Rhumatologie, Hôpital Cochin, APHP, Université de Paris, Hôpital Cochin, 27 rue du Faubourg Saint-Jacques, 75014 Paris, France; Division of Rheumatology, Department of Medicine-DIMED, University of Padova, 35126 Padova, Italy.
Radiologie B, Hôpital Cochin, APHP, Université de Paris, 75014 Paris, France.
Semin Arthritis Rheum. 2020 Dec;50(6):1339-1347. doi: 10.1016/j.semarthrit.2020.03.009. Epub 2020 Apr 9.
We aimed to clarify the definition, distribution, clinical association and outcomes of large calcinosis in patients with systemic sclerosis (SSc).
We conducted a systematic literature review (SLR) focusing on SSc-related large calcified masses. Upon updating the terminology and definition, all cases of "pseudotumoral" calcinosis seen at the Cochin and Padova University Hospitals were reviewed.
The SLR yielded 30 SSc cases, with large calcified masses mainly defined as "tumoral" or "pseudotumoral". Among the 629 SSc cases included in the Cochin and Padova cohorts, 19 (3%) living and 7 deceased patients were affected by pseudotumoral calcinosis; among these, the great majority had a severe vascular phenotype. The mean age in the whole population (56 cases) was 59 ± 11.4 years with a median disease duration at calcinosis onset of 7 (5-10) years. Twenty-five out of 56 patients (44.6%) had the diffuse cutaneous form of SSc. Anti-topisomerase I and anticentromere were found equally. Pseudotumoral calcinosis were commonly symmetrical and the size ranged from 2 to 15.5 cm. Most patients had multiple site involvement: 52% hand/wrist, 29% shoulders and elbows, 20% hips and 25% spinal calcinosis. Fistulization/ulceration and infections were reported in 32% and 23% of cases, respectively; nerve compression was found in 40% of spinal calcinosis and in one patient with limb calcinosis. There was no clear evidence of clinical and radiological improvement with any treatment. A partial improvement was seen in 7 patients that underwent surgery.
Pseudotumoral calcinosis may occur in about 3% of SSc patients, commonly symmetrical and in multiple sites without differences regarding the cutaneous subtypes but often in those with a severe vascular phenotype. Medical treatment seems ineffective, whereas a surgical approach may be considered.
我们旨在阐明系统性硬化症（SSc）患者中大型钙化的定义、分布、临床关联和结局。
我们进行了一项系统文献综述（SLR），重点关注与 SSc 相关的大型钙化肿块。在更新术语和定义后，回顾了 Cochin 和帕多瓦大学医院所见的所有“假性肿瘤样”钙化病例。
SLR 产生了 30 例 SSc 病例，其中大型钙化肿块主要定义为“肿瘤样”或“假性肿瘤样”。在 Cochin 和帕多瓦队列中纳入的 629 例 SSc 病例中，19 例（3%）存活和 7 例死亡患者患有假性肿瘤样钙化；其中绝大多数患者具有严重的血管表型。整个人群（56 例）的平均年龄为 59 ± 11.4 岁，钙化发病时的中位疾病持续时间为 7（5-10）年。56 例患者中有 25 例（44.6%）患有弥漫性皮肤型 SSc。抗拓扑异构酶 I 和抗着丝粒抗体的检出率相等。假性肿瘤样钙化通常是对称的，大小范围为 2 至 15.5 厘米。大多数患者多部位受累：52%手/腕、29%肩肘、20%髋和 25%脊柱钙化。瘘管/溃疡和感染分别在 32%和 23%的病例中报告，神经压迫在 40%的脊柱钙化和 1 例肢体钙化中发现。任何治疗均未明显改善临床和影像学表现。7 例接受手术的患者有部分改善。
假性肿瘤样钙化可能发生在约 3%的 SSc 患者中，通常是对称的，多部位受累，与皮肤亚型无差异，但常发生于严重血管表型患者中。药物治疗似乎无效，而手术方法可能是可行的。
