Unexplained fever, weight loss, and worsened dyspnea with pulmonary hypertension as the presenting symptoms of mixed connective tissue disease with interstitial lung disease: A case report with review of literature

S. Patil, G. Gondhali, G. Narwade
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Abstract

Pulmonary manifestations of mixed connective tissue disease (MCTD) include as bronchiolitis, bronchiectasis, and interstitial lung disease with or without pulmonary hypertension. Tuberculosis (TB) is the most common diagnosis in India in the presence of constitutional symptoms such as cough, fever, and weight loss with lung parenchymal abnormality, irrespective of microscopic or nucleic acid amplification test abnormalities due to high tuberculosis prevalent tropical setting. In this case report, a 35 year old female presented with constitutional symptoms and lung parenchymal nodules, interstitial involvement with lung fibrosis and pulmonary hypertension. Mediastinal window documented necrotic mediastinal lymph nodes, without negative mycobacterial microscopic (smear examination) or genome documentation (Gene Xpert MTB/RIF). She had received empirical anti tuberculosis treatment for three months without clinical or radiological response, resulted in progression of disease with clinical and radiological worsening and referred to our unit for further workup. Bronchoscopy guided evaluation for tropical screen including bacterial, fungal and tuberculosis with malignancy was inconclusive. Vasculitis and CTD workup documented antinuclear antibody (ANA) test strongly positive with very highly raised titres, with positive antigen as U1 small nuclear ribonucleoprotein particle, SSA/RO, single strand DNA, and Scl 70. Treatment initiated with systemic steroids, tadalafil, mycophenolate and diuretics, and satisfactory clinical response documented as near complete resolution of pulmonary parenchymal abnormalities in 24 weeks and pulmonary hypertension in 12 weeks. Pulmonary manifestations of MCTD are common, underestimated in the presence of constitutional symptoms, and early pickup of entity in course of illness will have good outcome with excellent prognosis.
不明原因发热、体重减轻、呼吸困难加重伴肺动脉高压为混合性结缔组织病合并间质性肺疾病的临床表现1例并文献复习
混合性结缔组织病(MCTD)的肺部表现包括细支气管炎、支气管扩张和间质性肺疾病,伴或不伴肺动脉高压。肺结核(TB)在印度是最常见的诊断,表现为咳嗽、发烧、体重减轻和肺实质异常等体质症状,而不考虑由于结核病高发的热带环境导致的显微镜或核酸扩增试验异常。在这个病例报告中,一名35岁女性表现为体质症状和肺实质结节,肺间质受累伴肺纤维化和肺动脉高压。纵隔窗记录了坏死的纵隔淋巴结,没有阴性分枝杆菌镜检(涂片检查)或基因组记录(基因Xpert MTB/RIF)。她曾接受经验性抗结核治疗3个月,无临床或放射反应,导致疾病进展,临床和放射恶化,并转至我科进一步检查。支气管镜指导下的热带筛查包括细菌、真菌和恶性结核的评估尚无定论。血管炎和CTD检查显示抗核抗体(ANA)检测强烈阳性,滴度非常高,阳性抗原为U1小核核糖核蛋白颗粒,SSA/RO,单链DNA和scl70。治疗开始时使用全身类固醇、他达拉非、霉酚酸盐和利尿剂,临床反应令人满意,24周内肺实质异常几乎完全消失,12周内肺动脉高压几乎完全消失。MCTD的肺部表现是常见的,在存在体质症状时被低估,在病程中早期发现实体将有良好的结果和良好的预后。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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