Anti-SRP myositis: a diagnostic and therapeutic challenge
Background. Anti-signal recognition protein (anti-SRP) myopathy is a rare idiopathic inflammatory myopathy in children. Herein, a 3-year-old patient with severe anti-SRP myopathy showing a rapidly progressive disease course is presented in order to increase the awareness of pediatricians about idio...
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| Format: | Article |
| Language: | English |
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Hacettepe University Institute of Child Health
2024-12-01
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| Series: | The Turkish Journal of Pediatrics |
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| Online Access: | https://turkjpediatr.org/article/view/4916 |
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| author | Merve Cansu Polat Didem Ardıçlı Banu Çelikel Acar Beril Talim Nesrin Şenbil Elif Çelikel |
| author_facet | Merve Cansu Polat Didem Ardıçlı Banu Çelikel Acar Beril Talim Nesrin Şenbil Elif Çelikel |
| author_sort | Merve Cansu Polat |
| collection | DOAJ |
| description |
Background. Anti-signal recognition protein (anti-SRP) myopathy is a rare idiopathic inflammatory myopathy in children. Herein, a 3-year-old patient with severe anti-SRP myopathy showing a rapidly progressive disease course is presented in order to increase the awareness of pediatricians about idiopathic inflammatory myopathies.
Case Presentation. A previously healthy 3-year-old girl presented with progressive symmetrical proximal muscle weakness that caused difficulty in climbing stairs for two months prior to evaluation, and a marked elevation of the serum creatine kinase levels. A skeletal muscle biopsy revealed necrotic and regenerating processes, with mild inflammatory changes. Myositis-specific and associated autoantibodies tested by the immunoblot method were positive for anti-SRP. Pulse corticosteroid, intravenous immunoglobulin, and methotrexate were administered. However, muscle weakness progressed, respiratory distress and dysphagia developed. Rituximab was initiated. While on rituximab treatment, she was able to walk independently and muscle enzymes were within normal range at the 15th month of diagnosis.
Conclusion. Early diagnosis of patients with anti-SRP myositis is important to control inflammation and prevent disease progression and complications. To our knowledge, our patient is the youngest case reported in the literature and was successfully treated with rituximab added to conventional therapy.
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| format | Article |
| id | doaj-art-d7d27c370b2345a8ae54bffa8fcf3cfd |
| institution | OA Journals |
| issn | 0041-4301 2791-6421 |
| language | English |
| publishDate | 2024-12-01 |
| publisher | Hacettepe University Institute of Child Health |
| record_format | Article |
| series | The Turkish Journal of Pediatrics |
| spelling | doaj-art-d7d27c370b2345a8ae54bffa8fcf3cfd2025-08-20T02:01:50ZengHacettepe University Institute of Child HealthThe Turkish Journal of Pediatrics0041-43012791-64212024-12-0166610.24953/turkjpediatr.2024.4916Anti-SRP myositis: a diagnostic and therapeutic challengeMerve Cansu Polat0https://orcid.org/0000-0003-3279-8435Didem Ardıçlı1https://orcid.org/0000-0001-7054-3623Banu Çelikel Acar2https://orcid.org/0000-0002-1808-3655Beril Talim3https://orcid.org/0000-0003-2375-7543Nesrin Şenbil4https://orcid.org/0000-0002-4143-3553Elif Çelikel5https://orcid.org/0000-0003-0129-4410Division of Pediatric Rheumatology, Department of Pediatrics, University of Health Sciences, Ankara Bilkent City Hospital, Ankara, TürkiyeDivision of Pediatric Neurology, Department of Pediatrics, University of Health Sciences, Ankara Bilkent City Hospital, Ankara, TürkiyeDivision of Pediatric Rheumatology, Department of Pediatrics, University of Health Sciences, Ankara Bilkent City Hospital, Ankara, TürkiyePediatric Pathology Unit, Department of Pediatrics, Hacettepe University Faculty of Medicine, Ankara, TürkiyeDivision of Pediatric Neurology, Department of Pediatrics, Kırıkkale University Faculty of Medicine, Kırıkkale, TürkiyeDivision of Pediatric Rheumatology, Department of Pediatrics, University of Health Sciences, Ankara Bilkent City Hospital, Ankara, Türkiye Background. Anti-signal recognition protein (anti-SRP) myopathy is a rare idiopathic inflammatory myopathy in children. Herein, a 3-year-old patient with severe anti-SRP myopathy showing a rapidly progressive disease course is presented in order to increase the awareness of pediatricians about idiopathic inflammatory myopathies. Case Presentation. A previously healthy 3-year-old girl presented with progressive symmetrical proximal muscle weakness that caused difficulty in climbing stairs for two months prior to evaluation, and a marked elevation of the serum creatine kinase levels. A skeletal muscle biopsy revealed necrotic and regenerating processes, with mild inflammatory changes. Myositis-specific and associated autoantibodies tested by the immunoblot method were positive for anti-SRP. Pulse corticosteroid, intravenous immunoglobulin, and methotrexate were administered. However, muscle weakness progressed, respiratory distress and dysphagia developed. Rituximab was initiated. While on rituximab treatment, she was able to walk independently and muscle enzymes were within normal range at the 15th month of diagnosis. Conclusion. Early diagnosis of patients with anti-SRP myositis is important to control inflammation and prevent disease progression and complications. To our knowledge, our patient is the youngest case reported in the literature and was successfully treated with rituximab added to conventional therapy. https://turkjpediatr.org/article/view/4916anti-signal recognition protein myopathyanti-SRPrituximabinflammatory myopathychildren |
| spellingShingle | Merve Cansu Polat Didem Ardıçlı Banu Çelikel Acar Beril Talim Nesrin Şenbil Elif Çelikel Anti-SRP myositis: a diagnostic and therapeutic challenge The Turkish Journal of Pediatrics anti-signal recognition protein myopathy anti-SRP rituximab inflammatory myopathy children |
| title | Anti-SRP myositis: a diagnostic and therapeutic challenge |
| title_full | Anti-SRP myositis: a diagnostic and therapeutic challenge |
| title_fullStr | Anti-SRP myositis: a diagnostic and therapeutic challenge |
| title_full_unstemmed | Anti-SRP myositis: a diagnostic and therapeutic challenge |
| title_short | Anti-SRP myositis: a diagnostic and therapeutic challenge |
| title_sort | anti srp myositis a diagnostic and therapeutic challenge |
| topic | anti-signal recognition protein myopathy anti-SRP rituximab inflammatory myopathy children |
| url | https://turkjpediatr.org/article/view/4916 |
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