Department of Immunology and Allergy, Ankara Atatürk Sanatoryum Training and Research Hospital, University of Health Sciences, Ankara, Türkiye.
Department of Pulmonary Diseases, Ankara University Faculty of Medicine, Ankara, Türkiye.
Tuberk Toraks. 2023 Dec;71(4):367-377. doi: 10.5578/tt.20239605.
Sarcoidosis is a multisystem granulomatous disease with an unpredictable clinical course. Chitotriosidase is a chitinase mainly expressed by activated macrophages. Increased chitotriosidase activity has been reported in serum and bronchoalveolar lavage (BAL) of sarcoidosis patients compared to healthy controls. This study aims to evaluate the role of serum and BAL chitotriosidase activity on diagnosis, disease characteristics, and prognosis of sarcoidosis.
Patients referred with suspected sarcoidosis or other interstitial lung disease were prospectively included in the study. All patients underwent bronchoscopy with BAL. Serum and BAL chitotriosidase activity, BAL differential cell counts, and lymphocyte phenotypes were determined. Sarcoidosis patients were followed up regularly.
Forty-two sarcoidosis and 28 non-sarcoidosis patients were included in the study. Serum chitotriosidase activity was higher in sarcoidosis group 247.5 (2.78-461) vs 108 (2.78-272) nmol/h/mL (p< 0.001). BAL chitotriosidase activity tended to be higher in sarcoidosis group 11 (2-308) vs 6.95 (2.27-44) nmol/h/mg but was not found to be statistically significant (p= 0.11). Serum and BAL chitotriosidase activities were correlated with each other (p= 0.023, r= 0.355). No significant difference was found between the diagnostic performance of BAL CD4/CD8 ratio and serum chitotriosidase activity (p= 0.079). Serum chitotriosidase and ACE activities were correlated with each other (p= 0.004, r= 0.457). No significant difference was found between serum or BAL chitotriosidase activity and stage or extrapulmonary involvement. Serum chitotriosidase activity was higher in patients who needed systemic therapy at diagnosis (p= 0.046). However, no significant difference was found between serum or BAL chitotriosidase activities and disease progression (p= 0.395 and p= 0.723, respectively).
Serum chitotriosidase activity can be helpful in the differential diagnosis of sarcoidosis with a similar diagnostic performance with BAL CD4/CD8 ratio. Although serum chitotriosidase activity at diagnosis does not predict progressive disease, it is associated with the need for systemic therapy at diagnosis. Serial chitotriosidase measurements may be useful in monitoring disease progression during follow-up.
结节病是一种多系统肉芽肿性疾病，其临床表现不可预测。壳三糖苷酶是一种主要由活化的巨噬细胞表达的壳聚糖酶。与健康对照组相比，结节病患者的血清和支气管肺泡灌洗液（BAL）中壳三糖苷酶活性升高。本研究旨在评估血清和 BAL 壳三糖苷酶活性在结节病诊断、疾病特征和预后中的作用。
前瞻性纳入疑似结节病或其他间质性肺疾病的患者。所有患者均行支气管镜检查及 BAL。检测血清和 BAL 壳三糖苷酶活性、BAL 细胞分类计数和淋巴细胞表型。对结节病患者进行定期随访。
本研究共纳入 42 例结节病患者和 28 例非结节病患者。结节病组血清壳三糖苷酶活性显著高于非结节病组[247.5（2.78-461）比 108（2.78-272）nmol/h/mL，p<0.001]。结节病组 BAL 壳三糖苷酶活性[11（2-308）]虽有升高趋势，但与非结节病组相比无统计学差异（p=0.11）。血清和 BAL 壳三糖苷酶活性之间存在相关性（p=0.023，r=0.355）。BAL CD4/CD8 比值与血清壳三糖苷酶活性的诊断性能无显著差异（p=0.079）。血清壳三糖苷酶和 ACE 活性之间存在相关性（p=0.004，r=0.457）。血清或 BAL 壳三糖苷酶活性与分期或肺外受累之间无显著差异。诊断时需要全身治疗的患者血清壳三糖苷酶活性较高（p=0.046）。然而，血清或 BAL 壳三糖苷酶活性与疾病进展之间无显著差异（p=0.395 和 p=0.723）。
血清壳三糖苷酶活性有助于结节病的鉴别诊断，与 BAL CD4/CD8 比值的诊断性能相似。尽管诊断时的血清壳三糖苷酶活性不能预测疾病进展，但与诊断时需要全身治疗有关。在随访期间，连续监测壳三糖苷酶可能有助于监测疾病进展。
