Case of combined pulmonary fibrosis with emphysema(CPFE) and its importance to recognize Abstract ID -1188

72yrs male Chronic smoker Presented with complaints of cough with breathlessness for last 3months PFT: – Near normal spirometry and lung volume – Reduced diffusion capacity

Chest Radiograph: Bilateral linear and reticular opacities Sub pleural cystic changes Normal lung volume

NCCT CHEST Bilateral upper lobe paraseptal and centriacinar emphysematous changes Multiple upper lobe bullae Basal and sub pleural predominance of inter lobar septal thickening,honey combing and traction bronchiectesis s/o Fibrosis

NCCT CHEST Axial section –Upper lobe Paraseptal and centriacinar emphysema with bulla

Axial section –Lower lobe fibrosis Axial section-Lower lobe fibrosis

Coronol section-Upper lobe emphysema and lower lobe fibrosis

Diagnosis On the basis of patient clinical history, PFT findings and HRCT findings of Upper lobe emphysematous changes and lower lobe fibrosis, the diagnosis of combined pulmonary fibrosis with emphysema has been established

Discussion Possible new addition to a growing list of smoking related lung disease characterized by the coexistence of UIP or NSIP with emphysema in smokers. Epidemiology(1): – 5-10% of cases of diffuse interstitial lung disease Pathology(4) : – Pathogenesis and exact association is yet to be elucidated in larger studies. – It is important to be aware of probable inherent susceptibility of some patient to pulmonary fibrosis from cigarette smoking

Clinical features (4): – Male – Tobacco smoking – Severe dyspnoea – Unexpected subnormal spirometry findings – Severely impaired transfer capacity for carbon monoxide – hypoxaemia on exercise

Three distinct patterns have been described in recent literature – Progressive transition with diffuse emphysema (centrilobular and/or bullous) and zone of transition between bullae and honeycombing – Paraseptal emphysema with predominant sub pleural bullae of enlarging size at the base – Separate processes with independent areas of fibrosis and emphysema(4,5)

Importance to recognize Development of pulmonary hypertension is higher in those patients with CPFE than in those with IPF without emphysema(1,2). The therapeutic options for CPFE are limited. Systemic corticosteroids and immunomodulator therapy, similar to that for treating IPF, has been used, but without beneficial results in the published series.(3,4) Prognosis will be grave than isolated IPF/Emphysema

Importance to recognize Possibility of using the specific therapy approved for treating pulmonary arterial hypertension (endothelin-1 receptor antagonists, prostanoids, or phosphodiesterase type 5 inhibitors), as has been tested in COPD or IPF, has been considered by some authors. It is important to point out that the presence of emphysema and abnormal changes in pulmonary vascular bed, as hypoxic vasoconstriction, is one of the main mechanisms to avoid worsening arterial oxygenation. These vasodilator drugs can worsen hypoxemia by inhibiting this mechanism. (4,5) However, no studies have been published to date on this issue. Thus, appropriately designed trials are necessary to study the effect of these drugs on gas exchange in these patients.

References 1.Cottin V, Cordier JF. The syndrome of combined pulmonary fibrosis and emphysema. Chest. 2009;136 (1): 1-2. doi: /chest Jankowich MD, Rounds SI. Combined pulmonary fibrosis and emphysema syndrome: a review. Chest. 2012;141 (1): doi: /chest Cottin V, Nunes H, Brillet PY et-al. Combined pulmonary fibrosis and emphysema: a distinct underrecognised entity. Eur. Respir. J. 2005;26 (4): Portillo K, Morera J. Combined Pulmonary Fibrosis and Emphysema Syndrome: A New Phenotype within the Spectrum of Smoking-Related Interstitial Lung Disease. Pulm Med. 2012;2012 : Kitaguchi Y, Fujimoto K, Hayashi R et-al. Annual changes in pulmonary function in combined pulmonary fibrosis and emphysema: over a 5-year follow-up. Respir Med. 2013;107 (12):

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