Amirpour Anahita, Ghorbani Parastoo, Ghadirzadeh Erfan, Charkazi Elham, Paraandavaji Elham
Department of Rheumatology Shahid Beheshti University of Medical Sciences Tehran Iran.
Department of Internal Medicine Mazandaran University of Medical Sciences Sari Iran.
Clin Case Rep. 2023 Oct 19;11(10):e8090. doi: 10.1002/ccr3.8090. eCollection 2023 Oct.
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.
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.
即使没有硬皮病的典型皮肤症状,在对最初诊断为特发性间质性肺病的患者进行鉴别诊断时,也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。虽然皮肤增厚是系统性硬化症的标志性表现,但有一种称为无硬皮病的系统性硬化症(ssSSc)的亚型,在没有明显皮肤表现的情况下,会出现内脏器官受累和血清学标志物阳性。我们报告了一例36岁伊朗女性病例,其以杵状指作为ssSSc的初始症状。值得注意的是,杵状指作为该疾病唯一的初始症状此前尚未见报道。及时诊断ssSSc对于促进适当治疗和预防疾病进展至关重要。在评估临床特征有限的患者时,医生应采取综合方法,因为这些特征可能提示潜在的ssSSc。