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Granulomatosis with Polyangiitis initially misdiagnosed as lung cancer

Granulomatosis with Polyangiitis initially misdiagnosed as lung cancer

Abstract

Granulomatosis with Polyangiitis (GPA), which was formerly named Wegener's Granulomatosis (WG) is a systemic disease characterized by necrotizing granulomatous inflammation and vasculitis that primarily involves upper and lower respiratory tract, as well as kidneys. Diagnosing GPA on the basis of transthoracic fine needle aspiration (TFNA) may be problematic, as it can be misdiagnosed as cancer. We describe a patient with a probable GPA which was originally diagnosed as malignancy, but who responded to lung cancer chemotherapy.

Keywords: Systemic vasculitis. Non-small-cell lung carcinoma. Fine-needle biopsy.

Full Text


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Introduction

Granulomatosis with Polyangiitis (GPA), more frequently called Wegener's Granulomatosis (WG) is a systemic disease characterized by necrotizing granulomatous inflammation and vasculitis that primarily involves upper and lower respiratory tract, as well as kidneys. It can sometimes present as solitary or multifocal pulmonary lesions, without signs of extrapulmonary involvement.

Diagnosing GPA on the basis of transthoracic fine needle aspiration (TFNA) may be problematic, as it can be misdiagnosed as cancer. We describe a patient with GPA originally diagnosed as malignancy.

Case history

A 45-year-old man, 20 pack-year smoker, diagnosed with stage IV non-small cell lung cancer (NSCLC), by TFNA, underwent 6 cycles of chemotherapy with carboplatinum and gemcitabin, and complete remission was observed after 3 cycles (Figure 1). At that time the cytological studies were reviewed, the diagnosis of NSCLC was maintained and antineutrophil cytoplasmic antibodies (ANCA) performed were negative. He was kept under surveillance by his Pulmonary Oncology team.

Figure 1. Chest CT at the time of diagnosis (A) and 3 months after chemotherapy (B). (A) Multiple nodular lesions in both lungs. The bigger lesions present in the apices. (B) Resolution of the nodular lesions after 3 cycles of chemotherapy. Some fibrotic sequelar changes can be seen in both apical regions.

After an asymptomatic period of 15 months, with no signs of recurrence, he started having exertional dyspnea, dry cough, anorexia and weight loss (3kg over a month). Physical examination was unremarkable. Chest X-ray showed multiple nodules and masses in both lungs some of which exhibited cavitation, as confirmed by Chest CT (Figure 2A and B).

Figure...


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S. Campainha a, , M. Gonçalves b, V. Tavares b, P. Castelões b, A. Marinho c, S. Neves a,

a Serviço de Pneumologia, Centro Hospitalar de Vila Nova de Gaia/Espinho EPE, Vila Nova de Gaia, Portugal
b Unidade de Cuidados Intensivos Polivalente, Centro Hospitalar de Vila Nova de Gaia/Espinho EPE, Vila Nova de Gaia, Portugal
c Departamento de Doenças Auto-imunes do Serviço de Medicina Interna, Centro Hospitalar do Porto, Porto, Portugal
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