Gavica Jenifer Centeno, Raymond Leslie
Department of Internal Medicine NCH Healthcare System Naples Florida USA.
Clin Case Rep. 2023 Sep 27;11(10):e7972. doi: 10.1002/ccr3.7972. eCollection 2023 Oct.
Vasculitis is a multisystemic disease that affects vessels of different sizes. Its presentation can vary widely depending on the system involved. It may present with constitutional symptoms or with specific features of end-organ involvement. The diagnosis is built on a compatible pattern of clinical features supported by specific serological or radiological investigations and confirmatory biopsy. Tissue biopsy is vital to confirm the diagnosis of vasculitis; however, this should not delay treatment when presentation strongly suggests vasculitis. We describe a case of a 72-year-old man treated with steroids, plasma exchange, and rituximab for suspected granulomatosis with polyangiitis (GPA) given his clinical presentation including suspected scleritis of the right eye, hearing changes, sinusitis, diffuse alveolar hemorrhage, pulmonary lesions, kidney failure, palpable purpura, and radiological evidence of pansinusitis and pulmonary lesions without waiting for serology or tissue confirmation. This case highlights the importance of recognizing the clinical features of GPA to initiate prompt treatment as it can progress rapidly and be fatal.
血管炎是一种影响不同大小血管的多系统疾病。其表现因受累系统的不同而有很大差异。它可能表现为全身症状或终末器官受累的特定特征。诊断基于特定血清学或影像学检查以及确诊活检所支持的符合临床特征的模式。组织活检对于确诊血管炎至关重要；然而，当临床表现强烈提示血管炎时，不应因此而延迟治疗。我们描述了一例72岁男性病例，鉴于其临床表现包括疑似右眼巩膜炎、听力改变、鼻窦炎、弥漫性肺泡出血、肺部病变、肾衰竭、可触及的紫癜以及全鼻窦炎和肺部病变的影像学证据，在未等待血清学检查或组织确诊的情况下，就给予其类固醇、血浆置换和利妥昔单抗治疗，以怀疑患有肉芽肿性多血管炎（GPA）。该病例突出了认识GPA临床特征以启动及时治疗的重要性，因为它可能迅速进展并导致死亡。
