European Journal of Internal Medicine 25 (2014) e115–e116
Contents lists available at ScienceDirect
European Journal of Internal Medicine journal homepage: www.elsevier.com/locate/ejim
Internal Medicine Flashcard
Dyspnea in crackling lungs Luca Novelli, Stefano Aliberti ⁎, Alberto Pesci Health Science Department, University of Milan-Bicocca, Respiratory Unit, San Gerardo Hospital, via Pergolesi 33, 20900 Monza, Italy
a r t i c l e
i n f o
Article history: Received 1 May 2014 Received in revised form 31 May 2014 Accepted 13 June 2014 Available online 4 July 2014
1. Indication A 60-year-old man, former smoker (40 packs/year), retired metalworker, with no exposition to asbestos or other pathogens, referred to his general practitioner (GP) because of dyspnea and recurrent dry cough for 3 months. His medical history was unremarkable except for gastro-esophageal reflux disease (GERD). At physical examination, digital clubbing was present and GP noted bibasal inspiratory crackles on chest. Patient denied any arthralgic symptoms. Oxygen saturation on room air was 96%. Chest X-ray showed a diffuse parenchymal lung disease (DPLD). Body plethysmography showed a forced vital capacity (FVC) of 70% pred., forced expiratory volume at 1 s (FEV1) of 77% pred., FEV1/FVC ratio of 0.87, total lung capacity of 67% pred. and residual volume of 72% pred. Transfer lung of CO was 59% pred. Highresolution computed tomography (HRCT) of the chest confirmed a DPLD with an apico-basal gradient and honeycombing pattern, see Fig. 1. Serum immunology was negative. Bronchoalveolar lavage indicated increased neutrophils and eosinophils counts.
What is the diagnosis? Idiopathic pulmonary fibrosis.
Fig. 1. Subpleural and basal clustered cystic airspaces (honeycombing), associated with traction bronchiectasis.
⁎ Corresponding author. Tel.: +39 0392339284; fax: +39 0392339437. E-mail address: [email protected] (S. Aliberti).
http://dx.doi.org/10.1016/j.ejim.2014.06.011 0953-6205/© 2014 European Federation of Internal Medicine. Published by Elsevier B.V. All rights reserved.
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L. Novelli et al. / European Journal of Internal Medicine 25 (2014) e115–e116
2. Diagnosis The patient was diagnosed with idiopathic pulmonary fibrosis (IPF). IPF is defined as a chronic, progressive fibrosing interstitial pneumonia of unknown cause, occurring primarily in older adults, limited to the lungs, and associated with a histopathologic and radiologic pattern of usual interstitial pneumonia (UIP) [1]. The exclusion of other forms of DPLD associated with environmental exposure, medications, or systemic disease (i.e. connectivitis) is crucial. Smoking is strongly associated with IPF and recent studies suggest that GERD is also a risk factor [1–3]. The presence of “velcro-like” crackles together with dyspnea and recurrent cough may suggest the presence of IPF and the need of further investigations [3]. A confident UIP-pattern is characterized by the presence of subpleural and basal reticular opacities on chest HRCT, associated with traction bronchiectasis, see Figure in [1]. Honeycombing (clustered cystic airspaces) is common and is critical for a definite diagnosis. HRCT is highly accurate to detect UIP on histopathology. A compatible clinical history associated to a confident UIP-pattern on chest
HRTC along with the exclusion of other DPLD allows to avoid lung biopsy [1]. IPF has a poor prognosis with a delay of diagnosis of 2.2 years. Recently, pirfenidone has been registered as possible therapy with an effect in limiting the decline of lung function in patients with mild-tomoderate IPF [2]. Conflict of interests No conflict of interest. References [1] An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidencebased guidelines for diagnosis and management. Am J Respir Crit Care Med 2011;183:788–824. [2] Noble PW, Albera C, Bradford WZ, Costabel U, Glassberg MK, Kardatzke D, et al. Pirfenidone in patients with idiopathic pulmonary fibrosis (CAPACITY): two randomised trials. Lancet 2011;377:1760–9. [3] Cottin V, Cordier JF. Velcro crackles: the key for early diagnosis of idiopathic pulmonary fibrosis? Eur Respir J 2012;40(3):519–21.
