Pastré Jean, Bouvry Diane, Juvin Karine, Benattia Amira, Annesi-Maesano Isabella, Valeyre Dominique, Nunes Hilario, Israël-Biet Dominique
Service de Pneumologie et Soins Intensifs, Assistance Publique-Hôpitaux de Paris Centre, Hôpital Européen Georges Pompidou, 75015 Paris, France.
Service de Pneumologie, Assistance Publique-Hôpitaux de Paris, Hôpital Avicenne, 93009 Bobigny, France.
J Clin Med. 2021 May 5;10(9):1988. doi: 10.3390/jcm10091988.
(1) Background: Systemic granulomatosis developed in a context of malignancy has already been reported. Our objective was to describe the clinical, radiological, functional, biological, and evolutive characteristics of sarcoidosis-like cancer-associated granulomatosis (SLCAG) and to compare them to those of sarcoidosis. (2) Methods: 38 patients with a biopsy-proven SLCAG developed after a diagnostic of malignancy were included. The control group consisted of sarcoidosis patients matched for age, sex, and radiologic stage. Clinical, biological, physiological, radiological, and outcome data were collected. (3) Results: The mean age of SLCAG patients was 51 ± 14 years. They were diagnosed within 15 ± 14 months of the cancer diagnosis (breast cancer most frequently). All SLCAG patients presented a thoracic involvement, extrathoracic locations were observed in 32% of subjects. SLCAG was more often asymptomatic than sarcoidosis ( < 0.0001). During follow-up, systemic treatment was less often required in SLCAG than in sarcoidosis (58% vs. 32%, = 0.04 respectively) and SLCAG were characterized by a significantly less severe progression profile according to the Sarcoid Clinical Activity Classification, with a complete recovery more frequent at 5 years ( = 0.03). (4) Conclusion: This case-control study shows that SLCAG differs from sarcoidosis with a significantly more benign course. These results might argue for true differences in the physiopathology, which remain to be elucidated.
(1) 背景:恶性肿瘤背景下发生的系统性肉芽肿病已有报道。我们的目的是描述类肉瘤样癌症相关肉芽肿病(SLCAG)的临床、放射学、功能、生物学及演变特征,并将其与结节病的特征进行比较。(2) 方法:纳入38例经活检证实为恶性肿瘤诊断后发生的SLCAG患者。对照组由年龄、性别和放射学分期相匹配的结节病患者组成。收集临床、生物学、生理学、放射学及转归数据。(3) 结果:SLCAG患者的平均年龄为51±14岁。他们在癌症诊断后15±14个月内被诊断(最常见为乳腺癌)。所有SLCAG患者均有胸部受累,32%的患者有胸外部位受累。SLCAG比结节病更常无症状(<0.0001)。在随访期间,SLCAG患者比结节病患者更少需要全身治疗(分别为58%对32%,P=0.04),并且根据结节病临床活动分类,SLCAG的病情进展明显较轻,5年时完全恢复更为常见(P=0.03)。(4) 结论:这项病例对照研究表明,SLCAG与结节病不同,病程明显更良性。这些结果可能支持生理病理学上存在真正差异,这仍有待阐明。