Thoracic Manifestation of Granulomatosis with Polyangiitis: A Case Report
Granulomatosis with polyangiitis (GPA) is an antineutrophil cytoplasmic antibody (ANCA)-associated disorder with necrotic vasculitis of small- and medium-size arteries and veins. In the literature, there are many case reports of patients with GPA of diffe
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Thoracic Manifestation of Granulomatosis with Polyangiitis: A Case Report Bartosz Tomczyk, Zuzanna Janeczko, Adrianna Kruczkowska, Beata Maciążek-Chyra, Wojciech Tański, and Mariusz Chabowski Abstract
Granulomatosis with polyangiitis (GPA) is an antineutrophil cytoplasmic antibody (ANCA)associated disorder with necrotic vasculitis of small- and medium-size arteries and veins. In the literature, there are many case reports of patients with GPA of different, sometimes unusual, clinical manifestations. In this paper, we present difficulties that accompanied the process of diagnosing GPA in a 54-year-old symptomatic patient who was. Computer tomography scans showed numerous tumor-
B. Tomczyk, Z. Janeczko, and A. Kruczkowska (*) Student Research Club No 180, Faculty of Medicine, Wroclaw Medical University, Wrocław, Poland e-mail: [email protected] B. Maciążek-Chyra Department of Rheumatology and Internal Medicine, Wroclaw Medical University, Wrocław, Poland W. Tański Department of Internal Medicine, Fourth Military Teaching Hospital, Wrocław, Poland M. Chabowski Division of Oncology and Palliative Care, Department of Clinical Nursing, Faculty of Health Science, Wrocław Medical University, Wrocław, Poland Department of Surgery, Fourth Military Teaching Hospital, Wrocław, Poland
like lesions of various and irregular sizes in both lungs. Positron emission tomography scans suggested a lymphoproliferative disease, otherwise failing to provide a clue concerning its nature or localization. After a series of diagnostic twists and turns, inclusive of bronchoalveolar lavage, cervical mediastinoscopy, paratracheal lymph biopsy, and histopathologic examinations, and other tests, the diagnosis of GPA was established as the most probable. The patient was acutely treated with loading doses of methylprednisolone and cyclophosphamide, gradually tapered off during the long-term follow-up. He was discharged from the hospital in a good condition. We conclude that GPA is an uncommon disease with indistinctive signs, which raises the risk of its being overlooked. A diagnostic algorithm is required for patients with suspected GPA. A timely diagnosis is essential as the disease may quickly progress into renal or multiorgan dysfunction, and ultimately lead to death if untreated. Pulmonary involvement may also suggest neoplastic changes.
B. Tomczyk et al.
Keywords
Antineutrophil cytoplasmic antibody · Granulomatosis with polyangiitis · Lung lesions · Nodules · Vasculitis
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presence of two or more abnormalities is associated with a high possibility of the disease. ANCA antibodies play a role in recognition but are not decisive in the diagnostic process due to relatively low sensitivity. Herein we report diagnosing difficulties concerning the GPA.
Introduction 2
Granulomatosis with polyangiitis (GPA, Wegener’s granulomatosis) is an antineutrophil cytoplasmic antibody (ANCA)-associated disorder with necrotic vasculitis of small- and medium-size arteries and veins. The course of illness differs between the patients, but in most cas
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