Contents lists available at ScienceDirect
European Journal of Internal Medicine journal homepage: www.elsevier.com/locate/ejim
Internal Medicine Flashcard
Dyspnea in crackling lungs Luca Novelli, Stefano Aliberti ⁎, Alberto Pesci Health Science Department, University of Milan-Bicocca, Respiratory Unit, San Gerardo Hospital, via Pergolesi 33, 20900 Monza, Italy
a r t i c l e
i n f o
Article history: Received 1 May 2014 Received in revised form 31 May 2014 Accepted 13 June 2014 Available online 4 July 2014
1. Indication A 60-year-old man, former smoker (40 packs/year), retired metalworker, with no exposition to asbestos or other pathogens, referred to his general practitioner (GP) because of dyspnea and recurrent dry cough for 3 months. His medical history was unremarkable except for gastro-esophageal reflux disease (GERD). At physical examination, digital clubbing was present and GP noted bibasal inspiratory crackles on chest. Patient denied any arthralgic symptoms. Oxygen saturation on room air was 96%. Chest X-ray showed a diffuse parenchymal lung disease (DPLD). Body plethysmography showed a forced vital capacity (FVC) of 70% pred., forced expiratory volume at 1 s (FEV1) of 77% pred., FEV1/FVC ratio of 0.87, total lung capacity of 67% pred. and residual volume of 72% pred. Transfer lung of CO was 59% pred. Highresolution computed tomography (HRCT) of the chest confirmed a DPLD with an apico-basal gradient and honeycombing pattern, see Fig. 1. Serum immunology was negative. Bronchoalveolar lavage indicated increased neutrophils and eosinophils counts.
What is the diagnosis? Idiopathic pulmonary fibrosis.
Fig. 1. Subpleural and basal clustered cystic airspaces (honeycombing), associated with traction bronchiectasis.
⁎ Corresponding author. Tel.: +39 0392339284; fax: +39 0392339437. E-mail address: [email protected] (S. Aliberti).
http://dx.doi.org/10.1016/j.ejim.2014.06.011 0953-6205/© 2014 European Federation of Internal Medicine. Published by Elsevier B.V. All rights reserved.
e116
L. Novelli et al. / European Journal of Internal Medicine 25 (2014) e115–e116
2. Diagnosis The patient was diagnosed with idiopathic pulmonary fibrosis (IPF). IPF is defined as a chronic, progressive fibrosing interstitial pneumonia of unknown cause, occurring primarily in older adults, limited to the lungs, and associated with a histopathologic and radiologic pattern of usual interstitial pneumonia (UIP) [1]. The exclusion of other forms of DPLD associated with environmental exposure, medications, or systemic disease (i.e. connectivitis) is crucial. Smoking is strongly associated with IPF and recent studies suggest that GERD is also a risk factor [1–3]. The presence of “velcro-like” crackles together with dyspnea and recurrent cough may suggest the presence of IPF and the need of further investigations [3]. A confident UIP-pattern is characterized by the presence of subpleural and basal reticular opacities on chest HRCT, associated with traction bronchiectasis, see Figure in [1]. Honeycombing (clustered cystic airspaces) is common and is critical for a definite diagnosis. HRCT is highly accurate to detect UIP on histopathology. A compatible clinical history associated to a confident UIP-pattern on chest
HRTC along with the exclusion of other DPLD allows to avoid lung biopsy [1]. IPF has a poor prognosis with a delay of diagnosis of 2.2 years. Recently, pirfenidone has been registered as possible therapy with an effect in limiting the decline of lung function in patients with mild-tomoderate IPF [2]. Conflict of interests No conflict of interest. References [1] An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidencebased guidelines for diagnosis and management. Am J Respir Crit Care Med 2011;183:788–824. [2] Noble PW, Albera C, Bradford WZ, Costabel U, Glassberg MK, Kardatzke D, et al. Pirfenidone in patients with idiopathic pulmonary fibrosis (CAPACITY): two randomised trials. Lancet 2011;377:1760–9. [3] Cottin V, Cordier JF. Velcro crackles: the key for early diagnosis of idiopathic pulmonary fibrosis? Eur Respir J 2012;40(3):519–21.
