[Combined pulmonary fibrosis and emphysema associated with microscopic polyangiitis].
BACKGROUND
Combined pulmonary fibrosis and emphysema (CPFE) is a rare entity of unknown etiology. It usually occurs in the context of smoking and, less commonly, connective tissue disease. However, it has been rarely previously described in the context of vasculitis.


METHODS
We report a case of CPFE occurring in a 44-year-old man, who was a light smoker without any previous medical history. He presented with fever, chronic cough and breathlessness that progressively evolved to acute respiratory failure. At the initial evaluation, CT scan showed emphysema and patchy bilateral areas of ground-glass opacity. Three years later, the patient simultaneously developed a honeycomb fibrosis and a microscopic polyangiitis with renal involvement justifying the introduction of an immunosuppressive treatment in combination with high dose of systemic corticosteroids. After a stabilization period of 6years, the patient gradually developed chronic respiratory failure with moderate pulmonary hypertension requiring long-term oxygen therapy and nocturnal non-invasive ventilation.


CONCLUSIONS
The association of microscopic polyangiitis to CFPE suggests that autoimmune diseases may have a common pathogenic role in the development of emphysematous and fibrotic lesions in CPFE.
Service de pneumo-allergologie, CHU Hédi-Chaker, faculté de médecine de Sfax, route al Ain, Km 0,5, 3029 Sfax, Tunisie. Electronic address: msaadsameh@yahoo.fr.
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M'Saad
Service de néphrologie, CHU Hédi-Chaker, faculté de médecine de Sfax, Sfax, Tunisie; Unité de pathologie rénale, UR 12ES14, faculté médecine de Sfax, Sfax, Tunisie.
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Kammoun
Service de pneumo-allergologie, CHU Hédi-Chaker, faculté de médecine de Sfax, route al Ain, Km 0,5, 3029 Sfax, Tunisie.
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Yangui
Service d'imagerie médicale, CHU Hédi-Chaker, faculté de médecine de Sfax, Sfax, Tunisie.
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Fourati
Service de pneumo-allergologie, CHU Hédi-Chaker, faculté de médecine de Sfax, route al Ain, Km 0,5, 3029 Sfax, Tunisie.
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Feki
Service de pneumo-allergologie, CHU Hédi-Chaker, faculté de médecine de Sfax, route al Ain, Km 0,5, 3029 Sfax, Tunisie.
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Marouen
Service d'imagerie médicale, CHU Hédi-Chaker, faculté de médecine de Sfax, Sfax, Tunisie.
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Daoud
Service de pneumo-allergologie, CHU Hédi-Chaker, faculté de médecine de Sfax, route al Ain, Km 0,5, 3029 Sfax, Tunisie.
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Kammoun
D000328:Adult; D006801:Humans; D008297:Male; D055953:Microscopic Polyangiitis / Q000150:complications* / Q000175:diagnosis; D011656:Pulmonary Emphysema / Q000175:diagnosis / Q000209:etiology*; D011658:Pulmonary Fibrosis / Q000175:diagnosis / Q000209:etiology*
Anticorps anti-myéloperoxydase; Antimyeloperoxidase antibodies; Combined pulmonary fibrosis and emphysema; Ground-glass opacity; Micropolyangéite; Microscopic polyangiitis; Opacités en verre dépoli; Syndrome d’emphysème des sommets et fibrose pulmonaire des bases; Vascularite; Vasculitis