Sorbonne Universités, UPMC Univ Paris 06, UMR 7211, and Inflammation-Immunopathology-Biotherapy Department (DHU i2B), 75005 Paris, France; AP-HP, Groupe Hospitalier Pitié-Salpêtrière, Department of Internal Medicine and Clinical Immunology, 75013 Paris, France.
Service de Médecine Interne et Rhumatologie, HIA Legouest, Metz, France.
Joint Bone Spine. 2019 Nov;86(6):789-793. doi: 10.1016/j.jbspin.2019.07.009. Epub 2019 Jul 30.
To describe the clinical presentation, distribution of lesions, treatment, and outcomes of osseous sarcoidosis.
A French retrospective multicenter study of patients with biopsy-proven sarcoidosis analyzed patients with 1) a biopsy-proven granuloma without caseous necrosis, and either 2) osseous clinical manifestations, or 3) abnormal osseous imaging. Sarcoidosis patients with osseous involvement (cases) were compared with 264 age- and sex-matched sarcoidosis patients with no osseous manifestations (controls).
In the osseous sarcoidosis group (n=88), forty-two (48%) patients had osseous-related symptoms involving the axial (69%) and/or appendicular (58%) skeleton. On imaging, the most commonly affected bones were in the spine (52%), pelvis (42%), hands (22%) and femur (19%). Compared with controls, cases had higher rates of mediastinal (93% vs. 47%) and extra-thoracic lymph node involvement (66% vs. 21%), pulmonary (90% vs. 65%) and cutaneous involvement (44% vs. 23%) (all P<0.0001), and hypercalcemia (8.5% vs. 2%, P=0.014). Spleen/liver and gastrointestinal involvement were less frequent in the osseous sarcoidosis group (29% vs. 45%, and 1% vs. 17%, respectively, P<0.0001). Response rates to with glucocorticoids alone, glucocorticoids plus methotrexate or glucocorticoids plus hydroxychloroquine were 23/44 (52%), 9/13 (69%) and 4/6 (67%), respectively.
In patients with osseous sarcoidosis the spine and pelvis were the most commonly affected bones. Compared with controls, cases with osseous sarcoidosis have higher rates of thoracic and extra-thoracic lymph node involvement, pulmonary and cutaneous involvement, and hypercalcemia. Most patients with osseous sarcoidosis had a good response to glucocorticoids in combination with methotrexate or hydroxychloroquine.
描述骨结节病的临床表现、病变分布、治疗和转归。
对法国一项回顾性多中心研究中经活检证实的结节病患者进行分析，纳入有以下特征的患者：1）活检证实为无干酪样坏死的肉芽肿，或 2）有骨临床表现，或 3）骨影像学异常。将有骨受累的结节病患者（病例组）与 264 例年龄和性别匹配、无骨表现的结节病患者（对照组）进行比较。
在骨结节病组（n=88）中，42 例（48%）患者有骨相关症状，累及脊柱（69%）和/或四肢（58%）骨骼。影像学上，最常受影响的骨骼是脊柱（52%）、骨盆（42%）、手部（22%）和股骨（19%）。与对照组相比，病例组的纵隔（93% vs. 47%）和胸外淋巴结受累（66% vs. 21%）、肺部（90% vs. 65%）和皮肤受累（44% vs. 23%）的发生率更高（均 P<0.0001），高钙血症的发生率也更高（8.5% vs. 2%，P=0.014）。骨结节病组中脾脏/肝脏和胃肠道受累的发生率较低（29% vs. 45%，1% vs. 17%，均 P<0.0001）。单独使用糖皮质激素、糖皮质激素联合甲氨蝶呤或糖皮质激素联合羟氯喹的有效率分别为 23/44（52%）、9/13（69%）和 4/6（67%）。
在有骨结节病的患者中，脊柱和骨盆是最常受影响的骨骼。与对照组相比，有骨结节病的病例组中，胸部和胸外淋巴结受累、肺部和皮肤受累以及高钙血症的发生率更高。大多数有骨结节病的患者对糖皮质激素联合甲氨蝶呤或羟氯喹治疗有良好的反应。
