Myopathy Associated With Antibodies to Signal Recognition Particle: Disease Progression and Neurological Outcome | Global Health | JAMA Neurology | JAMA Network
[Skip to Navigation]
Access to paid content on this site is currently suspended due to excessive activity being detected from your IP address Please contact the publisher to request reinstatement.
Reeves WH, Nigam SK, Blobel G. Human autoantibodies reactive with the signal-recognition particle.  Proc Natl Acad Sci U S A. 1986;83(24):9507-95112432596PubMedGoogle ScholarCrossref
Targoff IN, Johnson AE, Miller FW. Antibody to signal recognition particle in polymyositis.  Arthritis Rheum. 1990;33(9):1361-13702403400PubMedGoogle ScholarCrossref
Miller T, Al-Lozi MT, Lopate G, Pestronk A. Myopathy with antibodies to the signal recognition particle: clinical and pathological features.  J Neurol Neurosurg Psychiatry. 2002;73(4):420-42812235311PubMedGoogle ScholarCrossref
Kao AH, Lacomis D, Lucas M, Fertig N, Oddis CV. Anti-signal recognition particle autoantibody in patients with and patients without idiopathic inflammatory myopathy.  Arthritis Rheum. 2004;50(1):209-21514730618PubMedGoogle ScholarCrossref
Hengstman GJ, ter Laak HJ, Vree Egberts WT,  et al.  Anti-signal recognition particle autoantibodies: marker of a necrotising myopathy.  Ann Rheum Dis. 2006;65(12):1635-163816679430PubMedGoogle ScholarCrossref
Takada T, Hirakata M, Suwa A,  et al.  Clinical and histopathological features of myopathies in Japanese patients with anti-SRP autoantibodies.  Mod Rheumatol. 2009;19(2):156-16419089533PubMedGoogle ScholarCrossref
Valiyil R, Casciola-Rosen L, Hong G, Mammen A, Christopher-Stine L. Rituximab therapy for myopathy associated with anti-signal recognition particle antibodies: a case series.  Arthritis Care Res (Hoboken). 2010;62(9):1328-133420506493PubMedGoogle ScholarCrossref
Dimitri D, Andre C, Roucoules J, Hosseini H, Humbel RL, Authier FJ. Myopathy associated with anti-signal recognition peptide antibodies: clinical heterogeneity contrasts with stereotyped histopathology.  Muscle Nerve. 2007;35(3):389-39517143889PubMedGoogle ScholarCrossref
Suzuki S, Satoh T, Sato S,  et al.  Clinical utility of anti-signal recognition particle antibody in the differential diagnosis of myopathies.  Rheumatology (Oxford). 2008;47(10):1539-154218687709PubMedGoogle ScholarCrossref
Suzuki S, Ohta M, Shimizu Y, Hayashi YK, Nishino I. Anti-signal recognition particle myopathy in the first decade of life.  Pediatr Neurol. 2011;45(2):114-11621763952PubMedGoogle ScholarCrossref
Okada N, Mimori T, Mukai R, Kashiwagi H, Hardin JA. Characterization of human autoantibodies that selectively precipitate the 7SL RNA component of the signal recognition particle.  J Immunol. 1987;138(10):3219-32232437184PubMedGoogle Scholar
van Swieten JC, Koudstaal PJ, Visser MC, Schouten HJ, van Gijn J. Interobserver agreement for the assessment of handicap in stroke patients.  Stroke. 1988;19(5):604-6073363593PubMedGoogle ScholarCrossref
Danieli MG, Calcabrini L, Calabrese V, Marchetti A, Logullo F, Gabrielli A. Intravenous immunoglobulin as add on treatment with mycophenolate mofetil in severe myositis.  Autoimmun Rev. 2009;9(2):124-12719386287PubMedGoogle ScholarCrossref
Benveniste O, Drouot L, Jouen F,  et al.  Correlation of anti-signal recognition particle autoantibody levels with creatine kinase activity in patients with necrotizing myopathy.  Arthritis Rheum. 2011;63(7):1961-197121400483PubMedGoogle ScholarCrossref
Satoh T, Okano T, Matsui T,  et al.  Novel autoantibodies against 7SL RNA in patients with polymyositis/dermatomyositis.  J Rheumatol. 2005;32(9):1727-173316142868PubMedGoogle Scholar
Hamaguchi Y, Kuwana M, Hoshino K,  et al.  Clinical correlations with dermatomyositis-specific autoantibodies in adult Japanese patients with dermatomyositis: a multicenter cross-sectional study.  Arch Dermatol. 2011;147(4):391-39821482889PubMedGoogle ScholarCrossref
Römisch K, Miller FW, Dobberstein B, High S. Human autoantibodies against the 54 kDa protein of the signal recognition particle block function at multiple stages.  Arthritis Res Ther. 2006;8(2):R3916469117PubMedGoogle ScholarCrossref
Arlet JB, Dimitri D, Pagnoux C,  et al.  Marked efficacy of a therapeutic strategy associating prednisone and plasma exchange followed by rituximab in two patients with refractory myopathy associated with antibodies to the signal recognition particle (SRP).  Neuromuscul Disord. 2006;16(5):334-33616616848PubMedGoogle ScholarCrossref
Original Contribution
June 2012

Myopathy Associated With Antibodies to Signal Recognition Particle: Disease Progression and Neurological Outcome

Author Affiliations

Author Affiliations: Department of Neurology (Drs S. Suzuki and N. Suzuki) and Division of Rheumatology, Department of Internal Medicine (Dr Kuwana), Keio University School of Medicine, and Department of Neuromuscular Research, National Institute of Neuroscience, National Center of Neurology and Psychiatry (Drs Hayashi, Tsuburaya, and Nishino), Tokyo, Japan.

Arch Neurol. 2012;69(6):728-732. doi:10.1001/archneurol.2011.1728

Objective To characterize the clinical course of myopathy associated with antibodies to signal recognition particle (SRP), or anti-SRP myopathy.

Design Case series.

Setting Keio University Hospitals and National Institute of Neuroscience, National Center of Neurology and Psychiatry, Tokyo, Japan.

Patients We reviewed clinical features of 27 patients with anti-SRP myopathy and analyzed disease progression and neurological outcome.

Main Outcome Measures Anti-SRP antibodies in serum were detected by RNA immunoprecipitation assay using extracts of K562 cells.

Results Of the 27 patients, 5 (19%) showed chronic progressive muscle weakness as well as atrophy of limbs and trunk muscles from a younger age with more severe neurological outcomes compared with the other 22 patients (81%) with the subacute form.

Conclusion A subset of patients with anti-SRP myopathy can show a chronic progressive form associated with severe clinical deficits.