle weakness unresponsive to conventional therapy (corticosteroids and immunosuppressant agents) who were treated with plasma-exchange. As the patients were at high risk of "aspiration pneumonia", tracheostomy and PEG tubes were placed. The patients underwent a series of plasma-exchange for a mean of 15 weeks, during which time they progressively recovered muscle strength, their serum muscle enzyme values returned to normal levels, and MRI showed resolution of muscle edema. The tracheostomy and PEG tubes could be removed. Our findings suggest that plasma-exchange in association with immunosuppressant agents could play a relevant role in the management of dermato/polymyositis in acute phase.
Rheumatology Unit, Department of Medicine - DIMED, University of Padova, Italy. Electronic address: franco.cozzi@unipd.it.
Franco
F
Cozzi
Apheresis Unit, University Hospital of Padova, Italy.
Piero
P
Marson
Rheumatology Unit, Department of Medicine - DIMED, University of Padova, Italy.
Erika
E
Pigatto
Apheresis Unit, University Hospital of Padova, Italy.
Tiziana
T
Tison
Rheumatology Unit, Department of Medicine - DIMED, University of Padova, Italy.
Pamela
P
Polito
Rheumatology Unit, Department of Medicine - DIMED, University of Padova, Italy.
Paola
P
Galozzi
Apheresis Unit, University Hospital of Padova, Italy.
Giustina
G
De Silvestro
Rheumatology Unit, Department of Medicine - DIMED, University of Padova, Italy.
Leonardo
L
Punzi
D000293:Adolescent; D000328:Adult; D003882:Dermatomyositis / Q000097:blood / Q000503:physiopathology / Q000628:therapy*; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage*; D008297:Male; D009124:Muscle Proteins / Q000097:blood; D010951:Plasma Exchange
D007166:Immunosuppressive Agents; D009124:Muscle Proteins
Acute phase; Dermato/polymyositis; Plasma-exchange; Tracheostomy/PEG tubes