Elucidation of predictors of disease progression in patients with relapsing polychondritis at the onset: potential impact on patient monitoring
Elucidation of predictors of disease progression in patients with relapsing polychondritis at the onset: potential impact on patient monitoring
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DOI：
10.1186/s41927-020-00141-8
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发表时间：
2020-01-01
期刊：
影响因子：
2.2
通讯作者：
Suzuki, Noboru
中科院分区：
文献类型：
--
作者：
Shimizu, Jun;Yamano, Yoshihisa;Suzuki, Noboru
复发性多囊卵巢炎（RP）患者中，病程进展和/或较长的患者会出现器官受累。对于他们的管理，阐明一个亚组提示疾病进展是waiting.MethodsWe以前进行了一个医生的病历为基础的回顾性研究，以阐明日本RP患者的主要临床特征。我们在此评估了疾病发作时和末次随访时的器官受累情况。然后，我们计算了229例RP患者末次随访时受累器官的累积数量，并将其与疾病发作时受累器官的数量进行比较，作为疾病进展的可能指标。我们在最后一次随访时将他们的预后分为“患者预后阶段”，从没有药物治疗（第1阶段）到死亡（第5阶段）。结果发病时每例患者受累器官数为1.13 ± 0.03，末次随访时为3.25 ± 0.10病程4.69 ± 0.33年，随患者预后分期的增加而沿着增加，发病时耳廓受累者分别为135例和48例（59%的229例患者，定义为耳廓发作亚组; AO）和呼吸系统受累（21%，呼吸道发作亚组; RO）。46例患者出现其他疾病（20%，错发亚组; MO），包括CNS、眼和内耳受累等。RO患者的预后比AO患者差。MO患者此后发生呼吸道和/或耳部受累，然后显示出显著较高的死亡率在117例末次随访时未发生呼吸道受累的RP患者中，MO组26例死亡率为19%，AO组91例死亡率为3.3%。因此，RO患者和MO患者与相对较差的预后相比AO patients.ConclusionsAllocation的患者RO和MO亚组，建议与RP的预后较差比AO亚组，特别是AO亚组没有呼吸系统的参与。所有RP患者都应仔细监测，但应特别注意MO患者，因为他们的疾病进展多样且加速。
BackgroundIn patients with relapsing polychondritis (RP), organ involvement developed in those with progressive and/or long disease courses. For their management, elucidation of a subgroup suggesting disease progression is awaited.MethodsWe previously conducted a physician's questionnaire-based retrospective study to elucidate major clinical features of Japanese patients with RP. We here evaluated organ involvement at disease onset and at the last follow-up. We then counted cumulative numbers of involved organs at the last follow-up in 229 RP patients and compared them with involved organ numbers at disease onset, as possible indicators of disease progression. We assigned their prognosis at the last follow-up into "patient prognostic stages" from no medication (stage 1) to death (stage 5). We utilized nonparametric tests for group comparisons.ResultsInvolved organ numbers per-patient were 1.130.03 at disease onset and 3.250.10 at the last follow-up (disease duration was 4.690.33years), and increased along with the patient prognostic stages.At disease onset, 135 and 48 patients had auricular involvement (59% of 229 patients, defined as auricular-onset subgroup; AO) and respiratory involvement (21%, respiratory-onset subgroup; RO), respectively. 46 patients presented with other conditions (20%, miscellaneous-onset subgroup; MO) including CNS, ocular, and inner ear involvement, among others.RO patients showed worse (poorer) prognostic stages than AO patients. MO patients developed respiratory and/or auricular involvement thereafter and then showed significantly higher mortality rate (15%; 7/46) than AO patients (5.9%; 8/135).In RP patients who did not develop respiratory involvement until the last follow-up (throughout the disease course; 117 patients), mortality rate was 19% in 26 MO patients and 3.3% in 91 AO patients. Accordingly, RO patients and MO patients associated with relatively poor prognosis compared with AO patients.ConclusionsAllocation of patients to RO and MO subgroups was suggested to associate with poorer prognosis of RP than AO subgroups, especially AO subgroups without respiratory involvement throughout. All RP patients deserve careful monitoring but special attention should be paid to MO patients because of their diverse and accelerated disease progression.