Childhood autoimmune necrotizing myopathy with anti-signal recognition particle antibodies.

Abstract:

INTRODUCTION:The aim of this study was to describe the therapeutic effects of immunomodulatory therapy in 3 patients with childhood autoimmune necrotizing myopathy with anti-signal recognition particle antibodies (SRP-ANM). METHODS:Before treatment, data on clinical features, muscle pathology, and thigh MRIs were obtained. After definitive diagnoses, all 3 patients were treated with intravenous immunoglobulin and corticosteroids, and thigh MRIs were performed. RESULTS:Clinical improvements were associated with declines in serum creatine kinase levels. Pretreatment thigh MRIs revealed diffuse, but uneven or focal edema, mostly in the posterior thigh muscles, which was alleviated after immunomodulatory therapy. DISCUSSION:Childhood SRP-ANM responded well to immunomodulatory therapy. The extent of edema, as monitored by thigh MRI, appears to be a useful marker of disease severity and therapeutic benefit. Muscle Nerve 56: 1181-1187, 2017.

journal_name

Muscle Nerve

journal_title

Muscle & nerve

authors

Zhao Y,Liu X,Zhang W,Yuan Y

doi

10.1002/mus.25575

subject

Has Abstract

pub_date

2017-12-01 00:00:00

pages

1181-1187

issue

6

eissn

0148-639X

issn

1097-4598

journal_volume

56

pub_type

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