Pulmonary pattern in systemic vasculitis: granulomatosis, lung cancer or both?

Abstract

As clinical manifestations of systemic vasculitides share many common features with other conditions, the rate of diagnostic errors and delayed diagnoses is high. Hence we performed an analysis of the available data regarding misdiagnosis of vasculitis as lung cancer and vice versa, as well as coexistence of vasculitis and lung cancer. The present case-based review highlights the diagnostic challenges encountered when granulomatosis with polyangiitis (GPA) mimics lung cancer. The importance of a multidisciplinary team approach to patients with pulmonary involvement and multisystem manifestations is essential for appropriate planning of further diagnostic steps and for minimizing the delay in correct diagnosis and treatment. In the present case, although computed tomography raised suspicion for lung cancer, further biopsies and laboratory screening for systemic vasculitides rejected malignancy and confirmed the diagnosis of GPA.

Keywords: granulomatosis with polyangiitis; lung cancer; misdiagnosis; systemic vasculitis.

Fig. 1

The Preferred Reporting Items for Systematic Reviews and Meta-Analyses flow diagram of searching methods.

Fig. 2

Appearance of the patient at presentation: a photo demonstrating episcleritis, proptosis, nose deformity (A). The photo is used with the patient’s consent. Computed tomography images demonstrating osteolytic changes of nasal bones and of the walls of paranasal sinuses (B, C).

Fig. 3

Computed tomography scans demonstrating a mass in segment 3 in the right lung (A), a cavitation in segment 10 (B), pulmonary nodules of polygonal shape with peribronchovascular and peripheral localization (C), and a mass in segment 10 of the right lung (D).

Fig. 4

Computed tomography scans (A–C) and positron-emission tomography images (D–F) demonstrating narrowing of the trachea and subglottic stenosis (arrows).

Fig. 5

Lung tissue with accumulation of fluid, fibrin, and squamous cells in some of the alveolar spaces and focal carnification (asterisk). A multinucleated giant cell is seen (arrow). Hematoxylin and eosin staining, × 200 (A). Fibrosis of the interalveolar septa, infiltrated by lymphocytes, histiocytes/macrophages, plasma cells, eosinophilic leukocytes and monocytes. Hematoxylin and eosin staining, × 200 (B).