Volume 4, Issue 3, March – 2019 International Journal of Innovative Science and Research Technology

ISSN No:-2456-2165

A Case Series on Combined Pulmonary Fibrosis and

Emphysema
Amina Jabin A N*1, Iram Naz Ansari1, Deepika R1, Anjana Sankar A J1, Kesiya Simon1, Nikhil M1, Safna N Fazil2
5th year Pharm D1, Assistant Professor2,
Department of Pharmacy Practice, The Dale View College of Pharmacy and Research Centre, Thiruvananthapuram, Kerala, India

Abstract:- In 2005, Cottin et al., put forth a term,  CASE 1

Combined Pulmonary Fibrosis and Emphysema (CPFE) A 74-year-old female patient was admitted in the
which is a rare respiratory disorder and is characterized respiratory department of a tertiary care hospital. The patient
by exertional dyspnoea, upper-lobe emphysema and had complaints of cough with sputum, wheezing, chest
lower-lobe fibrosis, preserved lung volume and severely congestion for 1 month. The patient’s history showed that she
diminished capacity of gas exchange.[1][2] The main was exposed to biomass fuel and had dust allergy for several
etiologies behind CPFE are heavy smoking history, years. She was a known case of Type 2 Diabetes Mellitus
hypoxemia, unexpected subnormal lung volumes and (DM), Hypertension (HTN), Sinusitis, Coronary Artery
severe reduction of carbon monoxide transfer. High- Disease (CAD) and Obstructive Sleep Apnoea (OSA). She
resolution CT (HRCT) is the mainstream diagnostic had a history of Total Knee Replacement (TKR) surgery in
parameter for CPFE. Apart from HRCT, spirometry 2014.
values are also used to assess the severity of the disease. [4]
Treatment options include symptomatic therapy as there On examination the patient’s vitals were as follows:
is no specific treatment available till date, and also
includes smoking cessation and oxygen therapy.[3] This PARAMETERS VALUES
case series involves 3 cases of CPFE with different BP 140/90 mmHg
symptoms and treatment has been given accordingly.[8]
BODY WEIGHT 68 kg
Keywords:- Combined Pulmonary Fibrosis and Emphysema
RESPIRATORY RATE 20 breaths/min.
(CPFE), Hypoxemia, HRCT, Spirometry.
PULSE RATE 65 beats/min.
I. INTRODUCTION
SPO2 97%
Combined pulmonary fibrosis and emphysema (CPFE) Table 1
is a rare pulmonary condition characterized by the
involvement of both upper lobe emphysema and lower lobe  Chest HRCT Examination:
fibrosis with very low diffusion capacity in contrast with  Heterogenous lung attenuation with areas of air trapping
subnormal spirometry that occurs mainly in heavy smokers and mosaic perfusion – likely secondary to obstructive
with severe dyspnoea and exercise limitation.[1][6] Cough and lung disease.
dyspnoea are common symptoms in patients with CPFE or  Multiple peripherally placed paraseptal bullae in the
Chronic Obstructive Pulmonary Disease (COPD) or upper lobe.
Idiopathic Pulmonary Fibrosis (IPF).[7] From several studies  There is evidence of multiple contiguous rows of
it has been found that a person with an already existing peripherally located lung cysts in the basal distribution,
COPD when exposed to cigarette smoke becomes vulnerable showing adjacent mild fibrotic component and subtle
to developing emphysema and pulmonary fibrosis. The high- traction bronchiectasis – Findings likely to represent
resolution computer tomography (HRCT) scanning has been honey combing.
adopted as the main diagnostic method for CPFE. The HRCT
would typically show centrilobular or paraseptal emphysema Therapeutic management of this condition includes rest,
which is often predominant in the upper zone.[4] The desired periodic assessment of oxygen saturation (SPO2) and oxygen
treatment option for a CPFE patient is a long term oxygen supplementation. Patient was initially treated with Foracort
therapy. Anti-fibrotic drugs (Pirfenidone, Nintedanib) have 200 MDI (Budesonide 200 mcg + Formoterol 6 mcg) at a
proven to relieve CPFE symptoms to a limit.[7] dose of 1 puff/twice daily which has to be used with Zerostat
VT spacer. After administering this, the patient developed
slight tremor and palpitation which resolved eventually on its
own. The patient was prescribed with T. ABFLO
(Acebrophylline) 100mg, T. Montek LC (Levocetirizine 5mg

