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Combined Pulmonary Fibrosis and Emphysema (CPFE) in a Connective Disease
March 2014, Vol 145, No. 3_MeetingAbstracts Diffuse Lung Disease | March 2014
Combined Pulmonary Fibrosis and Emphysema (CPFE) in a Connective Disease Olivier Gilbert, MD; Alain Van Meerhaeghe, MD; Myriam Remmelink, MD; Benjamin Bondue, MD; Matteo Cappello, MD; Paul Devuyst, MD; Philippe Pierard, MD CHU of Charleroi, Charleroi, Belgium
Chest. 2014;145(3_MeetingAbstracts):216A. doi:10.1378/chest.1835476
Abstract SESSION TITLE: ILD Case Report Posters II SESSION TYPE: Case Report Poster PRESENTED ON: Sunday, March 23, 2014 at 01:15 PM - 02:15 PM INTRODUCTION: A 35-year-old African patient goes to pneumologist because of abnormal chest X-ray (during tuberculosis screening at work). CASE PRESENTATION: The CT scan showed extensive emphysema bubbles and sub pleural interstitial disease. The patient is non-smoking and serum 1- alpha antrypsine is normal. Raynaud's phenomenon is present for many years and exertional dyspnea (Sadoul 2). Pulmonary auscultation revealed no crackles. Laboratory tests showed a serum antinuclear factor (FAN) to 1/640 with a specific anti SCL 70 ( 117U) suggesting scleroderma. Lung volumes can be described as normal, but the diffusion capacity is deeply lowered ( DLCO 41 % PV). The walk test six-minute walk, the patient desaturated so deeply (SaO2 97% at rest and 79 % at the end of effort with an evaluation of 5 dyspnea on the Borg scale). This presentation is not usual, it was decided to conduct lung biopsies by VAT. Histological examination shows real damage emphysema but also nonspecific infiltrative lung. DISCUSSION: In the literature, the concomitant presence of emphysema in patients, nonsmokers, with systemic sclerosis has been described. A murine model that has a duplication of the fibrillin 1 gene resembles those observations. The possibility of abnormalities of surfactant proteins has been reported in the CPFE. Of course, in this case, environmental origin emphysema may also be suspected (use of wood-burning oven in Africa). Treatment of cellular NSIP is
atypical with scleroderma because of the risk of «renal crisis» induced by high corticoid dosis. It consists of low doses of cortisone associated with intravenous cyclophosphamid. CONCLUSIONS: The diagnosis of cellular NSIP in scleroderma is confirmed and Combined pulmonary fibrosis and emphysema (CPFE) syndrome is identified in a non smoker african female. Reference #1: Curr Opin Pulm Med 2012 sep; 18 (5): 418-27. Combined pulmonary fibrosis and emphysema in connective tissue disease. Cottin V, Cordier JE. Reference #2: Arthritis Rheum. 2011; 63 (1): 295-304. Cottin V, Nunes H, Mouthon L, et al. Groupes d’Etudes et de recherche sur les maladies « Orphelines » Pulmonaires. Combined pulmonary fibrosis and emphysema syndrome in connective tissue disease. Reference #3: Am Rev Respir Dis 1981 ; 123 : 680-5. Szapiel SV, FulmerJD, HunninghakeGW, Elson NA, KawanamiO, Ferrans VJ et al. Hereditary emphysema in the tight-skin. DISCLOSURE: The following authors have nothing to disclose: Olivier Gilbert, Alain Van Meerhaeghe, Myriam Remmelink, Benjamin Bondue, Matteo Cappello, Paul Devuyst, Philippe Pierard No Product/Research Disclosure Information
Combined Pulmonary Fibrosis and Emphysema (CPFE) in a Connective Disease Olivier Gilbert, MD; Alain Van Meerhaeghe, MD; Myriam Remmelink, MD; Benjamin Bondue, MD; Matteo Cappello, MD; Paul Devuyst, MD; Philippe Pierard, MD CHU of Charleroi, Charleroi, Belgium
Chest. 2014;145(3_MeetingAbstracts):216A. doi:10.1378/chest.1835476
Abstract SESSION TITLE: ILD Case Report Posters II SESSION TYPE: Case Report Poster PRESENTED ON: Sunday, March 23, 2014 at 01:15 PM - 02:15 PM INTRODUCTION: A 35-year-old African patient goes to pneumologist because of abnormal chest X-ray (during tuberculosis screening at work). CASE PRESENTATION: The CT scan showed extensive emphysema bubbles and sub pleural interstitial disease. The patient is non-smoking and serum 1- alpha antrypsine is normal. Raynaud's phenomenon is present for many years and exertional dyspnea (Sadoul 2). Pulmonary auscultation revealed no crackles. Laboratory tests showed a serum antinuclear factor (FAN) to 1/640 with a specific anti SCL 70 ( 117U) suggesting scleroderma. Lung volumes can be described as normal, but the diffusion capacity is deeply lowered ( DLCO 41 % PV). The walk test six-minute walk, the patient desaturated so deeply (SaO2 97% at rest and 79 % at the end of effort with an evaluation of 5 dyspnea on the Borg scale). This presentation is not usual, it was decided to conduct lung biopsies by VAT. Histological examination shows real damage emphysema but also nonspecific infiltrative lung. DISCUSSION: In the literature, the concomitant presence of emphysema in patients, nonsmokers, with systemic sclerosis has been described. A murine model that has a duplication of the fibrillin 1 gene resembles those observations. The possibility of abnormalities of surfactant proteins has been reported in the CPFE. Of course, in this case, environmental origin emphysema may also be suspected (use of wood-burning oven in Africa). Treatment of cellular NSIP is
atypical with scleroderma because of the risk of «renal crisis» induced by high corticoid dosis. It consists of low doses of cortisone associated with intravenous cyclophosphamid. CONCLUSIONS: The diagnosis of cellular NSIP in scleroderma is confirmed and Combined pulmonary fibrosis and emphysema (CPFE) syndrome is identified in a non smoker african female. Reference #1: Curr Opin Pulm Med 2012 sep; 18 (5): 418-27. Combined pulmonary fibrosis and emphysema in connective tissue disease. Cottin V, Cordier JE. Reference #2: Arthritis Rheum. 2011; 63 (1): 295-304. Cottin V, Nunes H, Mouthon L, et al. Groupes d’Etudes et de recherche sur les maladies « Orphelines » Pulmonaires. Combined pulmonary fibrosis and emphysema syndrome in connective tissue disease. Reference #3: Am Rev Respir Dis 1981 ; 123 : 680-5. Szapiel SV, FulmerJD, HunninghakeGW, Elson NA, KawanamiO, Ferrans VJ et al. Hereditary emphysema in the tight-skin. DISCLOSURE: The following authors have nothing to disclose: Olivier Gilbert, Alain Van Meerhaeghe, Myriam Remmelink, Benjamin Bondue, Matteo Cappello, Paul Devuyst, Philippe Pierard No Product/Research Disclosure Information