26rhk143 10.3205/26rhk143 urn:nbn:de:0183-26rhk1431 Meeting Abstract Büschgens Büschgens Janina J

Uniklinik RWTH Aachen, Klinik für Kinder- und Jugendmedizin, Kinder-Rheumatologie, Aachen, Deutschland

author Häusler Häusler Martin M

Uniklinik RWTH Aachen, Klinik für Kinder- und Jugendmedizin, Neuropädiatrie und Sozialpädiatrie, Aachen, Deutschland

author Kontny Kontny Udo U

Uniklinik RWTH Aachen, Klinik für Kinder- und Jugendmedizin, Pädiatrische Hämatologie, Onkologie und Stammzelltransplantation, Aachen, Deutschland

author Moser Moser Olga O

Uniklinik RWTH Aachen, Klinik für Kinder- und Jugendmedizin, Pädiatrische Hämatologie, Onkologie und Stammzelltransplantation, Aachen, Deutschland

author Wagner Wagner Norbert N

Uniklinik RWTH Aachen, Klinik für Kinder- und Jugendmedizin, Kinder-Rheumatologie, Aachen, Deutschland

author Nolte Nolte Kay K

Uniklinik RWTH Aachen, Klinik für Neuropathologie, Aachen, Deutschland

author Weis Weis Joachim J

Uniklinik RWTH Aachen, Klinik für Neuropathologie, Aachen, Deutschland

author Tietz Tietz Eric E

Uniklinik RWTH Aachen, Klinik für Diagnostische und Interventionelle Radiologie, Aachen, Deutschland

author Tenbrock Tenbrock Klaus K

Uniklinik RWTH Aachen, Klinik für Kinder- und Jugendmedizin, Kinder-Rheumatologie, Aachen, Deutschland

author German Medical Science GMS Publishing House

Düsseldorf

610 20260909 engl This is an Open Access article distributed under the terms of the Creative Commons Attribution 4.0 License. Dieser Artikel ist ein Open-Access-Artikel und steht unter den Lizenzbedingungen der Creative Commons Attribution 4.0 License (Namensnennung). M0656 143 Deutsche Gesellschaft für Rheumatologie Deutsche Gesellschaft für Orthopädische Rheumatologie Gesellschaft für Kinder- und Jugendrheumatologie 54. Kongress der Deutschen Gesellschaft für Rheumatologie und Klinische Immunologie (DGRh), 36. Jahrestagung der Gesellschaft für Kinder- und Jugendrheumatologie (GKJR), 40. Jahrestagung der Deutschen Gesellschaft für Orthopädische Rheumatologie (DGORh) Deutscher Rheumatologiekongress 2026 Der besondere Fall Leipzig 20260909 20260912 FA.59 TextHistory: The previous history was blank except for two seizures, no pharmacological treatment was required. The patient has been living with a foster family since she was 10-month-old.Cardinal symptom by manifestation of disease: A 9-year-old female presented herself with persistent soreness in her limbs. The patient reported having trouble climbing stairs and changing her clothes due to difficulty raising her arms. The physical examination revealed severe muscle weakness without skin involvement. Strength and reflexes in the upper extremities were diminished.Diagnostics: Laboratory tests revealed elevated levels of Aspartate Aminotransferase 871 U/l, Alanine Aminotransferase 498 U/l, Creatine Kinase (CK) 19,619 U/l, CK-MB 2,052 U/l, Troponin T 5,487 pg/ml and Antinuclear Antibody (ANA) test 1:5,200 (positive: SS-A/Ro-52/Nucleosomen). The childhood myositis assessment scale (CMAS) was 3/52. Whole-body magnetic resonance imaging (MRI) demonstrated proximal muscle inflammation. A muscle biopsy showed signs of necrotizing myositis. Testing for myositis antibodies confirmed the presence of anti-signal recognition particle (anti-SRP). Further examinations including an electrocardiogram, echocardiography, diagnostics for infectious disease, electroencephalogram, MRI of the brain and computed tomography of the chest revealed no abnormalities.Therapy: Immune modulated therapy (IMT) was administered . Therapy included pulsed methylprednisolone in combination with intravenous immunoglobulin, methotrexate, mycophenolate mofetil, cyclophosphamide, rituximab and abatacept. IMT was accompanied by daily intensive physical therapy. Despite CK-level improvement, IMT failed to significantly improve muscle strength measured by weekly CMAS scores. CMAS level did not exceed 10/52. Thus, an immunoablation followed by an autologous stem cell transplant (ASCT) was considered and performed. An intensive rehabilitation was started promptly afterwards.Further course: Four weeks after ASCT a successful decline in disease activity was observed. The patient was walking with little support. Muscle strength continued to increase steadily. Full muscle strength was achieved one year after ASCT. ASCT induced the remission of the disease.Detection of anti-SRP led to the diagnosis of immune-mediated necrotizing myopathy (IMNM). IMNM is characterized by proximal muscle weakness and elevated levels of serum muscle enzymes. Anti-SRP associated myopathy is rare in children and often fails to respond to standard immunosuppression. Our case adds to the growing literature of ASCT in children with inflammatory myopathies.Disclosures: JB: None declared. Binns EL Moraitis E Maillard S Tansley S McHugh N Jacques TS Wedderburn LR Pilkington C Yasin SA Nistala K UK Juvenile Dermatomyositis Research Group (UK and Ireland) Effective induction therapy for anti-SRP associated myositis in childhood: A small case series and review of the literature 2017 Pediatr Rheumatol Online J 77 Binns EL, Moraitis E, Maillard S, Tansley S, McHugh N, Jacques TS, Wedderburn LR, Pilkington C, Yasin SA, Nistala K; UK Juvenile Dermatomyositis Research Group (UK and Ireland). Effective induction therapy for anti-SRP associated myositis in childhood: A small case series and review of the literature. Pediatr Rheumatol Online J. 2017 Oct 31;15(1):77. DOI: 10.1186/s12969-017-0205-x http://dx.doi.org/10.1186/s12969-017-0205-x 0 0 0 0