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A New Clinical Scenario: the Presence of Neutrophil Anti-Cytoplasm Antibodies in the Combination of Pulmonary Fibrosis and Emphysema (CFPE)

Jose Salvador García-Morillo, David Blanco Alba, Santiago Rodríguez Suárez

Combination of pulmonary fibrosis and emphysema (CFPE) is an entity defined in last years, with tobacco as the main etiological factor. Recent studies have associated it with connective tissue diseases and autoimmunity phenomena. The objective of this case series is to review some key aspects of CFPE from the diagnostic and therapeutic point of view, as well as its association with autoimmune diseases. This is a case series of three patients with CPFE associated with ANCA positive type MPO vasculitis.

We present three elderly male patients with important accumulated tobacco consumption who initially presented non-specific general and pulmonary symptom. They had elevated acute phase reactants and positive ANCA antibodies type MPO in blood. HRCT revealed areas of emphysema in the upper lobes and fibrosis in the lower ones . Initially, both patients presented normal result in spirometry but a marked decrease of DLCO. In the three cases biopsies were compatible with medium-vessel vasculitis. We apply corticosteroids associated with immunosuppressant therapy, obtaining different results in each cases.