Achieng Sheilla, Harris Jonathan, Samaranayaka Muditha, Herrick Ariane L
School of Biological Sciences, Division of Musculoskeletal and Dermatological Sciences, The University of Manchester, Manchester Academic Health Science Centre, Manchester, UK.
Department of Rheumatology, Northern Care Alliance NHS Foundation Trust, Salford Care Organisation, Manchester, UK.
Rheumatol Adv Pract. 2024 Jan 27;8(1):rkae011. doi: 10.1093/rap/rkae011. eCollection 2024.
Calcinosis is a well-described entity that occurs in patients with systemic sclerosis (SSc) and dermatomyositis (DM). Calcinosis in SSc typically occurs over pressure points and is usually nodular. We present a case series of four patients with SSc with a much rarer, diffuse form of calcinosis to illustrate this poorly recognized pattern of extensive and debilitating disease.
Four patients with SSc and extensive calcinosis were identified from patients attending a tertiary rheumatology centre in the preceding 3 years. Their electronic case notes, radiographic images and medical photographs were reviewed.
All four patients had the diffuse cutaneous subtype of SSc (dcSSc) and additionally a myositis overlap. This was in the context of 102 of 461 (22%) patients with SSc whose clinical details had been recorded in the preceding 3 years having dcSSc. Their ages at diagnosis ranged from 27 to 65 years. Three were female, two were anti-Scl70 antibody positive, and two were anti-PMScl antibody positive. Development of calcinosis occurred between 1 and 6 years after onset of SSc. Plain radiography showed very extensive calcinosis in various sites, distributed in a pattern akin to sheets of calcium-containing deposits in the skin and subcutaneous tissue.
Although calcinosis is common in SSc, extensive sheet-like calcinosis is very rare. Our experience suggests that when this form of calcinosis does occur, this is in the context of the diffuse cutaneous subtype of disease and with myositis overlap. The four cases described should raise awareness of this unusual and extensive pattern of disease.
钙质沉着症是一种在系统性硬化症（SSc）和皮肌炎（DM）患者中已被充分描述的病症。SSc中的钙质沉着症通常发生在受压点，且通常为结节状。我们报告了一组4例SSc患者的病例系列，这些患者患有一种更为罕见的弥漫性钙质沉着症，以说明这种认识不足的广泛且使人衰弱的疾病模式。
从在过去3年中就诊于一家三级风湿病中心的患者中，识别出4例患有广泛钙质沉着症的SSc患者。对他们的电子病历、影像学图像和医学照片进行了回顾。
所有4例患者均为弥漫性皮肤型SSc（dcSSc），并且还存在肌炎重叠。在过去3年中记录了临床细节的461例SSc患者中，有102例（22%）为dcSSc。他们的诊断年龄在27至65岁之间。3例为女性，2例抗Scl70抗体阳性，2例抗PMScl抗体阳性。钙质沉着症在SSc发病后1至6年出现。X线平片显示在各个部位有非常广泛的钙质沉着，分布模式类似于皮肤和皮下组织中含钙质沉积物的片状分布。
尽管钙质沉着症在SSc中很常见，但广泛的片状钙质沉着症非常罕见。我们的经验表明，当出现这种形式的钙质沉着症时，是在弥漫性皮肤型疾病且伴有肌炎重叠的背景下。所描述的这4例病例应提高对这种不寻常且广泛的疾病模式的认识。