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Volume 4, Issue 3, March – 2019 International Journal of Innovative Science and Research Technology
ISSN No:-2456-2165
+ Montelukast 10mg), T. Medrol (Methyl Prenisolone) 8mg  CASE 3
and Syp. Lupituss (Levocloperastine) 10ml for symptom A 82-year-old male patient was admitted in the
relief. respiratory department of a tertiary care hospital. The patient
had complaints of fever, nausea, vomiting, cough with
 CASE 2 whitish expectoration since 2 days. He was a known case of
A 83-year-old male patient was admitted in the Type 2 Diabetes Mellitus (DM), Hypertension (HTN),
respiratory department of a tertiary care hospital. The patient Dyslipidemia (DLP), CAD and COPD. The patient’s lab
had complaints of increased shortness of breath for 3 days, report showed an abnormally high CRP.
wheezing and cough with mucoid sputum for 3 weeks. He
had a history of hemoptysis 6 months back and chest On examination the patient’s vitals were as follows:
discomfort was observed. The patients had comorbidities like
CAD, Non ST-elevated Myocardial Infarction (NSTEMI), PARAMETERS VALUES
severe aortic stenosis, anemia, old Pulmonary Tuberculosis BP 140/90 mmHg
(PTB), COPD. The patient was an ex-smoker as well as an
ex-alcoholic. BODY WEIGHT 68 kg
RESP. RATE 20 breaths/min.
On examination the patient’s vitals were as follows:
PULSE RATE 65 beats/min.
PARAMETERS VALUES
SPO2 97%
BP 120/80 mmHg
Table 3
RESPIRATORY RATE 22 breaths/min.
PULSE RATE 98 beats/min.  Chest X-ray:
Chest x-ray showed infiltration in the right lungs.
SPO2 96%
Table 2  Chest HRCT Examination:
 Diffused intralobular septal thickening predominantly in
 Chest X-ray: bilateral lower lobes with multiple fibrotic bands showing
Chest x-ray showed features of bilaterally scattered fibrosis. secondary traction bronchiectasis and tiny cystic lucencies
Hence HRCT was taken. arranged in multiple row like configuration towards basal
segments of bilateral lower lobes associated with minimal
 Chest HRCT Examination: bilateral nodular pleural thickening.
 Extensive centriolar emphysema bilaterally with  Findings likely to represent idiopathic pulmonary
predominant involvement of upper lobes. fibrosis.
 Mild fibrotic bands with traction bronchiectasis –  Tiny paraseptal bullae in the bilateral upper lobes and
Findings likely to represent honey combing. right middle lobe suggestive of background emphysema.

Therapy for this condition includes rest, periodic Therapeutic management of this condition includes rest,
assessment of oxygen saturation (SPO2) and oxygen periodic assessment of oxygen saturation (SPO2) and oxygen
supplementation. Patient was initially treated with Neb. supplementation. Patient was initially treated with Neb.
Foracort 0.5mg (Budesonide 500mcg + Formoterol 20mcg), Duolin, Neb. Budecort (Budesonide) 0.25mg and Inj. Viatran
Neb. Levolin 1.25mg (Levosalbutamol), Neb. Ipravent (Cefoperazone 2g + Sulbactam 1mg) 3g and T. Azithral
(Ipratropium Bromide). The patient was prescribed with T. (Azithromycin) 500mg. The patient was prescribed with T.
Telekast F (Montelukast 10mg + Fexofenadine 120mg), T. Mucolite for symptom relief.
Mucinac (Acetylcystiene), T. Doxovent (Doxophylline) for
symptom relief. The patient later improved and thus II. DISCUSSION
discharged with following medications T. Sompraz
(Esomeprazole) 40mg, Neb. Foracort 0.5mg, Neb. Duolin CPFE is a rare pulmonary condition characterized by
2.5ml ( Levosalbutamol, Ipratropium), T. Doxovent 400mg, the involvement of both upper and lower lobe with
T. Mucinac 600mg, T. Ivepred (Methyl prednisolone) 8mg, emphysema and fibrosis respectively. Cough and dyspnoea
T. Telekast 40mg. are the most common symptoms that can be seen in patients
with CPFE. This condition can be diagnosed and confirmed
with the help of spirometry values and HRCT impressions.
The HRCT would show honey combing structures as well as
centrilobular or paraseptal emphysema in patients with
CPFE. As there is no specific treatment available till date, the

