Journal de l'hypertension : accès libre

Granulomatosis with Polyangiitis Presenting as Postoperative Gastrointestinal Stromal Tumors: A Case Report

Abstract

Ye Rui-hai, Yang Li, Yang Jun-chao, Mao Yu-ping, Zhang Chun-li, Xu Jun, Zhu Yuan-hong and Wang Zhen

Introduction: Granulomatosis with polyangiitis (GPA) is a systemic vasculitis syndrome, previously known as Wegener’s granulomatosis, characterized by necrotizing granulomatous inflammation of the respiratory tracts, systemic necrotizing vasculitis, and necrotizing glomerulonephritis. This disorder represents a significant diagnostic dilemma due its diverse presentations, although presentation in the form of postoperative gastrointestinal stromal tumors is uncommon. Here, we report one of the first identified cases of GPA presenting as postoperative gastrointestinal stromal tumors (GISTs).

Case presentation: A 78-year-old Chinese man presented to our Respiratory Department in Zhejiang Hospital of Traditional Chinese Medicine with a history of progressively worsening fever and pulmonary nodules. He had history of postoperative gastrointestinal stromal tumors. Because of failed treatment with antibiotics, he had been diagnosed with a fungal infection of the lung, which was empirically treated with voriconazole for approximately two months. However, the condition did not improve until the patient was successfully treated with moderate-dose steroids. His clinical course was fever, pulmonary nodules and nasosinusitis, which are associated with granulomatosis with polyangiitis.

Conclusion: The recognition of a multisystem disease is critical for diagnosing GPA. Postoperative GISTs may be associated with manifestations of the disease, in which case, immediate and aggressive treatment with steroids can be life-saving.