Combined pulmonary fibrosis and emphysema syndrome in systemic sclerosis

SUMMARYCombined pulmonary fibrosis and emphysema is a distinct syndrome reported in patients who smoke. A 72-year-old, never-smoking female dairy farmer was referred for progressive dyspnoea on exertion, basal crackles on auscultation, normal spirometry and normal lung volumes but decreased diffusing capacity of the lung for carbon monoxide, centrilobular emphysema in the upper zones of the lungs and diffuse infiltrative lung disease in the lower zones on high-resolution CT scan. Bronchoalveolar lavage differential cell count showed 35% lymphocytosis, and precipitating antibodies for Wallemia sebi, Trichoderma species and Cladosporium sphaerospermum were identified. The diagnosis of farmer's lung disease with combined pulmonary fibrosis and emphysema was retained. This case highlights for the first time that hypersensitivity pneumonitis should be suspected in the setting of combined pulmonary fibrosis and emphysema in non-smoking patients. BACKGROUND

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“…Although combined pulmonary fibrosis and emphysema is mainly due to tobacco smoking, it has also been reported in non-smoking patients 7 8…”

Section: Discussionmentioning

confidence: 99%

Combined pulmonary fibrosis and emphysema in hypersensitivity pneumonitis

et al. 2015

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“…3 When CPFE was first described, patients with ILDs other than interstitial pulmonary fibrosis( IPF) were excluded from the study. 1 Later on, CPFE was described in patients with other ILDs, such as connective tissue disease (CTD) associated ILD, [4][5][6][7] as well as in patients with microscopic polyangiitis .8 The fact that IPF has the worst prognosis in relation to other chronic lung fibrotic diseases, it is important to establish the interstitial lung disease that constitutes the fibrotic component of CPFE. Patients with CPFE are predominantly male, with a history of heavy tobacco exposure, and usually present with severe breathlessness and cough.…”

Section: Introductionmentioning

confidence: 99%

Combined pulmonary fibrosis and emphysema (CPFE)

Khanam

2017

Bangladesh Crit Care J

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Combined pulmonary fibrosis and emphysema (CPFE) is rare but increasingly recognized condition characterized by simultaneous coexistence of both upper lobe predominant emphysema and diffuse pulmonary fibrosis mainly in lower lobe. Patients with CPFE are usually heavy smokers or former smokers. HRCT has a pivotal role in diagnosis. Pulmonary function test showed relatively preserved lung volumes and reduced diffusing capacity of the lung for carbon monoxide (DLCO). Development of pulmonary hypertension (PH) is largely attributed to morbidity in patients with CPFE which is the principal prognostic factor for this condition. However more studies are needed to establish natural history of the disease & treatment option. In this review, we will discuss the current knowledge of the pathogenesis, clinical characteristics, treatment options and prognostic factors of CPFE.Bangladesh Crit Care J September 2017; 5(2): 122-125

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The Syndrome of Combined Pulmonary Fibrosis and Emphysema

Cottin

Cordier

2014

Orphan Lung Diseases

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Combined pulmonary fibrosis and emphysema syndrome in systemic sclerosis

Cited by 6 publications

References 6 publications

Combined pulmonary fibrosis and emphysema in hypersensitivity pneumonitis

Combined pulmonary fibrosis and emphysema in hypersensitivity pneumonitis

Combined pulmonary fibrosis and emphysema (CPFE)

The Syndrome of Combined Pulmonary Fibrosis and Emphysema

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