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Autoimmune necrotizing myopathies (AINM) in adult patients are characterized by severity of muscle damage, presence of necrosis with little inflammation on muscle biopsy and anti-HMGCR or anti-SRP auto-antibodies. Data on AINM in children are currently lacking. The purpose of this study is to specify the characteristics at AINM diagnosis, treatments and evolution of juvenile AINM with anti-HMGCR or anti-SRP antibodies.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| autoimmune necrotizing myopathy beginning before 18 |
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| Measure | Description | Time Frame |
|---|---|---|
| Type of muscle involvment | Proximal or distal or axial muscle involvement by muscle testing | At diagnosis |
| Type of cutaneous involvment | Rash or Gottron lesions | At diagnosis |
| Other clinical manifestations | Dysphagia or dyspnea or fever or interstitial lung involvement or articular involvement or gastrointestinal involvment | At diagnosis |
| Blood CK level | Blood CK level | At diagnosis |
| Histology | Results of muscle biopsy : necrosis | At diagnosis |
| Histology | Results of muscle biopsy : inflammation | At diagnosis |
| MMT score | MMT score | At diagnosis |
| CMAS score | CMAS score | At diagnosis |
| Measure | Description | Time Frame |
|---|---|---|
| Type of drugs used | Drugs ever used : name and duration | At last visit |
| Iatrogenic complications | Infectious complications | At last visit |
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Inclusion Criteria:
Exclusion Criteria:
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AINM histologically proved (i.e. predominant muscle fibers necrosis lesions associated with minimal or absent inflammatory lesions), either anti-SRP or anti-HMGCR antibodies positivity and a disease onset before age 18
| Name | Role | Phone | Extension | |
|---|---|---|---|---|
| Irène LEMELLE | Contact | 03 83 15 45 96 | i.lemelle@chru-nancy.fr |
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| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Hopital d'enfants CHRU Nancy | Vandœuvre-lès-Nancy | 54 500 | France |
| PubMed Identifier | Type | Citation | Retractions |
|---|---|---|---|
| 27538058 | Background | Benveniste O, Stenzel W, Allenbach Y. Advances in serological diagnostics of inflammatory myopathies. Curr Opin Neurol. 2016 Oct;29(5):662-73. doi: 10.1097/WCO.0000000000000376. | |
| 30208379 | Background | Mariampillai K, Granger B, Amelin D, Guiguet M, Hachulla E, Maurier F, Meyer A, Tohme A, Charuel JL, Musset L, Allenbach Y, Benveniste O. Development of a New Classification System for Idiopathic Inflammatory Myopathies Based on Clinical Manifestations and Myositis-Specific Autoantibodies. JAMA Neurol. 2018 Dec 1;75(12):1528-1537. doi: 10.1001/jamaneurol.2018.2598. |
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| Iatrogenic complications | Bone complications | At last visit |
| Status of the disease at last visit | Status of the disease at last visit : active or remission or death | At last visit |
| Number of relapses | Number of relapses | At last visit |
| Type of muscle involvment | Proximal or distal or axial muscle involvement by muscle testing | At last visit |
| Type of cutaneous involvment | Rash or Gottron lesions | At last visit |
| Other clinical manifestations | Dysphagia or dyspnea or fever or interstitial lung involvement or articular involvement or gastrointestinal involvment | At last visit |
| Blood CK level | Blood CK level | At last visit |
| CMAS score | CMAS score | At last visit |
| MMT score | MMT score | At last visit |
| 27147697 | Background | Watanabe Y, Uruha A, Suzuki S, Nakahara J, Hamanaka K, Takayama K, Suzuki N, Nishino I. Clinical features and prognosis in anti-SRP and anti-HMGCR necrotising myopathy. J Neurol Neurosurg Psychiatry. 2016 Oct;87(10):1038-44. doi: 10.1136/jnnp-2016-313166. Epub 2016 May 4. |
| 24797170 | Background | Allenbach Y, Drouot L, Rigolet A, Charuel JL, Jouen F, Romero NB, Maisonobe T, Dubourg O, Behin A, Laforet P, Stojkovic T, Eymard B, Costedoat-Chalumeau N, Campana-Salort E, Tournadre A, Musset L, Bader-Meunier B, Kone-Paut I, Sibilia J, Servais L, Fain O, Larroche C, Diot E, Terrier B, De Paz R, Dossier A, Menard D, Morati C, Roux M, Ferrer X, Martinet J, Besnard S, Bellance R, Cacoub P, Arnaud L, Grosbois B, Herson S, Boyer O, Benveniste O; French Myositis Network. Anti-HMGCR autoantibodies in European patients with autoimmune necrotizing myopathies: inconstant exposure to statin. Medicine (Baltimore). 2014 May;93(3):150-157. doi: 10.1097/MD.0000000000000028. |
| 28864670 | Background | Anti-HMGCR Autoantibodies in Juvenile Idiopathic Inflammatory Myopathies Identify a Rare but Clinically Important Subset of Patients. J Rheumatol. 2017 Sep;44(9):1417. doi: 10.3899/jrheum.160871.C1. No abstract available. |
| 27818386 | Background | Liang WC, Uruha A, Suzuki S, Murakami N, Takeshita E, Chen WZ, Jong YJ, Endo Y, Komaki H, Fujii T, Kawano Y, Mori-Yoshimura M, Oya Y, Xi J, Zhu W, Zhao C, Watanabe Y, Ikemoto K, Nishikawa A, Hamanaka K, Mitsuhashi S, Suzuki N, Nishino I. Pediatric necrotizing myopathy associated with anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase antibodies. Rheumatology (Oxford). 2017 Feb;56(2):287-293. doi: 10.1093/rheumatology/kew386. Epub 2016 Nov 6. |
| 28096458 | Background | Tiniakou E, Pinal-Fernandez I, Lloyd TE, Albayda J, Paik J, Werner JL, Parks CA, Casciola-Rosen L, Christopher-Stine L, Mammen AL. More severe disease and slower recovery in younger patients with anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase-associated autoimmune myopathy. Rheumatology (Oxford). 2017 May 1;56(5):787-794. doi: 10.1093/rheumatology/kew470. |
| 29089059 | Background | 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. |