{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,4]],"date-time":"2026-05-04T01:13:22Z","timestamp":1777857202010,"version":"3.51.4"},"reference-count":29,"publisher":"SAGE Publications","issue":"1","license":[{"start":{"date-parts":[[2025,1,1]],"date-time":"2025-01-01T00:00:00Z","timestamp":1735689600000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"},{"start":{"date-parts":[[2025,1,1]],"date-time":"2025-01-01T00:00:00Z","timestamp":1735689600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["Journal of Neuromuscular Diseases"],"published-print":{"date-parts":[[2025,1]]},"abstract":"<jats:sec>\n                    <jats:title>Background:<\/jats:title>\n                    <jats:p>Inflammatory myopathy with abundant macrophage [IMAM] is marked by macrophage infiltration and muscle fibers damage, resembling dermatomyositis [DM] but with unique pathology. Its mechanism remains unclear. Our study focused on exploring the clinicopathological characteristics, underlying pathogenic mechanisms, and the challenges in diagnosing and managing IMAM.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods:<\/jats:title>\n                    <jats:p>A systematic analysis of medical literature databases (Pub-med, Cochrane, Scopus, Google Scholar) was performed using the term \u201cIMAM,\u201d excluding studies on other inflammatory myopathies [IMs]. Selected studies were independently assessed with the Newcastle-Ottawa Scale, and quantitative data underwent inter-statistical analysis, descriptive and odds ratio, to identify relevant findings.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results:<\/jats:title>\n                    <jats:p>\n                      Eight studies, including 49 IMAM cases from 2003 to 2024, were analyzed. Five were case reports, and three were cross-sectional studies. IMAM showed no age or sex predilection. Common symptoms included proximal muscle weakness, pain, and fatigue, with atypical DM-like skin features in 65% of cases. Other association included hemophagocytosis, cutaneous panniculitis, and interstitial lung infiltration. Histologically, all cases showed myonecrosis infiltrated with CD68+ macrophages. Scattered CD3+ and CD4+ T-cells expressing IL-10 with no or rare CD8+ T-cells were identified. MAC deposition was limited to necrotic fibers, and perifascicular atrophy was absent in all cases. Anti-PL-7 and anti-U1 RNP antibodies were detected in 4% of cases. Elevated TNF\u03b1 and IFN-\u03b3 levels, with low STAT1 and STAT6, were observed. Genetic analysis revealed\n                      <jats:italic toggle=\"yes\">MEFV<\/jats:italic>\n                      polymorphisms in 7 cases and a\n                      <jats:italic toggle=\"yes\">TNFRSF1A<\/jats:italic>\n                      mutation [C43R] in single case. Treatment involved steroids, with or without immunotherapy or chemotherapy, leading to remission and recovery in 43.7% of cases.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion:<\/jats:title>\n                    <jats:p>IMAM is a distinct type of IMs that requires muscle biopsy for diagnosis as myositis antibody and cytokine tests are usually insensitive.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1177\/22143602251315376","type":"journal-article","created":{"date-parts":[[2025,1,23]],"date-time":"2025-01-23T08:28:12Z","timestamp":1737620892000},"page":"15-22","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":0,"title":["Inflammatory myopathy with abundant macrophage [IMAM]: Systemic analysis and pathological approach to distinguish it from dermatomyositis"],"prefix":"10.1177","volume":"12","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0115-9457","authenticated-orcid":false,"given":"Ahmed","family":"Bamaga","sequence":"first","affiliation":[{"name":"Department of Pediatrics, Faculty of Medicine, King Abdulaziz University, Jeddah, Saudi Arabia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8979-3849","authenticated-orcid":false,"given":"Maher","family":"Kurdi","sequence":"additional","affiliation":[{"name":"Department of Pathology, Faculty of Medicine, King Abdulaziz University, Rabigh, Saudi Arabia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alaa","family":"Alkhotani","sequence":"additional","affiliation":[{"name":"Department of Pathology, College of Medicine, Umm Al-Qura University, Mecca, Saudi Arabia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Huda","family":"Alghefari","sequence":"additional","affiliation":[{"name":"Department of Pathology, Faculty of Medicine, King Abdulaziz University, Rabigh, Saudi Arabia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0009-0009-4090-5051","authenticated-orcid":false,"given":"Raed","family":"Gasemaltayeb","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Faculty of Medicine, King Abdulaziz University, Rabigh, Saudi Arabia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Faisal","family":"Mrair","sequence":"additional","affiliation":[{"name":"Department of Pathology, Faculty of Medicine, King Abdulaziz University, Rabigh, Saudi Arabia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0754-7896","authenticated-orcid":false,"given":"Zahir T","family":"Fadel","sequence":"additional","affiliation":[{"name":"Department of Surgery, Faculty of Medicine, King Abdulaziz University, Jeddah, Saudi Arabia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Riffat","family":"Mehboob","sequence":"additional","affiliation":[{"name":"National Institute of Health, Bethesda, MD, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Awab","family":"Tayyib","sequence":"additional","affiliation":[{"name":"Department of Pathology, Faculty of Medicine, University of Jeddah, Jeddah, Saudi Arabia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ahmad R","family":"Abuzinadah","sequence":"additional","affiliation":[{"name":"Department of Neurology, Faculty of Medicine, King Abdulaziz University, Jeddah, Saudi Arabia"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"179","published-online":{"date-parts":[[2025,1]]},"reference":[{"key":"e_1_3_4_2_2","doi-asserted-by":"publisher","DOI":"10.1093\/jnen\/62.5.464"},{"key":"e_1_3_4_3_2","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410160206"},{"key":"e_1_3_4_4_2","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/124.9.1821"},{"key":"e_1_3_4_5_2","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-009-0570-8"},{"key":"e_1_3_4_6_2","doi-asserted-by":"publisher","DOI":"10.1097\/00019052-200410000-00006"},{"key":"e_1_3_4_7_2","doi-asserted-by":"publisher","DOI":"10.1111\/cei.12407"},{"key":"e_1_3_4_8_2","doi-asserted-by":"publisher","DOI":"10.1007\/s00296-010-1662-4"},{"key":"e_1_3_4_9_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.nmd.2013.11.004"},{"key":"e_1_3_4_10_2","doi-asserted-by":"publisher","DOI":"10.1093\/jnen\/nlx011"},{"key":"e_1_3_4_11_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.nmd.2006.05.008"},{"key":"e_1_3_4_12_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0190-9622(98)70044-1"},{"key":"e_1_3_4_13_2","first-page":"262","article-title":"Update on panniculitis","volume":"68","author":"Phelps RG","year":"2001","unstructured":"Phelps RG, Shoji T. 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