Krauspe R, Girschick H, Huppertz H I
Orthopädische Klinik, König-Ludwig-Haus, Julius-Maximilians-Universität Würzburg.
Orthopade. 1997 Oct;26(10):894-901. doi: 10.1007/PL00003339.
Chronic lymphoplasmacellular osteomyelitis may occur in children, adolescents and adults, but has not been found in newborns or babies either in our series or in the literature. Symptoms suggesting an acute disease like fever are uncommon, but a primary chronic course with symptomatic and asymptomatic periods is typical. Pain and swelling are the main symptoms; painless masses are rare. In children and adolescents the clavicle and metaphyseal regions of long bones are typical sites of chronic abacterial osteomyelitis. In adults the clavicles or the first two ribs are mainly affected with synovitis of the adjacent joints, but the long bones are rarely involved. Laboratory findings are non-specific but important for the differential diagnosis. The sedimentation rate and c-reactive protein might be elevated. The X-ray examination shows osteolytic, sclerotic or mixed bony changes and, in case of a diaphyseal involvement, onionskinlike periosteal bone formation may be present, suggesting a malignant process. In late stages sclerotic bone formations may be seen as a rest. Uni- or multifocal lesions can be detected by bonescan, as can asymptomatic lesions. Magnetic resonance imaging shows gross signal intensity differences both in the bone and perifocal soft tissue and involvement of the synovium with gadolinium DPTA enhancement in T1-weighted images. In early stages of the disease granulocytes, microabscesses and new bone formations might suggest bacterial osteomyelitis that cannot be differentiated by histology. In intermediate phases lymphocytic and plasma-cellular infiltrates are found, whereas in late phases sclerotic bone formations and fibrosis of the bone marrow are seen histologically. In chronic lymphoplasmacellular osteomyelitis, all clinical, radiological and histological findings, as well as negative bacteriological cultures, are mandatory and will allow a definitive diagnosis to be made. The disease may be uni- or multifocal, and new bone lesions may occur over time, as well as skin manifestations, which can be found years before or after bone involvement. The association with dermatological diseases and/or synovitis led to the acronym SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis, osteomyelitis). For the treatment nonsteroidal anti-inflammatory drugs are effective for pain relief, reduction of swelling and dysfunction. Antibiotics have been used in several series and are not effective. Major surgery is not recommended even for recurrences, and the prognosis for growth and function is excellent in the long term despite recurrences over several years.
慢性淋巴细胞浆细胞性骨髓炎可发生于儿童、青少年及成人，但在我们的病例系列以及文献中均未在新生儿或婴儿中发现。像发热这类提示急性疾病的症状并不常见，但以有症状期和无症状期交替出现的原发性慢性病程较为典型。疼痛和肿胀是主要症状，无痛性肿块较为罕见。在儿童和青少年中，锁骨及长骨干骺端是慢性非细菌性骨髓炎的典型发病部位。在成人中，锁骨或前两根肋骨主要受累，伴有相邻关节的滑膜炎，但长骨很少受累。实验室检查结果无特异性，但对鉴别诊断很重要。血沉和C反应蛋白可能升高。X线检查显示骨质溶解、硬化或混合性骨质改变，若骨干受累，可能出现葱皮样骨膜骨形成，提示恶性病变。在疾病后期，可见硬化性骨形成，表现为静止状态。骨扫描可检测到单灶或多灶性病变，也能发现无症状病变。磁共振成像显示骨及病灶周围软组织的信号强度有明显差异，在T1加权像上，钆喷替酸葡甲胺增强后可见滑膜受累。在疾病早期，粒细胞、微脓肿及新骨形成可能提示细菌性骨髓炎，但组织学上无法鉴别。在疾病中期可见淋巴细胞和浆细胞浸润，而在后期组织学上可见硬化性骨形成及骨髓纤维化。在慢性淋巴细胞浆细胞性骨髓炎中，所有临床、放射学及组织学检查结果以及阴性细菌培养结果都是必要的，可据此做出明确诊断。该病可为单灶性或多灶性，随着时间推移可能出现新的骨病变，还可能出现皮肤表现，可在骨受累之前或之后数年出现。与皮肤病和/或滑膜炎的关联导致了SAPHO综合征（滑膜炎、痤疮、脓疱病、骨肥厚、骨髓炎）这一缩写词的出现。对于治疗，非甾体类抗炎药对缓解疼痛、减轻肿胀及功能障碍有效。在多个病例系列中曾使用过抗生素，但无效。即使复发也不建议进行大手术，尽管数年内会复发，但从长期来看，生长和功能的预后良好。
