Dr Gillani"s Blog: A 45 year old woman presents to your OPD with increasing shortness of breath and fatigue that is progressive over the last 8 months.

A 45 year old woman presents to your OPD with increasing shortness of breath and fatigue that is progressive over the last 8 months.

Her oxygen saturation is 85 % on air

There is no oedema on the feet .

She sometimes gets cough but it is non -productive.

On auscultation , there is bilateral crackles in lower lobes.

She does not get chest pains and her ECG is normal.

Her Chest X Ray is shown below :

1 . What are findings in the Chest X ray ?

2 . What is the most likely diagnosis ?

3 . What are the causes for this condition ?

4 . What are clinical features of this condition?

5. Which investigations should be performed ?

6 . What is the Differential Diagnosis of this condition ?

7 . What is the long term prognosis of this condition ?

8 . What are the management options for this condition ?

Answers given in comments section

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1.What are findings in the Chest X ray :

Bilateral reticular opacities in lower lobes

Appearance of variably sized cysts in a background of densely scarred lung tissue.

Honeycomb lung : in Lower lobes both sides.

Honeycomb lung refers to the characteristic appearance of variably sized cysts in a background of densely scarred lung tissue.

Microscopically, enlarged airspaces surrounded by fibrosis with hyperplastic or bronchiolar type epithelium are present. However, these changes are nonspecific and are often seen in numerous end-stage interstitial lung diseases (ILDs). Recognition of honeycomb change is imperative as its presence is associated with a poor prognosis.

2. What is the most likely diagnosis ?

Interstitial Lung Disease / ILD

Interstitial lung diseases (ILD), also called diffuse parenchymal lung disease, is a broad classification encompassing mainly non-neoplastic and inflammatory lung diseases that cause alterations to the lung parenchyma in a diffuse pattern.

Location

The location of honeycomb changes depends somewhat on the underlying disease; however, in most cases of interstitial lung disease (ILD), the changes begin in the subpleural regions of the lung and are most severe in the lower lobes and lower portions of all lobes.

3. What are the causes for this condition :

Although a large and diverse list of ILDs have been described, the majority seen in clinical practice are idiopathic pulmonary fibrosis (IPF),

Chronic hypersensitivity pneumonitis (HP),

Collagen vascular disease (CVD)–associated ILD, and sarcoidosis.

Idiopathic usual interstitial pneumonia (UIP) is the most common idiopathic interstitial pneumonia, and it is associated with a poor prognosis and eventual honeycomb changes.

Furthermore, nonspecific interstitial pneumonia (NSIP), although less common, can be associated with honeycomb changes. CVDs include systemic lupus erythematosus, rheumatoid arthritis, progressive systemic sclerosis (diffuse scleroderma), Sjögren syndrome, and dermatomyositis/polymyositis.

An increased frequency of bronchiolar histotypes in lung carcinomas appears to be associated with IPF, in which abnormal bronchiolar proliferation occurs in transformed small airways in honeycomb lung regions.

Dozens of drugs have been linked to ILD; however, methotrexate and bleomycin are the two agents most strongly associated with fibrosing interstitial pneumonia and they are capable of producing UIP and/or NSIP patterns.

Regardless of the underlying disease process, the universal pathophysiology is believed to be acute injury to lung parenchyma leading to chronic interstitial inflammation, tissue destruction, fibroblastic activation and proliferation, pulmonary fibrosis and, eventually, architectural remodeling with honeycomb changes. This process usually evolves over a period of months to years; however, it can be accelerated.

4 .What are clinical features of this condition?

Characterization of interstitial lung disease (ILD) requires clinical, radiologic, and pathological correlation.

Clinically, patients often note progressive dyspnea or nonproductive cough present over a period of months to years.

Other symptoms may include hemoptysis, wheezing, and chest pain; however, ILD can occasionally occur in asymptomatic patients in which ILD is suspected only radiographically.

In honeycomb lung secondary to idiopathic usual interstitial pneumonia (UIP), most patients are over age 50 years and have had symptoms for more than 6 months.

Patients with collagen vascular disease (CVD) often have associated rheumatologic signs and symptoms, although lung disease may occasionally be the first manifestation of their CVD.

The possibility of CVD should be explored in any patient with ILD, particularly young women in whom CVD is more common.

5 .Which investigations should be performed ?

From the perspective of the pathologist, it is important to understand that high-resolution computed tomography (CT) scanning is a critical tool for the diagnosis of ILD as it creates detailed images of the lung parenchyma and airways.

Basic patterns of ILD on CT scans include reticular patterns (thickened interlobular and intralobular septa), nodular patterns (airspace and interstitial nodules), cystic patterns (bronchiectatic or honeycomb cysts), and altered attenuation (ground glass opacification or mosaic attenuation patterns).

Based on the imaging, the radiologist is often able to provide a differential diagnosis for the pattern of ILD that can be correlated with pathologic impressions of the biopsy. Furthermore, radiographic findings help to guide the surgeon in appropriate sampling of the lung tissue.

6 . What is the Differential Diagnosis of this condition ?

Differential Diagnosis

The most important diagnostic consideration is correctly identifying patients with a usual interstitial pneumonia pattern, because the prognosis is much worse in comparison to other patterns of interstitial lung disease.

7. What is the long term prognosis of this condition ?

Prognosis and Predictive Factors

The prognosis in interstitial lung disease varies with the underlying etiology, but when honeycomb changes are present, the prognosis is poor. Upon being diagnosed with usual interstitial pneumonia, most patients without a lung transplant die within 3 years.

8. What are the management options for this condition ?

Treatment

There remains to be curative therapy for the causative entities of honeycomb lung; however, emerging modalities exist which can slow the progression of disease.

Immunosuppresive or antifibrotic agents, depending on the etiology of the disease (ie, immunosuppressive therapy for collagen vascular disease (CVD)–related disease or anti-fibrotic agents in idiopathic pulmonary fibrosis [IPF]), may be appropriate.

In the treatment of IPF, nintedanib and pirfenidone are commercially available in certain countries, and they can help to slow the progression of disease.

As cases progress, single or double lung transplantation can be performed, with patients having a median survival of 4.7 years, with survival benefit and an improved quality of life.

Further Reading :

https://emedicine.medscape.com/article/2078590-overview#a3

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