Department of Pediatrics, Faculty of Medicine and Health Sciences, Andrzej Frycz Modrzewski Cracow University, Cracow, Poland.
Clinical Department of Pediatrics and Rheumatology, St. Louis Regional Specialised Children's Hospital, Cracow, Poland.
Am J Case Rep. 2024 Sep 22;25:e943579. doi: 10.12659/AJCR.943579.
BACKGROUND Chronic nonbacterial osteomyelitis (CNO) is a multifocal autoinflammatory bone disease mainly affecting children and adolescents. Sacroiliitis is an inflammation of the sacroiliac joint, diagnosed with the use of musculoskeletal MRI due to its ability to visualize active inflammatory lesions. Ehlers-Danlos syndrome (EDS) is non-inflammatory hereditary disorder of connective tissue. Here, we report the case of a 17.5-year-old female patient with classical EDS and long-term course of the CNO with coexistence of sacroiliac arthritis. CASE REPORT On admission, a patient with CNO reported pain in the scapula, thoracic spine, shoulders, and iliac region, with morning stiffness present for 5 months. Physical examination revealed knee and elbow joint hyperextension, hypermobility of the phalanges, increased range of motion of the hip joints, and the presence of reticular rash on the face. In the laboratory blood tests, minor leukocytosis was reported. During hospitalization, a whole-body MRI was performed, detecting bone marrow edema in the Th3, Th4, and Th7 vertebral bodies and the head of seventh rib on the left side, as well as bilaterally in the sacroiliac joints. The patient was diagnosed with sacroiliitis and EDS and successfully treated with risedronate sodium, methotrexate with folic acid, sulfasalazine, and meloxicam, achieving CNO remission and reduced severity of axial skeleton pain. CONCLUSIONS The coexistence of these 3 diseases - CNO, sacroiliac arthritis, and EDS - in the same patient is rare and requires interphysician collaboration to determine the correct diagnosis and subsequently arrange multi-speciality therapeutic management to achieve remission.
慢性非细菌性骨髓炎(CNO)是一种多灶性自身炎症性骨病,主要影响儿童和青少年。骶髂关节炎是骶髂关节的炎症,由于其能够可视化活跃的炎症病变,因此使用肌肉骨骼 MRI 进行诊断。埃勒斯-当洛斯综合征(EDS)是一种非炎症性遗传性结缔组织疾病。在这里,我们报告了一例 17.5 岁的女性患者,患有经典型 EDS 和 CNO 的长期病程,伴有骶髂关节炎共存。
入院时,一名患有 CNO 的患者报告肩胛、胸椎、肩部和髂区疼痛,伴有 5 个月的晨僵。体格检查显示膝关节和肘关节过度伸展、指骨过度活动、髋关节活动范围增加以及面部出现网状皮疹。实验室血液检查显示轻度白细胞增多。住院期间,进行了全身 MRI 检查,发现左侧第 3、4、7 胸椎和第 7 肋骨头部以及双侧骶髂关节有骨髓水肿。患者被诊断为骶髂关节炎和 EDS,并成功接受利塞膦酸钠、甲氨蝶呤加叶酸、柳氮磺胺吡啶和美洛昔康治疗,实现了 CNO 缓解和轴性骨骼疼痛减轻。
这三种疾病(CNO、骶髂关节炎和 EDS)同时存在于同一患者中非常罕见,需要跨学科合作以确定正确的诊断,随后安排多学科治疗管理以实现缓解。