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Volume 4, Issue 3, March – 2019 International Journal of Innovative Science and Research Technology
ISSN No:-2456-2165
condition can be managed with long term oxygen therapy III. CONCLUSION
along with other medications which will provide
symptomatic relief. CPFE is a distinct pulmonary condition, so it is
important to recognise the severity of various pulmonary
This case series of CPFE showed three patients who symptoms associated with this condition. It will influence the
were confirmed with the condition by help of HRCT patient’s physical and social well being. Smoking may be the
impression. The treatment with corticosteroids and prime etiological factor for causing emphysema or fibrosis
bronchodilators are effective in improving the clinical course dominant. CPFE patients tend to exhibit a delay in the
of patients with CPFE. All the patients above showed a reduction of FVC and monitoring disease progression and
reduction in their oxygen saturation. Therefore, correction of therapeutic response to anti-fibrotic patients can be
this is the most important aim in the treatment of CPFE. Here challenging. It is extremely important to identify and urgently
the patients were advised to follow proper usage of Meter refer potential severe cases in order to have the appropriate
Dose Inhalers (MDI) and nebulizers for improved quality of investigations and have the appropriate care administered.
life. The patients who were prescribed with nebulizers
showed slightly more response than the patients who got IV. CONFLICT OF INTEREST
inhalers in their therapy. Therefore, nebulizers have a more
significantly curative effect, as it can effectively improve The authors declare no conflict of interest.
symptoms.

V. ABBREVIATIONS

CPFE Combined Pulmonary Fibrosis & Emphysema

COPD Chronic Obstructive Pulmonary Disease
IPF Idiopathic Pulmonary Fibrosis
HRCT High-Resolution Computer Tomography
DM Diabetes Mellitus
HTN Hypertension
CAD Coronary Artery Disease
OSA Obstructive Sleep Apnoea
TKR Total Knee Replacement
DLP Dyslipidemia
BP Blood Pressure
SPO2 peripheral capillary oxygen saturation
MDI Meter Dose Inhaler
Table 4

REFERENCES [6]. Jankowich M, Rounds S. Combined Pulmonary Fibrosis

and Emphysema Syndrome. Chest. 2012;141(1):222-
[1]. Cottin V. Combined pulmonary fibrosis and 231.
emphysema: a distinct underrecognised entity. European [7]. Cottin V, Nunes H, Mouthon L, Gamondes D, Lazor R,
Respiratory Journal. 2005;26(4):586-593. Hachulla E et al. Combined pulmonary fibrosis and
[2]. Cottin V. Combined pulmonary fibrosis and emphysema syndrome in connective tissue disease.
emphysema: bad and ugly all the same?. European Arthritis & Rheumatism. 2010;63(1):295-304.
Respiratory Journal. 2017;50(1):1700846. [8]. Tzilas V, Bouros D. Combined Pulmonary Fibrosis and
[3]. Cottin V, Brown K. Interstitial lung disease associated Emphysema, a clinical review. COPD Research and
with systemic sclerosis (SSc-ILD). Respiratory Practice. 2016;2(1).
Research. 2019;20(1).
[4]. Manjunath K, Udnur H. HRCT diagnosis of combined
pulmonary fibrosis and emphysema in a patient of
chronic obstructive pulmonary disease with pulmonary
hypertension and clinical or radiograph suspicion of
pulmonary fibrosis. BJR|case reports.
2016;2(4):20150070.
[5]. Jankowich M, Rounds S. Combined Pulmonary Fibrosis
and Emphysema Syndrome. Chest. 2012;141(1):222-
231.

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