关键词: atrial septal defect case report clubbing interstitial lung disease systemic sclerosis sine scleroderma

来  源:   DOI:10.1002/ccr3.8090   PDF(Pubmed)

Abstract:
UNASSIGNED: Even in the absence of characteristic cutaneous symptoms of scleroderma, systemic sclerosis should be considered in the differential diagnosis of patients initially diagnosed with idiopathic interstitial lung disease.
UNASSIGNED: Systemic sclerosis (SSc) is an idiopathic connective tissue disorder characterized by multisystem involvement. Although skin thickening is a hallmark manifestation of SSc, a subset known as systemic sclerosis sine scleroderma (ssSSc) presents with internal organ involvement and positive serologic markers in the absence of significant cutaneous manifestations. We report the case of a 36-year-old Iranian woman who presented with clubbing as an initial symptom of ssSSc. Notably, clubbing as the sole initial sign of the disease has not been previously reported. Timely diagnosis of ssSSc is crucial to facilitate appropriate treatment and prevent disease progression. Physicians should adopt a comprehensive approach when evaluating patients presenting with limited clinical features, as they might be indicative of underlying ssSSc.
摘要:
即使没有硬皮病的特征性皮肤症状,在最初诊断为特发性间质性肺病的患者的鉴别诊断中,应考虑系统性硬化症。
系统性硬化症(SSc)是一种特发性结缔组织疾病,其特征是多系统受累。虽然皮肤增厚是SSc的标志表现,一个被称为系统性硬化症硬皮病(ssSSc)的子集在没有明显皮肤表现的情况下表现为内脏器官受累和血清学标志物阳性.我们报告了一名36岁的伊朗妇女的病例,该妇女表现为ssSSc的最初症状。值得注意的是,以前没有报道过该疾病的唯一初始体征。及时诊断ssSSc对于促进适当治疗和预防疾病进展至关重要。医师在评估临床特征有限的患者时,应采用全面的方法。因为它们可能表明潜在的ssSSc。
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