Hormaza-Jaramillo Andrés, Bedoya-Joaqui Vanessa, Puerta-Sarmiento Germán, Bautista Mario, Rios-Serna Lady J, Delgado-Mora Tatiana, Nieto-Aristizábal Ivana, Ruiz-Ordoñez Ingrid
Unidad de Reumatología, Fundación Valle del Lili, Cali, Colombia.
Unidad de Reumatología, Departamento de Medicina Interna, Facultad de Ciencias de la Salud, Universidad ICESI, Cali, Colombia.
Front Med (Lausanne). 2022 Aug 15;9:954937. doi: 10.3389/fmed.2022.954937. eCollection 2022.
Idiopathic inflammatory myopathies (IIMs) are chronic, autoimmune diseases with several forms of presentation. Diagnosis is mostly clinical in our region. Our aim was to evaluate the autoantibody profile of patients with IIMs.
This study is a cross-sectional study with a prospective recollection of data, conducted between 2019-2021, in a single center in Cali, Colombia. Patients with a clinical diagnosis or suspicion of IIM were included. The presence of myositis-specific/associated antibodies was evaluated by immunoblotting in serum samples. Phenotypic characterization was performed.
A total of 36 patients were included. The mean age was 50.6 (16.7) years, and 20 (55.6%) were female. Eighteen (50%) patients were seropositive, of which 11 (30.5%) presented one positive antibody, with anti-TIF1ɣbeing the most frequent ( = 4, 11.1%), followed by anti-Ro52 ( = 2, 5.6%). Seven patients (19.4%) showed >1 positive antibody. Dermatomyositis was the most frequent type of IIM in seropositive patients ( = 8, 44.4%), followed by anti-synthetase syndrome ( = 4, 22.2%). Weakness was symmetric and presented in the upper and lower extremities in 11 (61.1%) patients each. Both respiratory insufficiency and weight loss were seen in 7 (38.9%) patients, Gottron papules in six (33.3%) patients, and heliotrope rash, esophageal dysmotility, and myalgia in 5 (27.8%) patients. Pulmonary interstitial disease was seen in 4 (22.2%, with antibodies for anti-Ro52, anti-MDA5 + anti-Jo1 + anti-TIF1ɣ, anti-MDA5 + anti-SAE1 + anti-NXP2, and anti-cN1A + anti-Ro52) patients, and malignancy was seen in 2 (11.1%) patients (1 with anti-Mi2β and 1 with anti-TIF1ɣ + anti-Mi2α). In all, 7 (19.4%) patients required intensive care (2 seropositive, 1 with anti-PL7, 1 with anti-MDA5 + anti-Jo1 + anti-TIF1ɣ), and 1 (2.8%) (seronegative) patient died.
This study is the first study in the Southwest of Colombia that evaluates myositis-specific/associated antibodies in IIM. Half of the patients were seropositive. Anti-TIF1ɣwas the most frequent MSA and anti-Ro52 was the most frequent MAA. Several patients presented antibody combinations. Further studies are needed to fully associate phenotypes with antibodies.
特发性炎性肌病（IIMs）是具有多种表现形式的慢性自身免疫性疾病。在我们地区，诊断主要依靠临床症状。我们的目的是评估IIMs患者的自身抗体谱。
本研究为一项横断面研究，于2019年至2021年在哥伦比亚卡利的一个单一中心进行，前瞻性收集数据。纳入临床诊断或疑似IIM的患者。通过免疫印迹法检测血清样本中肌炎特异性/相关性抗体的存在情况，并进行表型特征分析。
共纳入36例患者。平均年龄为50.6（16.7）岁，女性20例（55.6%）。18例（50%）患者血清学呈阳性，其中11例（30.5%）出现一种阳性抗体，最常见的是抗TIF1ɣ（4例，11.1%），其次是抗Ro52（2例，5.6%）。7例（19.4%）患者出现>1种阳性抗体。在血清学阳性患者中，皮肌炎是最常见的IIM类型（8例，44.4%），其次是抗合成酶综合征（4例，22.2%）。11例（61.1%）患者的肌无力表现为对称性，累及上肢和下肢。7例（38.9%）患者出现呼吸功能不全和体重减轻，6例（33.3%）患者出现Gottron丘疹，5例（27.8%）患者出现向阳疹、食管动力障碍和肌痛。4例（22.2%）患者出现肺间质疾病（抗体分别为抗Ro52、抗MDA5 + 抗Jo1 + 抗TIF1ɣ、抗MDA5 + 抗SAE1 + 抗NXP2、抗cN1A + 抗Ro52），2例（11.1%）患者发生恶性肿瘤（1例抗Mi2β，1例抗TIF1ɣ + 抗Mi2α）。共有7例（19.4%）患者需要重症监护（2例血清学阳性，1例抗PL7，1例抗MDA5 + 抗Jo1 + 抗TIF1ɣ），1例（2.8%）（血清学阴性）患者死亡。
本研究是哥伦比亚西南部第一项评估IIM中肌炎特异性/相关性抗体的研究。半数患者血清学呈阳性。抗TIF1ɣ是最常见的肌炎特异性抗体，抗Ro52是最常见的肌炎相关性抗体。部分患者出现抗体组合。需要进一步研究以全面关联表型与抗体。
