Delgado-de la Mora Jesus, Martín-Nares Eduardo, Quintero-Bustos Gabriel, Montante-Montes de Oca Daniel, Martínez Benitez Braulio
Department of Pathology, Weill-Cornell Medicine, New York, USA.
Department of Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, MEX.
Cureus. 2024 Feb 9;16(2):e53907. doi: 10.7759/cureus.53907. eCollection 2024 Feb.
We present an intriguing case involving a rare occurrence of sclerosing angiomatoid nodular transformation (SANT) in a 57-year-old woman with a history of granulomatosis with polyangiitis (GPA). Despite the extensive literature on SANT, its pathogenesis remains elusive. The patient, diagnosed with serum anti-proteinase 3 antineutrophil cytoplasmic antibody (PR3-ANCA)-positive GPA seven years earlier, exhibited a splenic lesion during imaging, leading to laparoscopic splenectomy due to severe abdominal pain. Microscopic analysis unveiled nodular structures with vascular elements surrounded by fibrosclerotic stroma and chronic inflammatory cells. This case raises questions about the interplay between SANT, GPA activity, and vascular damage. Hypotheses regarding SANT's origin, including its potential association with organized hematoma or alterations in splenic blood flow, are discussed. The uniqueness of this case lies in the coexistence of PR3-ANCA-positive GPA and SANT, suggesting a potential link between GPA activity, vascular damage, and SANT development. While causality remains uncertain, this report marks the first documented case of a patient with PR3-ANCA-positive GPA developing SANT. The findings prompt reflection on a potential common pathophysiological mechanism and underscore the importance of considering SANT in cases of splenic lesions associated with conditions causing alterations in splenic blood flow. This contribution serves as a valuable addition to the existing knowledge, urging further research and consideration of SANT in diagnostic scenarios involving splenic abnormalities.
我们报告了一例有趣的病例，一名57岁有肉芽肿性多血管炎（GPA）病史的女性罕见地发生了硬化性血管瘤样结节性转化（SANT）。尽管关于SANT的文献很多，但其发病机制仍不清楚。该患者七年前被诊断为血清抗蛋白酶3抗中性粒细胞胞浆抗体（PR3-ANCA）阳性的GPA，影像学检查发现脾脏有病变，因严重腹痛接受了腹腔镜脾切除术。显微镜分析显示结节结构，有血管成分，周围是纤维硬化性间质和慢性炎症细胞。该病例引发了关于SANT、GPA活动和血管损伤之间相互作用的问题。讨论了关于SANT起源的假说，包括其与机化血肿或脾血流改变的潜在关联。该病例的独特之处在于PR3-ANCA阳性的GPA和SANT并存，提示GPA活动、血管损伤和SANT发展之间可能存在联系。虽然因果关系尚不确定，但本报告是首例记录PR3-ANCA阳性的GPA患者发生SANT的病例。这些发现促使人们思考潜在的共同病理生理机制，并强调在与导致脾血流改变的疾病相关的脾脏病变病例中考虑SANT的重要性。这一贡献为现有知识增添了有价值的内容，促使在涉及脾脏异常的诊断场景中对SANT进行进一步研究和考虑。
